The magnetic resonance characteristics of autoimmune demyelinating diseases are complex and represent a challenge for the radiologist. In this study we presented two different cases of detected autoimmune demyelinating diseases: one case of acute disseminated encephalomyelitis and one case of neuromyelitis optica, respectively. Expected and unexpected findings of magnetic resonance imaging examination for autoimmune demyelinating diseases were reported in order to provide a valuable approach for diagnosis. In particular, we highlight, review and discuss the presence of several uncommon imaging findings which could lead to a misinterpretation. The integration of magnetic resonance imaging findings with clinical and laboratory data is necessary to provide a valuable diagnosis. Autoimmune demyelinating diseases (ADD) constitute a heterogeneous group of diseases with variable clinical and imaging manifestations . MultiplDiagnosing ADD can be very challenging owing to their complex and variable radiological features. Magnetic resonance imaging (MRI) plays a pivotal role in the diagnostic workflow ,7. HowevAim of this paper is to describe two cases of diagnosed ADD: a case of ADEM and a case of NMO, respectively, which posed a real diagnostic challenge due to their heterogeneous radiological presentation, atypical for ADD, and to review epidemiological, clinical, laboratory and imaging characteristics of this group of disorders.A 57-year-old male with a history of chronic alcoholism presented to the emergency department with severe muscle weakness, which started with a progressive impairment in walking, followed by a feverish state (38 Celsius degrees), worsening up to being unable to walk. The patient was alert, collaborating, oriented in space, disoriented in time. Due to this clinical presentation, the patient was transferred to the neurology department for appropriate treatment. Osteo-tendon reflexes were normal in the upper limbs but absent in the lower limbs. Autoimmune screening, blood chemistry tests, and neoplastic markers were within normal limits. The search for pathogenic microorganisms was negative. Cyanocobalamin serum levels were normal. Anti-Myelin Oligodendrocyte Glycoprotein (MOG) and anti-Aquaporin-4 (AQP4) antibodies were absent, and the search for oligoclonal bands turned negative. Cerebrospinal fluid (CSF) examination demonstrated a clear rock water, marked pleocytosis , and high CSF proteins (128 mg/dL). MRI of the brain and spinal cord was acquired .The integration of clinical and imaging characteristics allowed ADEM diagnosis.Follow-up imaging revealed that the ADEM lesions were resolved and that no new lesions had appeared. As a result, the disorder was monophasic.A 57-year-old female presented to the emergency department with paresthesia of the trunk, pelvis, and lower limbs with progressive difficulty of movement up to the inability to walk and collapse when standing. On the same day, double sphincter disorder was reported. The diagnosis of spinal cord syndrome was considered after neurological counseling. The patient was hospitalized for diagnostic and therapeutic evaluation. The patient was alert and cooperative, with visual acuity reduction on the left associated with retrobulbar pain. The patient presented weakness of the upper limbs and severe deficiency of the lower limbs. Osteo-tendon reflexes were normal in the upper limbs but absent in the lower limbs. Anti-MOG antibodies and oligoclonal bands were absent. Anti-AQP4 antibodies were present. Microbiological examination of CSF and peripheral blood were positive for previous and latent human beta-herpesvirus-7 (HHV 7) infection. Marker of active HHV 7 infection were excluded. MRI of brain and spinal cord was acquired .Despite the absence of alterations of optic nerves on MRI, which represents a major criteria for NMO diagnosis, the integration of clinical and imaging features allowed the aforementioned diagnosis.Autoimmune demyelinating disorders such as ADEM and NMO may be particularly challenging to distinguish from MS, hampering a prompt and accurate diagnosis . MRI is MS is an acquired inflammatory demyelinating disease of the central nervous system (CNS). MS has high prevalence in northern Europe and is rare in regions located near the equator. For unknown reasons, women are affected more frequently than men with a 3:1 ratio) like in most diseases defined as autoimmune . The typ ratio liADEM is a demyelinating CNS disease which mainly affects the pediatric population, with higher incidence and prevalence in regions distant from the Equator and a slight male predominance (M:F ratio up to 2.6:1) ,23. The In the patient of case 1, the brain lesions location could lead to the misdiagnosis of MS, in particular for the presence of perpendicular periventricular lesions (Dawson’s fingers). Nevertheless, the sagittal FLAIR image showed hyperintense lesions affecting the ependymal surface of the corpus callosum with a marble pattern, as observed in NMO. On the other hand, the involvement of the spinal cord, both on axial and sagittal plane, as well as the lack of enhancement of spinal cord lesions suggested the diagnosis of ADEM. In this case, it was not possible to make an accurate diagnosis without the support of clinical and laboratory data: CSF analysis showed pleocytosis without oligoclonal bands, and the ADEM diagnosis was reasonably confirmed. This patient presented MRI brain findings atypical for ADEM diagnosis.Neuromyelitis optica spectrum disorders (NMOSD) are severe autoimmune inflammatory demyelinating diseases of the CNS with frequent involvement of the optic nerves and spinal cord . The NMOIn the patient of case 2, the main laboratory finding was the presence of positive serum and CSF anti-AQP4 antibodies, without oligoclonal bands. This finding suggested the diagnosis of NMO. However, MRI reported the absence of visible signs of optic neuritis, a major criteria for NMO diagnosis . On the Namely, Transverse Myelitis (TM) is an acute or subacute inflammation of the spinal cord, which can be idiopathic (iTM) or secondary to other diseases . IncidenMOG antibody disorders typically occur in young Caucasian patients, with low female prevalence ,83,84,85Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory CNS disorder involving predominantly the brainstem, described in 2010 for the first time by Pittock and colleagues . AlthougThe two cases presented in this paper highlight well the challenges that need to be solved in order to formulate an accurate diagnosis: the presence in both patients of hybrid MRI findings that could be present in more than one ADD, could have easily led to an incorrect interpretation and misdiagnosis. This does not mean that, in this scenario, the role of MRI has weakened, rather that the integration of MRI findings with clinical and laboratory data is mandatory. These cases and the literature review underline the complexity of magnetic resonance imaging in autoimmune demyelinating diseases diagnosis and the crucial need of clinical and laboratory data integration. Some magnetic resonance findings can interchange between different demyelinating pathologies or can not be expressed simultaneously in a single demyelinating disease.


Cases of patients with cystic partially differentiated nephroblastoma (CPDN) have been reported to date, which presented as polycystic renal tumor in all of them. It is a special pathological type of nephroblastoma. Here, we report the first case of a unicystic CPDN in a child. The patient was diagnosed with a simple renal cyst and underwent laparoscopic decortication. The naive nephron was found in the pathological section, and the diagnosis of CPDN was confirmed. The patient then underwent a radical nephrectomy and six cycles of postoperative chemotherapy. There was no recurrence or metastasis after 2 years of follow-up. Pediatric CPDN presenting as a unicystic renal tumor poses a new challenge to the diagnosis, differential diagnosis, and treatment of unicystic renal tumor. Cystic partially differentiated nephroblastoma (CPDN) usually occurs in children under 2 years old, with a male-to-female ratio of about 2:1 . More thA 3-year-old girl was diagnosed with a cystic tumor on the left kidney by abdominal ultrasound in another hospital 2 weeks prior after she experienced occasional abdominal pain, but she had no abdominal pain when she presented to our clinic. The reexamined ultrasound showed a cystic tumor inside the left kidney, with a size of 4.6 × 3.9 cm, with a clear boundary, thin wall, and uniform internal sound transmission .A measurement of the patient’s kidney showed a creatinine level of 33 µmol/L and a urea nitrogen level of 4.6 mmol/L. Tumor marker tests showed carcinoembryonic antigen (CEA) <0.30 ng/ml, alpha fetoprotein (AFP) 2.15 IU/ml, carbohydrate antigen 125 (CA-125) 17.0 U/ml, carbohydrate antigen 19-9 (CA19-9) 9.42 U/ml, β-human chorionic gonadotropin (β-HCG) < 0.20 mIU/ml, neuron-specific enolase (NSE) 11.6 ng/ml, and cytokeratin 19 fragment (CK-19) 1.8 ng/ml. However, the girl’s routine blood test and urinalysis were normal.The patient was diagnosed with a simple left renal cyst, and she underwent a laparoscopic decortication of the renal cyst. During the operation, a unicystic tumor in the middle and upper pole of the left kidney was revealed, the inner wall of the cyst was smooth, the cystic fluid was clear, and there was no obvious connection between the cyst and the renal pelvis . The cysIn the second operation, a radical nephrectomy was performed on the left kidney, and the operated area was rinsed with distilled water. No abnormal lymph nodes were found. The second histopathological examination also showed CPDN, with the immunohistochemistry showing vimentin (+), Wilms tumor protein (+), epithelial membrane antigen (+), acidic calcium-binding protein S-100 (−), neuron-specific enolase (−), and a protein Ki-67 index of 17%.Six cycles of chemotherapy were performed after the operation, and the chemotherapy protocol was vincristine combined with dactinomycin. There was no recurrence of the tumor and no occurrence of metastasis after 2 years of follow-up.Brown standardized the naming of CPDN for the first time in 1975 .A complete search of the PubMed, EMBASE, and Wanfang databases was performed in the period between January 1975 and May 2022 to identify all reports describing pediatric CPDN. We also included articles that described CPDN, as this is the most important differential diagnosis in children with CWT and CN. A cross-reference check was used to identify potential additional reports. For multiple case reports from the same author or institution, we checked each case carefully to avoid the same case being repeatedly included in the study. Cases with a high probability of repetition were also excluded from the study. The inclusion criteria of the articles included the following conditions: First, the article had to report well-described pediatric patients with CPDN. Second, the article had to be an original one. Third, the article had to be available as a full text or a well-described abstract. Fourth, the article had to be written in English, Spanish, Chinese, or Dutch. All manuscripts were screened by two independent reviewers (Liu Chao and Li Xiang). A total of 121 cases of pediatric CPDN were retrieved from 48 articles. In all cases, the tumors were polycystic, as described by surgical specimen pictures or literature. A polycystic renal tumor was considered the only manifestation of CPDN , preoperative chemotherapy cannot effectively reduce the size of a CPDN tumor . The 10 The postoperative recurrence rate of CPDN was low . The 92 In summary, CPDN is rarely reported in the clinic and can be easily confused with CN and CWT. In this study, a unicystic pediatric CPDN is reported for the first time, which poses a new challenge to the diagnosis and treatment of renal cystic tumor. There are no specific clinical characteristics of pediatric CPDN. Surgery is the first choice of treatment, and histopathological examination is the only means of diagnosis. For patients with stage II CPDN or above, postoperative chemotherapy is recommended. The recurrence rate of CPDN is low, and the overall prognosis is good.


Spigelian hernias are rare herniations through the Spigelian fascia, with an incidence rate of 0.12-2.0% of all hernias. Diagnosis may be difficult due to a potential lack of symptoms until complications arise. Therefore, imaging with either ultrasound or CT with oral contrast is recommended to confirm the diagnosis if a Spigelian hernia is suspected. Once the diagnosis has been established, it is essential that operative repair be performed as soon as possible because 24% of Spigelian hernias become incarcerated, and 27% of Spigelian hernias lead to strangulation. Management options include open surgery, laparoscopic surgery, and robotic surgery. This case report discusses the management of a 47-year-old man with an uncomplicated Spigelian hernia that was repaired with the robotic ventral transabdominal preperitoneal repair technique. A Spigelian hernia is defined as the herniation of abdominal contents or peritoneum through a defect in the Spigelian fascia, which consists of the combined aponeuroses of the transversus abdominis and internal oblique muscles . With anA 47-year-old man presented with several months of left lower abdominal quadrant discomfort and swelling, which was exacerbated by straining and alleviated with rest. When these symptoms initially began, the patient stated that he had first sneezed, then felt a tearing sensation in his left lower abdominal quadrant, and finally noticed a bulge in his left lower abdominal quadrant. He denied any nausea, vomiting, fever, chills, sweating, or changes in bowel movements. Past medical history was significant for hiatal hernia, right inguinal hernia, left inguinal hernia, hypercholesterolemia, and acid reflux. The patient was a non-smoker and only consumed alcohol occasionally.On examination, the patient was alert and oriented without tachycardia or tachypnea. No masses were palpated during the abdominal exam; however, the patient did describe a vague sensation of fullness below the level of the umbilicus in the left lower abdominal quadrant at the semilunar line. Additionally, there was no evidence of a recurrent bilateral inguinal hernia. Upon further investigation with CT imaging, a 3.5 cm left-sided Spigelian hernia and a 3 cm periumbilical incisional hernia were identified, as seen in Figure After the general anesthesia team had performed an initial tap block, they prepped and draped the patient’s abdomen and then intubated the patient. Timeout was then followed. First, a skin incision was made in the left upper abdominal quadrant, in which a Veress needle was inserted and used to establish pneumoperitoneum, along with an optical viewing trochar. Next, four robotic trochars were inserted in the patient’s right abdomen. Afterward, the Da Vinci robotic surgical system was docked.The initial step of the robotic surgery was used to dissect the periumbilical incisional hernia, reduce its contents, and pull the fat out of the defect. This left the preperitoneal space with an opening, as seen in Figure After both hernia defects had been closed, the sigmoid colon was pulled to the side in order to explore the pelvic cavity for the location of a suspected pelvic cyst. However, a location where fluid could have accumulated to create a pelvic cyst could not be found, and it was eventually determined that the pelvic cyst had probably been marsupialized. Therefore, it was decided that repeat imaging could be conducted in the future. Lastly, the pneumoperitoneum was released, and all incisions were closed with 4-0 Vicryl sutures and Dermabond. The patient was then taken to the recovery room in stable condition, and his postoperative recovery period was unremarkable and without complications.Spigelian hernias were first defined by Flemish anatomist Josef Klinkosch in 1764 and named after Belgian anatomist Adriaan van den Spiegel who defined the semilunar line in 1627 .SpigeliThe typical presentation of a Spigelian hernia is an intermittently palpable bulge along the semilunar line, which is most prominent when standing or during Valsalva maneuvers , 7. HoweOftentimes, a Spigelian hernia is not suspected until the patient experiences pre-operative complications, such as incarceration, strangulation, or bowel obstruction . ComparePostoperative complications from robotic repair of Spigelian hernias occur at a rate of 12%. They can include mesh infections, injuries to the abdominal viscera, seromas, hematomas, surgical site infections, and hernia reoccurrence .ApproprDue to the low incidence rate of Spigelian hernias, clinicians need to be aware of their typical presentation as an intermittent palpable bulge along the semilunar line, which is most prominent with standing or during Valsalva maneuvers. Once a Spigelian hernia has been confirmed with ultrasound imaging followed by CT imaging with oral contrast, it is essential to schedule an operative repair as soon as possible due to their 24% incarceration rate and 27% strangulation rate. If a Spigelian hernia patient undergoes surgery in a timely manner, then they are likely to experience better outcomes. Amongst the surgical modes available, robotic surgery offers the benefits of improved access to the different abdominal wall layers, better ergonomics, greater freedom of instrumentation, and increased image stability. Furthermore, robotic surgery should be considered for Spigelian hernia patients due to a lower hernia recurrence rate than laparoscopic procedures and a lower hernia complication rate than open procedures. Lastly, when repairing a hernia that is at risk of intraperitoneal adhesions, surgeons should consider the use a hybrid composite mesh that offers the benefits of high durability, low infection rate, and a low rate of adhesions.Learning objectives include (I) Recognizing the typical presentation of a Spigelian hernia and the associated risk factors; II) Acknowledging the high rate of complications resulting from Spigelian hernias, despite their low rate of incidence; III) Discerning the appropriate imaging modalities to be used for Spigelian hernias; IV) Comparing and contrasting the advantages of different surgical techniques that can be used to repair a Spigelian hernia; and V) Comparing and contrasting the advantages of different mesh materials.


Optic pathway glioma (OPG) comprises 10% of pediatric brain tumors and 40% of all pediatric low-grade gliomas (pLGGs). While generally considered benign pathologically, many require interventions with chemotherapy, radiation, or targeted therapies. Management has historically foregone tissue diagnosis given the classical clinical/radiographic presentation of these tumors, inability to safely remove the lesions surgically, and efficacy and safety of available chemotherapy options. Furthermore, when considering such aspects as their delicate location, the role of surgery continues to be heavily debated. More recently, however, a greater understanding of the genetic drivers of OPGs has made operative tissue sampling a critical step in management planning, specifically for patients without Neurofibromatosis, Type I (NF1). Given the need for long-term, complex management of pediatric OPGs, it is crucial that a multidisciplinary approach is employed, and the rapidly expanding role of molecular characterization be incorporated into their management. Table 1), which can present throughout childhood, NF1-associated OPGs typically arise by 5 to 6 years of age (Pediatric low-grade glioma (pLGG) is the most common CNS tumor in children, constituting 40%–50% of all pediatric brain tumors . While ts of age . Presents of age . Childres of age , 5, 6, 8Histologically, these tumors are low-grade with majority being Pilocytic Astrocytomas (PA) , 7, 8. HThe clinical course of OPG may be unpredictable due to variability in the tumor’s natural history. The behavior of these tumors, while commonly thought quite indolent, can include progression, stable disease, and, on occasion, regression , 8, 15. Management of OPGs is based on patient age, clinical presentation, location, surgical resectability, and, when available, histopathological findings. Treatment options include observation, chemotherapy, surgical biopsy, resection, radiotherapy, and molecularly targeted therapy. Despite the understanding that a significant portion of these tumors will remain stable, as many as 35% of OPGs in NF1 patients will require treatment at presentation and more than 90% of sporadic OPGs will require treatment , 11. LonComplete surgical resection of OPG is generally not possible due to their location and complex anatomical relationship to sensitive structures. It is important to note that various opinions exist regarding surgical strategies for OPG , 13, 17.The role of tissue biopsy has been variable due to the surgical risk associated with the location of OPG as well generally favorable response to chemotherapy. Biopsy rates have increased over recent years with the emergence of targeted therapies and a greater understanding of the genetic drivers of pLGG. A more sophisticated understanding of the biological landscape of pLGGs, first evident in NF1 patients harboring PAs, has refocused treatment of OPGs on molecularly targeted options. Compared to their adult counterparts, pLGGs exhibit a greater likelihood to activate BRAF, with subsequent upregulation of the Ras/MAP-Kinase pathway. In fact, extensive genetic analysis has discovered that they represent a single pathway disease with genetic alterations converging on the MAPK pathway , 20, 21.In NF1 patients, alteration of the neurofibromin 1 gene on chromosome 17 leads to dysregulation of neurofibromin activity. Neurofibromin works as a tumor suppressor by reducing RAS-GTP mediated activation of its effector pathways, including MAPK and phosphoinositide 3-kinase (PI3K), and thus regulates cell growth and proliferation though downstream proteins . Dysregulation of neurofibromin results in upregulation of Ras and mTOR activity, leading to a pro-mitotic state. In pLGG, abnormal MAPK activation is the most frequent biological aberration demonstrated, and OPGs also frequently exhibit mutations in the same pathway . The twoGenetic anomalies in pLGG vary based on histology, with BRAF fusion seen more commonly in Pilocytic Astrocytoma (PA), while V600E mutation is more prevalent in pleomorphic xanthoastrocytoma (PXA) and ganglioglioma , 28. TheThe goal of care in patients with OPG is to preserve neurological function and maximize quality of life. Based on the indolent nature of many OPGs and the success of current chemotherapy regimens, initial management may consist of close observation, with chemotherapy used in cases of visual deterioration or radiographic progression , 8, 11. Given today’s level of knowledge regarding the biology of pLGG, the value of tissue diagnosis is a critical factor. Tissue characteristics that can only be revealed through direct sampling may guide clinical management. For example, OPGs possessing BRAF fusion have shown a tendency to arrest and are even prone to senescence , 39, andWhile prospective, randomized control trials are ongoing, success with molecular targeted therapy in OPG has been well documented. The first MEK inhibitor (MEKi) was developed in 1995 and targeted BRAF inhibitors started with sorafenib, a broad-spectrum kinase inhibitor, followed by vemurafenib and dabrafenib, BRAF V600E specific targeted inhibitors. The initial clinical experience with sorafenib demonstrated the importance of knowing the underlying genetic make-up of a tumor being treated with targeted therapy. A phase II trial of sorafenib in pLGG was closed early to an unexpectedly high rate of rapid and early progressive disease, in 9 of 11 patients enrolled. It was determined that sorafenib may lead to paradoxical ERK activation in NF1-deficient cells as well as BRAF wild-type and KIAA1549:BRAF fusion cells .Table 2). Dabrafenib and vemurafenib have been studied in the management of V600E+ pediatric gliomas. A phase I/IIa trial with dabrafenib in pLGG found a 1-year PFS of 85% , however, tissue must be acquired surgically.Management goals in the era before targeted therapy were to preserve as much function as possible as well to maintain an acceptable quality of life. As stated above, this remains true, but increasingly individualized multidisciplinary management is now at the forefront of OPG treatment . In thisHistorically, the role for biopsy was limited in the setting of OPGs. They are not amenable to complete resection, are histologically benign, involve critical neuroendocrine structures, and often respond favorably to chemotherapy. Based on these criteria, treatment paradigms de-emphasized the role of surgery. Existing consensus supports biopsy in cases that demonstrate atypical imaging findings in patients with NF1 , or in sporadic OPG patients who are involved in relevant clinical trials  and concIn general, biopsy rates have increased in recent years, guiding the use of targeted therapies . There eIf surgical resection is not indicated, options for tissue sampling include endoscopically assisted biopsy, stereotactic needle biopsy, and open biopsy. The optimal approach is dependent on tumor location and its relationship to important anatomic structures and should be determined on an individualized basis. Improvements in imaging quality and intraoperative navigation allow for precise operative planning and anatomical understanding, thereby improving the safety of surgical biopsy, regardless of the specific approach that is selected . StereotWhile aggressive operative resection is not standard for most OPGs, neurosurgical intervention for patients with OPG in certain contexts is not controversial. The association between OPGs and hydrocephalus is well recognized and may require prompt surgical attention , 12, 18.An additional indication for surgical debulking is symptomatic mass effect –6, 18. WWhile we endorse the use of biopsy/surgery to obtain the most molecular data possible to help guide therapy, we recognize there may be limitations to the use of these methods in low/middle income countries (LMIC). The importance of subgroup directed therapy has been shown to be important and encouraged to be considered while treatment planning in countries with limited resources  and grouOptic pathway glioma can be associated with good outcomes regarding both tumor control and quality of life. The role of surgery for these patients is dynamic and exists on a spectrum that spans from clear indications to more controversial interventions. The promise and evolution of molecularly targeted treatments has increased the role of surgical intervention for these patients as tissue diagnosis is required for proper therapy selection. This management approach also demonstrates the need for well-established comprehensive multidisciplinary care in the treatment of optic pathway gliomas. Through utilization of a team of physicians that includes neuro-oncologists, neurosurgeons, ophthalmologists, pathologists, radiation oncologists, endocrinologists, and several other allied health professionals, optimal care of these patients throughout childhood will afford them the best chance of successful care.


The purpose of this case presentation was to highlight the importance of an adequate evaluation of images when suspicious of atypical leiomyoma and the importance of performing an extemporaneous biopsy during surgery to ensure the lesion is a benign muscular cell tumor.Here, we present a case of a 34-year-old nulliparous woman who presented with a history of infertility and irregular menstrual cycles. A highly vascularized pelvic mass was visualized by Doppler ultrasound and a contrast MRI suggestive of uterine fibroid. Intraoperatively, the lesion was found adjacent to the uterus, with a second lesion deeper into the myometrium. The pathology reported a highly cellular leiomyoma with a focus of adenomyosis. Both lesions were extirpated without complications. The patient recuperated favorably within three months of follow-up. This case shows an example of a variety of the typical histology that can be found in uterine fibroids. Although the management of atypical leiomyomas could vary in different scenarios, conservative treatment is recommended if fertility wishes are present. In all cases, it is mandatory to exclude any possibilities of malignancy, like sarcoma, which would completely change the intraoperative management. Uterine fibroid is a frequent benign tumor condition of the uterus, which is found in 70-80% of women above 50 years of age . The cauOn the other hand, leiomyosarcomas are rare malignant uterine tumors associated with a significant proportion of uterine cancer deaths and can be suspected when performing imaging studies . ChangesCellular leiomyoma (CL) is a rare variant of uterine leiomyoma, which is difficult to detect clinically or even with imaging. However, immunohistochemical and molecular changes have been proved present during this variation . Dundr eIn women without fertility wishes and significant symptomatology, a hysterectomy for leiomyomas could be done. More conservative treatments like myomectomy are often preferred in premenopausal women. However, it is mandatory to rule out the possibility of malignancy before attempting medical or local excision management .Uterine leiomyoma and adenomyosis can be present in the same uterus in about 15-57% of females with symptomatology, and in those cases, women can complain of more symptoms than when separate diagnostics are reported . AdenomyA 34-year-old nulliparous woman presented with a history of primary infertility and irregular menstrual cycles over two years. Physical examination was consistent with abdominal tenderness in the lower abdomen. During the genital examination, a tender mass was found and confirmed with pelvic ultrasonography and contrast MRI, documenting a homogeneous mass lesion right para uterine measuring 10.2×7.6 cm with high vascularization after IV contrast administration  composed of collagen, fibronectin, laminin, and proteoglycans . AlthougBased on clinical and image findings, our patient was offered surgical treatment. It is well-known that normal myometrial smooth muscle cells grow uniformly and parallel to the major axis of the cell, whereas uterine leiomyoma cells form globular aggregates or nodules, contributing to the swirled pattern . SurgicaThe case studies in Chapron et al. have shown smooth muscle growing as benign masses around endometrial tissue, usually exhibiting well-defined organization in the myometrium. Smooth muscle cells are increased in number and size compared to the surrounding myometrial cells . BecauseCellular leiomyoma is a histological subtype with more compact smooth muscle cells and less collagen, showing in MRI a substantial enhancement after injection of contrast agent . These fIn some situations, distinguishing between an ovarian lesion and a uterine mass is complex, and several signs have been described to differentiate the location of the ovarian lesion from the uterine mass . That waUterine fibroid is a benign condition found in different locations along the uterus and is associated with additional symptomatology that requires surgical intervention in many cases. The importance of adequate interpretation of images before surgery to identify possible features that can guide a malignant diagnosis of leiomyosarcoma or, in better circumstances, an associate condition like adenomyosis is noteworthy. Also, it is relevant to consider the presence of histological variations of leiomyoma, like high cellularity, that could make the surgical approach more difficult during the excision in patients with fertility wishes. Therefore, the relevance of performing extemporaneous biopsy to discard malignancy characteristic of the tissue with subsequent histology confirmation, especially in those cases like this one where high vascularity was observed before surgery and visible differences like thicknesses with irregular borders were seen. Although the presence of high cellularity and an adenomyosis focus are benign diagnoses that do not differ the conservative versus radical surgical treatment, histological confirmation is the clue for a successful outcome after surgery.


Bladder perforation is a potentially life-threatening condition, typically occurring after genitourinary trauma. The vast majority of cases are secondary to blunt abdominal trauma resulting in pelvic fractures, with motor vehicle accidents the commonest cause. There are however a wide range of underlying causes, including iatrogenic injuries and spontaneous perforations. This case series of 4 unusual cases of bladder perforations presenting to a single center under the same consultant within a 3-month period aims to highlight the diverse nature of patients who can present with bladder perforations and the different management options available. Bladder injuries are not uncommon and occur in up to 10% of abdominal trauma The majority of patients with significant bladder injuries present with visible hematuria, blood per urethra (PU), and/or lower abdominal pain The preferred method of diagnosis of a bladder perforation has historically been via stress retrograde cystography including post-void images In this case series, we present a single surgeon's experience of 4 patients presenting to our institution with bladder perforations in just a 3-month period. The electronic records of these patients were retrospectively reviewed and the relevant clinicopathological and radiological data were collected. Informed consent was obtained from all patients. This study was granted institutional review board exemption from the Beaumont Hospital ethics committee.Case 1 is of a 60-year-old male who presented with a large extraperitoneal bladder perforation sustained following a fall from a height. He was initially brought in by ambulance to his local hospital after he had sleep-walked through a second-floor window, sustaining a significant pelvic fracture. Following initial resuscitation in the emergency department (ED), he underwent a trauma protocol CT with a delayed urographic phase. This demonstrated a large anterior bladder wall defect with active contrast extravasation into his extraperitoneal space, tracking down into his scrotum . His pelIntraoperatively, he was found to have an 8 cm defect in the anterior wall of his bladder. This was repaired in 2 layers with absorbable sutures. A leak test was performed at the end of the case which was negative. A surgical drain and urethral catheter were left in situ. The drain was removed after 3 days and he was then transferred to the national pelvic/acetabular orthopedic tertiary referral center for definitive repair of his pelvic fracture. He underwent a successful trial without catheter (TWOC) there following a CT cystogram which was performed 10 days after his initial bladder repair .Case 2 is of a 38-year-old female who presented to her local hospital's ED with nausea, vomiting, abdominal pain, and visible hematuria 7 days following an elective caesarean section (CS) there. She was found to have a distended abdomen without an obviously palpable bladder. Blood results demonstrated an acute kidney injury (serum creatinine 135 µmol/L) and a Hb of 11g/dL. She was catheterized and resuscitated with intravenous fluids. She underwent a contrast-enhanced CT which showed a thickened uterus with a small hematoma and a trace of intra-abdominal free fluid only, all of which were in keeping with postoperative changes. Her serum creatinine improved initially following rehydration, however, following removal of her urethral catheter her creatinine level increased and she began to leak fluid through her CS wound. She thus underwent a follow-up CT scan on day 10 post-CS with a dedicated urographic phase which demonstrated a large volume of intraperitoneal-free fluid and a suggestion of a small defect in the dome of her bladder .Fig. 2CaFollowing consultation with ourselves, a large-bore silicone urethral catheter was inserted, intravenous antibiotics were commenced and she was transferred to our institution. Her vital signs were within normal limits, she was clinically well and her distension had improved significantly following catheterization so a decision was made to insert a percutaneous drain in interventional radiology and to treat her conservatively. This drained large volumes initially with a creatinine sample confirming it to be urine. She remained clinically well and her serum creatinine normalized quickly. Her drain output tapered steadily during the first few days of her admission and it was then removed. She was discharged home on oral antibiotics after her drain was removed but with her urethral catheter still in situ and she returned for a stress retrograde cystogram 3 weeks following her transfer to our institution . This coCase 3 is of an 18-year-old female who presented to our ED with abdominal pain and distension. She had a background history of spina bifida and mobilized with the aid of a wheelchair. She had previously undergone insertion of a ventriculoperitoneal shunt in early childhood, followed by an augmentation cystoplasty and mitrofanoff procedure, through which she performed clean intermittent self-catheterization. On presentation to the ED, she was found to have generalized and significant abdominal tenderness but was clinically quite well. Her blood results on presentation were all within normal limits, however, due the severity of her pain and her background history, she underwent a CT scan following initial fluid resuscitation and insertion of an indwelling urethral catheter via her mitrofanoff.Her CT revealed multiple stones within her bladder and delayed urographic images confirmed contrast extravasation in keeping with an IBP .Fig. 3CaGiven that her pain improved significantly following mitrofanoff catheterization and given that she was apyrexial and clinically well, a decision was made to manage her conservatively with intravenous antibiotics, an indwelling mitrofanoff catheter and placement of a large bore urethral catheter. She remained hemodynamically stable throughout her admission and was discharged home 5 days later with her urethral catheter on free drainage and her mitrofanoff catheter spigotted. She returned for a stress cytogram after 2 weeks which showed no ongoing leak, following which she returned to performing clean intermittent self-catheterization with removal of both catheters. She underwent a successful cystolitholapaxy with complete clearance of her bladder stones 6 weeks after her initial presentation.Case 4 is of a 59-year-old male who presented to the ED with severe abdominal pain. He suffered a minor and low impact fall from standing the previous night after moderate alcohol consumption. He was hemodynamically stable on presentation but unable to pass urine with blood PU. Examination revealed an exquisitely tender abdomen. A bedside focused assessment with sonography for trauma scan in ED revealed intra-abdominal free fluid. He underwent an urgent CT scan which suggested an IBP, which was then confirmed on a subsequent CT cystogram . He undeHis abdominal drain was removed 3 days postoperatively and he was discharged home on day 8 with his catheter in situ. He returned for a retrograde stress cystogram after 2 weeks which confirmed no ongoing urine leak , and he P = .02) and overall hospital stay  compared to those undergoing simultaneous bladder repair The description of bladder perforations is often simplified as either extraperitoneal or intraperitoneal, as extraperitoneal perforations can generally be managed conservatively, while IBP typically require surgical repair Patient one from our series did not undergo simultaneous bladder and pelvic fracture repair as the severity of his pelvic fracture necessitated transfer to a national pelvic and acetabular orthopedic tertiary referral center for definitive repair. The extent of his urine leak was so significant that the patient had developed significant cellulitis in his upper thighs and abdomen and developed large and tense bilateral scrotal collections due to the extravasated urine before he underwent his laparotomy. In addition, the authors felt that delaying his bladder repair would increase his long-term risk of fistula development and increase the risk of infection at the time of his pelvic fracture repair.Although iatrogenic injury only accounts for a small percentage of bladder perforations, the bladder is the urological organ most frequently affected by iatrogenic injury The standard of care for managing IBP remains urgent surgical repair in order to reduce the risk of peritonitis, sepsis and other sequelae of intraperitoneal extravasation of urine Bladder perforations can also occur following minor trauma, often related to alcohol intoxication, and often with a full bladder. Case 4 from our series is such an example and illustrates how even a minor fall can result in a significant bladder injury requiring urgent exploration. Similar cases have been previously reported and there is even a reported case of bladder perforation secondary to a minor fall while dancing ,18.Spontaneous bladder perforations (SBP) in the absence of any form of trauma are very rare. Zhang et al. performed a review of the literature and identified 713 cases of SBP, with a reported incidence of 1/126,000. The commonest causes for SBP were alcohol intoxication (39.2%) and lower urinary tract obstruction (18.3%) Although this is a small retrospective case series with the inherent limitations, it highlights the diverse patient cohort who can present with bladder perforations. It demonstrates that a high index of suspicion is needed when patients present with abdominal pain and difficulty voiding, often in the absence of a classic history of high impact abdominal trauma. It also highlights that while early surgical repair remains the standard of care for intraperitoneal perforations, conservative measures alone can be successful in select patients. It similarly highlights that some cases of extraperitoneal bladder perforation may require surgical exploration.This study was granted institutional review board exemption.The authors confirm that all patients provided both verbal and written consent.


Transjugular intrahepatic portosystemic stent–shunt (TIPSS) is a minimally invasive procedure used for the treatment of portal hypertension. Intrahepatic hematoma is the rare complication caused by hepatic arterial injuries from TIPSS procedure.This case report illustrated a 77-year-old man with hepatitis B virus-induced cirrhosis who underwent TIPSS.The patient suffered from intrahepatic hematoma and hepatic pseudoaneurysm because of the hepatic artery injury after TIPSS.The hepatic artery laceration began at the level of the branch of the left hepatic artery was embolized.The acute intrahepatic hematoma and hepatic pseudoaneurysm of the patient were cured.In this report, we describe a cirrhosis patient with a large intrahepatic hematoma secondary to TIPSS, and a literature review is also presented. The intrahepatic hematoma and hepatic pseudoaneurysm should be paid more attention after TIPSS while early-stage prevention should be carried out. A rare but life-threating complication of this procedure is intrahepatic hematoma caused by hepatic arterial injuries. Treatment options for this condition generally include conservative therapy, transcatheter arterial embolization, and surgery. Thus far, there are no literature review on this rare complication. Herein, we present a cirrhosis patient with a large intrahepatic hematoma secondary to TIPSS procedure treated with transcatheter arterial embolization, and a literature review is also presented.Transjugular intrahepatic portosystemic stent–shunt (TIPSS) has been in use to treat complications of portal hypertension for more than 30 years.9/L, total bilirubin 10.8 umol/L, albumin 26.9 g/L, prothrombin time 12.9s, alanine transaminase (ALT) 21 IU/L, and aspartate transaminase (AST) 46IU/L. A triple-phase computed tomography (CT) scan demonstrated signs of portal hypertension, moderate pleural effusion on right side with a small quantity on the other side. Ascites was managed with diuretics and salt restriction. Drainage of pleural cavity was tried; however, this was unsuccessful.A 77-year-old man with hepatitis B virus-induced cirrhosis was referred to our unit, who presented with melena and shortness of breath. Upon physical examination, his cardiovascular apparatuses were without alterations, the abdomen was flaccid, with a liver 7 cm from the right costal edge. The peritoneocentesis and thoracocentesis examination both revealed that no tumor cell was found. The pleural fluid was sterile and transudate with a serum-fluid albumin gradient > 1.1 g/dL, consistent with hydrothorax. Routine laboratory examination showed hemoglobin 82 g/L, platelets 95 × 10Due to hepatic hydrothorax and secondary prevention for variceal bleeding, a TIPSS implant was agreed on and performed. During TIPSS procedure, TIPSS needle assembly  was advanced into the liver parenchyma under fluoroscopic guidance. A total of 3 passes of the inner needle were made before a central portal vein branch was punctured. No sign of puncture into hepatic arteries, bile ducts, or extrahepatic puncture was seen. A 10 mm × 8 cm bare stent  was placed between the middle hepatic vein and the left branch of the portal vein. By expanding the stent through an 8 mm × 6 cm balloon catheter , the portosystemic pressure gradient between the portal vein and inferior vena cava decreased from 24 to 11 mmHg. The patient presently received a bolus of 2000 U heparin intravenously for prophylaxis of stent thrombosis and was then started on low molecular weight heparin at a 0.4-mL q12h for 3 days.9/L, prothrombin time 13.1 s, activated partial thromboplastin time 46.8 s, total bilirubin 16.6 umol/L, albumin 23.6 g/L, ALT 36 IU/L, and AST 61 IU/L. Abdominal Doppler Ultrasonography showed a 8.6 × 5.4 cm hybrid echogenic mass in porta hepatis with no blood flow  was advanced into the left hepatic artery. The precise margins of the hepatic artery laceration were identified by microcatheter angiography on left anterior oblique. Micro coils  were inserted for embolizing the laceration. Final angiography showed no filling of the pseudoaneurysm Fig. .The clinical course was favorable. The patient’s urine increased from 1200 mL/d up to 2800 mL/d. Two days after embolization management, the hepatic biological tests showed total bilirubin 20.1 umol/L, ALT 29 IU/L, AST 66 IU/L, as the same level of pre-TIPSS level. An abdominal CT scan realized coil emboli to the pseudoaneurysm and gradual resolution of the hematoma.–5 Schweiger et al first reported an intrahepatic hematoma secondary to TIPSS in a Budd-Chiari syndrome (BCS) patient. Since then, only 16 cases have been described in English literatures  have great value in patients suspected with hematoma, In the case reported by Hasegawa et.al, although angiography revealed active bleeding, embolization was not performed because the bleeding point was located in the proximal right hepatic artery, and as the patient showed no antegrade portal venous flow during TIPSS, embolization may result in infarction of the right lobe and hepatic failure occurring. But after conservative treatment, the hematoma gradually resolved and no evidence of pseudoaneurysm formation was found.Patients with intrahepatic hematoma usually have favorable prognosis after treatment. However, as intrahepatic hematoma often causes hemorrhagic shock and is vulnerable to infections, conservative management, including analgesics, antibiotics, fluid replacement, transfusion, and stopping anticoagulation or reducing heparin, if necessary, should be given, as well as reversal of any underlying coagulopathies. Other intervening procedures, including operation, embolization, should be considerate when conservative treatment fail. Embolization may be necessary for pseudoaneurysm and active bleeding, because they usually do not spontaneously resolve and pseudoaneurysm have a tendency to rupture causing death.Intrahepatic hematoma due to TIPSS procedure is rare but potential life-threatening complication. The diagnosis should be considered in any patients with sudden right upper abdominal pain and hemodynamic compromise. Ultrasound and enhanced CT or MRI is the diagnostic modality of choice, which is rapidly attainable and can provide detailed information regarding the injured branch of hepatic artery. If suspecting hepatic artery injuries, including pseudoaneurysm and active bleeding, selective angiography is necessary. Early resuscitation and reversal of coagulopathy is essential, and embolization should be done in patients with pseudoaneurysm or unstable hemodynamic status. Whether it is necessary to embolization or stopping anticoagulation when sign of hepatic arterial injuries is found during TIPSS procedure is need to be further investigated.Conceptualization: Ziyao Cheng, Mingming Deng, Hailong Zhang.Data curation: Ziyao Cheng, Jiayu Ju, Qingliang Zhu, Mingming Deng.Formal analysis: Ziyao Cheng, Jiayu Ju, Qingliang Zhu.Supervision: Hailong Zhang.Writing—original draft: Ziyao Cheng, Hailong Zhang.Writing—review and editing: Ziyao Cheng, Qingliang Zhu, Mingming Deng, Hailong Zhang.


Placental bands on T2-weighted magnetic resonance imaging (MRI) are a known imaging finding in placenta accreta spectrum (PAS). It is believed that these linear T2 hypo-intensities may reflect increased fibrin deposition in the setting of placental hemorrhage or infarct. However, to date there is little published data regarding histopathologic analysis of placental parenchyma at the site of identified bands. We report the case of a 34-year-old female with a single placental band demonstrated on preoperative MRI which was evaluated postoperatively and found to represent a placental infarct. MRI is an important tool in the diagnosis of placenta accreta spectrum (PAS), with key imaging features consisting of heterogeneous placental signal intensity, placental bands on T2-weighted imaging, abnormal placental contour and/or bulging, and direct visualization of myometrial invasion –3. To daA 34-year-old woman, G4P3003, presented at 30-weeks’ gestation for consultation regarding high concern for PAS on prenatal ultrasound. She had a history of one prior cesarean section and 2 prior vaginal deliveries. This was not an IVF pregnancy, and she had no history of additional uterine surgeries or instrumentation. Ultrasound demonstrated placenta previa with multiple placental lacunae, loss of the normal retroplacental hypoechoic space, and increased vascularity with uterine wall invasion noted by color Doppler , highly To further characterize the placenta, non-contrast MRI of the abdomen and pelvis was performed. MRI findings included complete placenta previa, lobulated placental contour, and a prominent T2 hypointense band in the anteroinferior placenta , all conA plan was made to deliver the fetus at 34-weeks’ gestation via cesarean hysterectomy. Bilateral uterine artery embolization was performed by interventional radiology intraoperatively after the cesarean section and before the hysterectomy. Intraoperative findings were consistent with placenta percreta with placenta visualized protruding through to the uterine serosa in the left lower uterine segment with confirmation of bladder invasion. The case had an estimated blood loss of 4500 mL, and she received 5 units of packed red blood cell, 3 units of fresh frozen plasma, and 2 units of cryoprecipitate. Although there was placental invasion into the detrusor, there was no invasion of the bladder mucosa and cystotomy was avoided. The patient progressed well and was discharged home on postoperative day 5. There were no neonatal complications outside of iatrogenic prematurity and both mother and baby were doing well at 6-week postpartum exam.Hysterectomy specimens including the morbidly adherent placenta were sent to pathology following the procedure for formal evaluation of the myometrial-placental interface, as well as the site of the placental band noted on MRI. The placental parenchyma was noted to be dark red brown with focal tan-white discoloration, measuring up to 2 cm. Areas of fibrosis concerning for infarct were noted and can be seen in PAS is typically diagnosed in the second or third trimester of pregnancy via ultrasound imaging, with MRI serving as an adjunctive tool in diagnosis and surgical planning The current standard management for patients with PAS is a planned fundal cesarean hysterectomy at 32-24 weeks, with the surgical approach influenced by the degree of vascular recruitment or structural invasion In summary, this case describes a patient with complete placenta previa and suspected PAS who presented for cesarean hysterectomy. MR imaging demonstrated features of placenta accreta spectrum including the presence of a singular placental band on T2-weighted images, which was found to represent a dense region of fibrous tissue consistent with placental infarct at pathological evaluation. This finding can help build upon the current hypothesis that placental bands represent areas of fibrous tissue due to increased fibrin deposition. Abnormal placentation has been previously associated with placental infarction, which could occur following pathologic development of the uteroplacental vasculature The patient reported in the manuscript signed the informed consent/authorization for participation in research which includes the permission to use data collected in future research projects including presented case details and images used in this manuscript.


Carcinoid syndrome can cause desmoplastic reactions to nearby tissues. When it involves the heart, it causes carcinoid heart disease (CHD) or Hedinger syndrome and usually involves the right-sided heart valves, causing tricuspid insufficiency and pulmonary stenosis (TIPS) and eventually leading to right-sided heart failure. The management of patients with CHD is complex, as both the systemic malignant disease and the heart involvement have to be addressed. Its prompt diagnosis and early treatment is paramount as CHD is associated with increased morbidity and mortality. A 61-year-old Caucasian male with a recently diagnosed metastatic neuroendocrine tumor presented to the heart failure clinic with decompensated heart failure, anasarca, flushing and diarrhea. This case highlights the common clinical features of carcinoid syndrome, its cardiac manifestations and the pathophysiology underlying the manifestations and treatment decisions that involve addressing both systemic and cardiac manifestations. Carcinoid heart disease (CHD) is a manifestation of valvular heart disease occurring in patients with neuroendocrine tumors. The pathophysiology of CHD involves serotonin metabolism as well as a variety of substances secreted by the neuroendocrine tumor itself. Previously, these tumors were known as carcinoid tumors; they are rare malignancies occurring with an incidence of 5.1/100,000 per year, most frequently arising out of the gastrointestinal tract (67.5%), most from small intestine, followed by the pulmonary system 25.3%) [5.3% [2].We report a case of a 61-year-old Caucasian male with no past medical history who was referred by his oncologist to the heart failure clinic after auscultating an abnormal murmur. The patient had been generally healthy and had not sought any medical attention until he developed bright red stool six months ago; he underwent a colonoscopy that was normal. Unfortunately, one month ago prior to the presentation to the clinic, he started developing peripheral edema, which progressively worsened leading to rapid development of anasarca over the course of two weeks. A review of systems was positive for diarrhea, progressive fatigue, flushing and diffuse pruritis. He reported gaining 12 kg over the course of two months. He was unable to perform daily chores, but was able to take a shower by himself. Exertional dyspnea was seen after walking only two blocks; he also had nausea without vomiting and generalized fatigue. He had tried taking oral diuretics for a few days, but had had no improvement. He also reported swelling around his penis and scrotum. His diarrhea was adequately managed with loperamide.Physical examination was remarkable for tachycardia of 101 beats per minute, tachypnea of 20 breaths per minute, with otherwise normal blood pressure and oxygen saturation. The patient was in mild respiratory distress and had been sitting up in bed with the bed elevated at 60°. He had anicteric sclera and no stigmata of cirrhosis. Skin examination revealed a diffusely erythematous blanching rash on his forehead, face and around his chest. His jugular venous pulse was at his earlobe and he had a positive Lancisi's sign. Cardiac examination was remarkable for regular tachycardic rhythm with normal S1 and S2. The patient had a loud holosystolic grade 3 out of 6 murmur at the left lower sternal border that was worsened by inspiration and leg raise, radiating to the axilla. He had no S3 gallops, no friction rub or clicks; lungs were clear to auscultation bilaterally and no rales or wheezes were noted. His abdomen was soft yet protuberant, mildly tender around the right upper quadrant and lower quadrants around the previous biopsy site. He had no hepatomegaly or splenomegaly on examination, and endorsed normoactive bowel sounds. He did have a positive hepatojugular reflux on examination. His extremities were warm to touch with profound tense woody edema up to the torso. Skin examination revealed warm diffuse blanching erythema from head to toe, including palms and soles; the nape of his neck was slightly darker. Neurological examination was nonfocal with grossly intact cranial nerves. The patient had a normal and steady gait with clear and fluent speech.Lab values revealed reactive leukocytosis, synthetic liver dysfunction with elevated liver enzymes and mildly elevated coagulation parameters, consistent with congestive hepatopathy Table . The patPrior to this presentation, he had been generally healthy and had been followed up annually by his primary care physician (PCP); he had never been hospitalized in the past. Given his ongoing diarrhea, his PCP ordered a computed tomography (CT) scan of the abdomen and pelvis that revealed masses to the liver and an enlarged mesenteric lymph node. Follow-up CT with the liver protocol revealed a 12.3 × 9.9 × 9.8 cm arterial enhancing mass in the right hepatic lobe, as well as five smaller masses in the right lobe, one mass in the left lobe, and a 3.3-cm mass in the mesentery in the right mid abdomen  scan revealing a focus of the upregulated somatostatin receptor in the small bowel, suspected to be the primary neuroendocrine tumor Figure . SeveralHedinger syndrome is a unique and unfortunate complication of carcinoid syndrome. More than half of CHD patients have had either a neuroendocrine tumor or carcinoid syndrome. This desmoplastic tumor generally involves the right side of the heart . In factEarly in the course, clinical manifestation can be difficult to diagnose, as patients can be asymptomatic from various degrees of tricuspid and pulmonary valve disease. This is partly due to the low-pressure pulmonary circulation ,7. EarlyAnother important yet rare association with carcinoid syndrome is pellagra, which occurs as a result of niacin deficiency. Endogenous niacin production is depressed, as tumor cells divert the available tryptophan substrate metabolism towards serotonin production. The decreased availability of endogenous and exogenous niacin eventually results in the depressed tissue niacin levels responsible for the development of pellagra, leading to dermatitis, dementia, diarrhea, and even death. Oral or parenteral administration of niacinamide (vitamin B3) may be required for rapid clearing of the pellagrous dermatitis.Treatment is based on the availability of serotonin analogues and on neuroendocrine cell expression of cell membrane serotonin (5-HT) receptors. Tumor debulking methods such as hepatic artery embolization and palliative cytoreductive surgery may improve the symptoms in carcinoid syndrome; they can make symptoms of heart failure better ,12. UnfoHedinger syndrome or carcinoid heart disease is a rare complication of advanced neuroendocrine tumors and is associated with increased morbidity and mortality. The pathophysiology in the development of CHD remains complex, but serotonin plays a pivotal role in the pathological process of valve destruction and dysfunction. Early recognition and surgical interventions, even before advanced heart failure consultation has occurred, may improve the outcomes in these patients. Our understanding of the molecular mechanism underlying the disease has increased significantly over the last decade, and pharmacotherapy designed for targeting the fibrosis in CHD may lead to the development of appropriate novel targets for the desmoplastic effect of the tumor. Patients should be managed in specialized centers by a multidisciplinary team including oncologists, advanced heart failure specialists, and cardiothoracic surgeons with experience in the treatment of this condition. Results of ongoing clinical trials may reveal further improvements in diagnosis and treatment.


Hemichorea is a unilateral movement disorder caused by acute ischemic or hemorrhagic stroke of contralateral cerebral lesions. It is followed by hyperglycemia, and other systemic diseases. Several cases of recurrent hemichorea associated with the same etiology have been reported, but cases with different etiologies have rarely been reported. We report a case in which the patient experienced both strokes and post-stroke-related hyperglycemic hemichorea. Magnetic resonance imaging of the brain appeared different in these two episodes. Our case demonstrates the importance of evaluating every patient presented with recurrent hemichorea carefully, as the disorder may be caused by different conditions. The unilateral movement disorder, hemichorea, commonly stems from acute ischemic or hemorrhagic stroke of contralateral cerebral lesions and is typically followed by hyperglycemia and other systemic diseases, such as hypoglycemia, uremia and anti-phospholipid antibody syndrome . SeveralA 58-year-old man with a history of type 2 diabetes for 12 years was presented at our hospital 4 years ago. Because of poor compliance, the patient has not been able to achieve the target glycemic treatment goal during the past 4 years. We observed two episodes of right hemichorea resulting in similar involuntary movements but in different brain MRI images and etiologies.The first episode of right hemichorea brought him to our emergency room 2 weeks after a fall. Hyperglycemia (712 mg/dl) was also noted at that time. However, a computed tomography (CT) scan of the brain disclosed no specific findings. He was admitted and an MRI scan of the brain showed an old lacunar infarct at the left basal ganglion . His rigAfter 4 years of follow-ups in the outpatient department, the patient’s compliance was still poor. He was hospitalized because of involuntary movement in his right upper limb and right jaw for 2 weeks. The involuntary movement subsided when he fell asleep. The neurological examination showed that muscle power and sensation of bilateral limbs were intact. His blood sugar was high (671 mg/dl) without ketoacidosis. The serum creatinine level was normal, and the HbA1c was 17.3%. A CT scan of brain showed increased density in the left basal ganglia . FurtherWe reported a case of recurrent hemichorea in a 58-year-old man exhibiting different etiologies demonstrated by specific brain MRI findings. The most common cause of hemichorea is acute ischemic or hemorrhagic stroke, and the second is hyperglycemic hemichorea. Other causes including hypoglycemia, Sydenham’s disease, uremia and anti-phospholipid antibody syndrome have also been reported. These different etiologies of hemichorea result in different brain imaging appearance . TypicalIn addition, hyperglycemic hemichorea is a rare neurologic complication of poorly controlled diabetes, which was first described by Bedwell in 1960 . Most ca


Urethral duplication is a rare anomaly observed in veterinary medicine. The surgical techniques described therein are associated with an uneventful recovery. The authors describe a major surgical complication after the correction of urethral duplication in a 2-year-old male Yorkshire terrier. After surgical correction using the perineal approach, the patient developed pollakiuria and urinary retention due to a valve effect caused by the remnant of the dorsal opening of the ectopic urethra. A second procedure, using an abdominopelvic approach, successfully corrected the complication by intraluminal correction of the dorsal urethral wall. An intact 2-year-old male Yorkshire, weighting 2.8 kg, presented with a history of aberrant urination and recurrent urinary tract infection. During micturition, the same volume of urine came simultaneously from the anus and urethra.Clinical examination and visualization of the opening at the level of the anus ventrally revealed urine leakage from the anus . InguinaSurgery was planned, which included contrast urethrocystography  prior to the intervention  and explPrior to anesthetic induction, the dog was premedicated with methadone   and acepromazine  , followed by anesthetic induction with midazolam   and propofol  . The dog was intubated, and the anesthesia was maintained with isoflurane and oxygen. Lactate Ringer's solution was administered at a rate of 5 ml/kg/h. The dog received cefazoline   at induction, and every 90 min during surgery, and robenacoxib  . The surgical technique was performed using the perineal approach described by Ralphs and Kramek . At thisTwo days after surgery, the dog presented to the hospital with pollakiuria and urinary retention that started after the surgical intervention, as reported by the owners. There were signs of partial obstruction- a full and tense bladder, pain on palpation, and non-competent urinary leakage. No relief was obtained after administration of buprenorphine. A urinary probe easily passed through the urethra, and this led to immediate relief of obstruction. Retrograde urethrocystography was performed once again, which revealed an apparently normal-sized urethra  and no signs of contrast leakage . The dogPostoperative recovery was uneventful, without recurrence of clinical signs. Six months after the latest surgical intervention, the patient presented with pollakiuria and urinary incontinence. Urine culture with antibiotic sensitivity testing was performed, and Enterococcus faecium was identified, which was sensitive only to sulfamethoxazole and trimethoprim. Antibiotic therapy was initiated with this combination  15 mg/kg BW, PO, q12 h for 15 days, and phenylpropanolamine  1.2 mg/kg BW, PO, q12 h. The dog continued to show signs of occasional uncontrolled urinary micturition associated with stress. Therefore, the frequency of phenylpropanolamine administration was corrected to q8 h and maintained for 2 months. Urethropexy was proposed; however, it was refused by the owner. Eight months later, the owner reported an improvement in the clinical signs of the dog. Uncontrolled urinary micturition related to stress occurred, although less frequently.Relevant complications associated with the surgical correction of urethral duplication must be reported as they can contribute to a better management of future cases.This rare congenital condition has likely multifactorial etiology, mostly found in males , 4. DiffUrethral duplication can either be asymptomatic or present with several signs, including inflammation, incontinence, urinary obstruction, recurrent urinary infection, and double urinary stream , 9. MostAlthough surgery of type IIA2 urethral duplication has been previously reported in dogs , 13, 14 The surgical options reported in human surgery literature include reconstruction or excision. Reconstruction includes the use of mucosal grafts or local tissue with mobilization of the ectopic urethra by perineal and/or transpubic approaches , 15, 16.The theory proposed by the authors of a valve effect causing outflow obstruction is also supported by other observations. First, there were no resistance while placing the urethral catheter. In this case we decided not to leave a urinary catheter in the post operatory period for two reasons: no expected obstruction/reduction of the luminal diameter and its presence could promote inflammation . Second,The stress induced incontinence observed at the long term could be related with several factors. First, the urinary infection. This event could be a result of an undiagnosed urinary colonization, which evolved to infection slowly, or could be a new event. Treatment of the infection contributed to attenuation of the stress induced incontinence. Second, an incompetent orthotopic urethra or a possible hormone responsive incontinence are other causes to consider. The latest cause usually leads to micturition during sleep or rest , the oppThis case report suggests that it is crucial to evaluate the type of duplication and functions of both urethras to choose the most appropriate surgical technique and avoid complications. In this case, the perineal approach described previously  was not The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.Ethical review and approval was not required for the study of animals in accordance with the local legislation and institutional requirements. Written informed consent was obtained from the owner of the animal involved in this case report.JM collected all clinical data and wrote the initial draft. RR, LI, AR, and LM performed the diagnostics and all medical and surgical procedures. All authors contributed to the writing of the manuscript, after discussion of the results, revised the manuscript, contributed equally to the final version, and agreed to the copyright conditions.This work was funded by FCT—Fundação para a Ciência e Tecnologia, grant UIDB/00276 /2020, from CIISA—Centro de Investigação Interdisciplinar de Sanidade Animal, Faculdade de Medicina Veterinária, Universidade de Lisboa, and LA/P/0059/2020 - AL4AnimalS, Portugal.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Hemangiomas are benign tumours, mostly seen in the soft tissues. The intraosseous appearance is rare, in particular in the jaws they represent a very seldom malformation.To present a combined endodontic and surgical management report of a clinical case with a rare intraosseous hemangioma diagnosis in the mandible.This well-documented case report describes the interdisciplinary treatment approach of an intraosseous hemangioma in the left mandible of a 70-year-old male patient. This incidental finding was detected through a routine dental examination. The panoramic radiograph revealed an asymptomatic, apical translucency approximately 15 mm diameter with contact to the mesial root of the tooth 36. The clinical examinations showed no abnormalities. The multifaceted specialized treatments started with the endodontic treatment of the tooth prior to the surgical removal of the lesion and were followed by the histological assessment. As derived from the histologically verified diagnosis, this rare case included the risk of severe bleeding complications during therapy. Vascular anomalies are differentiated into two groups: hemangiomas and vascular malformations. Hemangiomas are not congenital, women are affected twice as often as men, and they show endothelial hyperplasia . The worA 70-year old Caucasian male patient presented for the first time at the department of Operative Dentistry, Periodontology and Endodontology for routine dental control examination in November 2017. He was pain-free, had no history of trauma in the craniofacial area and was otherwise in a good to very good state of health, American Society of Anesthesiologists Physical Status Classification (ASA) . Within During the following conversation on the radiographic finding, the patient reported that his previous long-time family dentist had already addressed this finding ten years ago. Since he assumed this osteolytic process to be an aneurysmatic bony cyst, he informed the patient that there was no treatment need. In order to receive details regarding the progression and growth of the- at that point unknown- osteolytic process, the patient was asked to provide old panoramic radiographs from the family dentist. At that appointment, sensibility of the mental nerve was tested via discrimination between sharp and blunt and two-point discrimination. The patient was able to identify the applied stimuli correctly in 100 %. The pulp sensibility of the teeth 34, 35, 36, 37 and 38 was also tested and recorded positive for the teeth tested. Indeed, at the second appointment, a panoramic radiograph made in 2011 was submitted by the patient. Here, in general, the osteolytic process looked smaller and, interestingly, the mesial root of tooth 36 did not seem to be in contact to it  of the left mandible was made providing important details on the topographic relationship of the radiographic lesion and other anatomic structures, such as the inferior alveolar nerve Fig. a, b. In Based on the current CBCT, a decision was made to take a precautionary surgical tissue sample in order to obtain a distinct histopathologically verified diagnosis. Because of the close positional relationship of the intrabony lesion and the roots of tooth 36 with the resulting high risk of devitalization of this tooth, an endodontic treatment was performed before the surgical procedure. Pre-surgical endodontic treatment offers the possibility to remove potentially extruded sealer or obturation material that could potentially irritate the inferior alveolar nerve, during following surgery. Thereby, a second surgery could be avoided. The patient accepted the recommended therapy and oral and written informed consent was obtained for the endodontic-surgical-intervention.2 of the teeth in the third quadrant revealed a reliable positive sensibility of teeth 34, 35, 36, 37 and 38. Although the CO2 sensibility test, in particular, of tooth 36 was positive, the intra-oral periapical radiographic examination  revealed a topographical relationship of the mesial root of this tooth to the intrabony lesion , the root canal orifices were sealed with opaque white composite resin  and the remaining access cavity was filled with Filtek Supreme XTE composite resin . IAPOR after the root canal filling revealed a well-tapered and sufficient root canal filling with a minimal extrusion of sealer in the mesial periapical tissues  were used to carefully prepare the lesion in the posterior region. It was possible to detach the mass off the bony wall. In combination with gentle swinging and shaking movements the inferior alveolar nerve could be depicted in the posterior region. Integrity of the nerve could be controlled via mild pulling on the nerve covering tissue and observing the little movements outside the mental foramen. The same procedure was performed in the anterior part of the bony cavity until the nerve was depicted here also. The strong adherence of the nerve enclosing tissue was detached by spreading the mass with tweezers and blunt preparation with a scissor precisely to the border of the nerve sheath, beginning from the edges to the centre. Finally, both structures could be separated, whereupon the nerve was exposed in the middle of the bony cavity without any conspicuous damages or alterations of the nerve sheath  via infiltration anaesthesia. The design of the full thickness flap was as minimally-invasive as possible. The papilla 34 to 35 was detached, marginal incision was made along 35 to 36. Due to the close anterior positional relationship of the mental foramen to the space-consuming lesion, the decision was reached to perform a distal vertical relief incision, which was directly distal from the papilla 36 to 37. The mucoperiosteal flap was elevated with a raspatory, followed by blunt preparation with a gauze pad up to 1 cm near the expected mental foramen. Thereby, the nerve was gently exposed and could be protected by the use of a narrow, blunt retractor. The bony access was chosen in a more distal position above the suspected space-occupying lesion, and initially prepared with a rose head bur followed by a diamond bur after the color of the bone changed to darker. When the covering bone, which was consecutively put into 4 % formalin and turned in for histopathological examination, was completely removed, the dark red color of the lesion appeared. Surprisingly, the consistence of the lesion seemed not to be firm elastic as expected for an anticipated cystic lesion. In the contrary, the lesion appeared very solid, it was hardly possible to achieve a slight impression. At this point, it was clear that the originally planned surgical intervention could not be an ordinary biopsy. Moreover, due to the consistence and topographic location of the tissue in the lesion, it appeared also obligate to avoid an incision biopsy to minimize any risk for the alveolar nerve. The risk of an axonal injury was too high, because of the obscure position of the mental nerve. Therefore, the original plan to take a surgical probe excision, was neglected and in lieu thereof the entire removal of the tissue was intended. Thus, different sinus-lifting elevators (FriosThe intraoperative tissue samples of the space-occupying lesion were processed and evaluated at the Department of Pathology. The tissue exhibited large, irregular, dilated blood vessels filled with erythrocytes, and surrounded by a fibrous stroma Fig. a. At higAs early as in the evening after surgery the patient reported that sensibility in the lower lip was subjectively recovered. Wound healing period was completely uneventful and without any discomfort. Twelve days after surgical treatment, no signs of inflammation such as swelling could be detected so sutures were removed. Recall intervals were scheduled at every six weeks for the first seven months. Sensibility testing of teeth 33, 34 as well as 35 were positive and provocation via percussion on the teeth in the left mandible was unobtrusive within the whole recall period.Seven months after surgical procedure, an intact bone contour in the region of the former bony window was clinically palpable. Up to that point, the patient reported a slight numbness in the left mental dermal region, but no pain at all. The objective verification did not show any sort of numbness. Both, discrimination between sharp and blunt object as well as two-point discrimination were reproducible at 100%. In addition, slightest contacts in the left mental dermal region were realized by the patient without any difference to the corresponding right areas. The operation field itself was completely inconspicuous and the mucosa revealed no scars Fig. a. A partThe twelve months post-excision control demonstrated a further improvement with respect to the subjective dermal sensibility in the mental region, whereas in the patient’s opinion it was not yet equal to the contralateral side. Similar to the seven-month control, the patient precisely realized the objective verification measured with stump vs. sharp testing. The further panoramic radiograph twelve months postoperative presents a similar impression of the region of interest as the post- seven-months radiograph. A remarkable inhomogeneous reorganisation of the bony defect is observable including an increase of radio-opacity. Forty-five months after surgery, a panoramic radiograph of the left mandible was performed. No signs of recurrence could be observed. The former lesion showed increased radiopacity while the border of the lesion decreased and got more diffuse Fig. c.Moreover, the clinical situation appeared unobtrusive. Stable and unaltered sharp and blunt discrimination as well as two-point discrimination were reproducible in 100%. Slightest touches were also perceived. No signs of swelling or discoloration could be determined. Table Keeping in mind the last forty five months, the patient summarizes the entire interdisciplinary endodontic and surgical intervention including the follow-up examinations to be successful. Apart from the complication-free and scarless wound healing, the patient subjectively reports of a discrete irritation during shaving within the left mental region.The present case illustrates a rare example of an intraosseous hemangioma, which has been well-documented with respect to its interdisciplinary treatment and its follow-up history. In the current case, the diagnostic finding was an incidental finding after fabrication of a panoramic x-ray. In 2007, the patient firstly attained knowledge of an intrabony osteolytic process in the left mandible. At that time point as well as ten years later, both panoramic radiographs showed a homogeneous, unilocular, well-defined osteolytic lesion. This is in contrast to the radiological appearance of most of the intraosseous hemangiomas, which is described as soap bubble, honeycomb-like appearance, a sunray appearance, caused by bony trabeculae in the lesion or a poorly defined osteolytic lesion , 15. TheDentists, oral surgeons and maxillofacial surgeons have to be aware of clinical symptoms and radiological appearance of intraosseous hemangiomas to avoid severe complications even though these lesions occur very seldom in the jaws. Gentle and minimally invasive surgery protects neighbouring structures like teeth or nerves completely. Moreover, for the conservative dentist, this case report impressively supports the fabrication of a panoramic x-ray in addition to bite wings being made for the assessment of carious lesions during the first appointment of a new patient. As seen in the present case report, without doing so, the hemangioma would have been overlooked, perhaps resulting in serious consequences for the patient.


Cholecysto-antral fistula and gallstone ileus are rare complications of a common disease, gallbladder stone (GBS). This fistula is developed as a prolonged complication of cholelithiasis in which the gallbladder adheres to the adjacent antrum, and a stone erodes through the wall. Among the variety of cholecystoenteric fistulae, the cholecystoduodenal fistula occurs more commonly than the cholesysto-antral fistula.In this scientific study, we present a 98-year-old male patient who came to ER with a complaint of abdominal pain, vomiting, and constipation for five days. He was vitally stable and had normal laboratory results. The plain abdominal X-ray showed dilated loops with excessive gases. His computed tomography (CT) abdomen with contrast showed small bowel obstruction secondary to an impacted gallstone at the distal jejunum, fistulous communication between the gall bladder and the antrum, and pneumobilia.Our management included endoscopic retrieval of a single gallstone from the second part of the duodenum followed by open surgical enterolithotomy, partial cholecystectomy, and closing of the fistula. Despite our case sharing many aspects with the available literature, our case, to our knowledge, is the first case of ileus gallstone occurring in a 98-year-old patient. Cholecysto-antral fistula has not been widely published in the literature. The offending gallstone presented along with the radiological Mercedes Benz sign which does not present in all cases of GBS. Typically, the obstructing GBS stops at the terminal ileum, but in our case, it was dislodged in the distal jejunum with no previous biliary symptoms. Finally, we were able to remove another single GBS from the second part of the duodenum during the preoperative upper endoscopy. The clinical diagnosis may be missed due to the vague presentation of symptoms; hence imaging, especially of the CT abdomen is crucial in establishing the diagnosis, moreover, performing an upper endoscopy could have diagnostic and therapeutic benefits. In cases like this, the main surgical intervention should be to address the bowel obstruction, and cholecystectomy with fistula closure may be added if the patient’s condition is stable with minimal inflammation and adhesion. Gastro-antral fistula and gallstone ileus are rare complications of gallbladder stone (GBS) disease, which can lead to small bowel obstruction (SBO) secondary to the impaction of the GBS in the small bowel, typically in the terminal ileum. Usually, a large stone erodes through the gallbladder into the contiguous bowel, most frequently the duodenum, creating a cholecysto-enteric fistula (CEF) . ThroughA 98-year-old male presented to the emergency department of the Security Forces Hospital Program, Dammam with a five-day history of colicky periumbilical abdominal pain associated with non-bilious, non-blood-mixed vomiting and obstipation. He had no significant past medical or surgical history, and he was a nonsmoker. On examination, his vitals were as follows: blood pressure of 110/80, pulse rate of 86/minute, temperature of 37.1 Celsius, respiration rate of 18/minute, and SPO2 of 98% room air. He was not jaundiced and his abdomen was soft and lax with mild tenderness in the right hypochondrium on deep palpation. Routine blood tests were significant only for mild leukocytosis. The abdominal X-ray (AXR) showed dilated loops with excessive gases Figure , SBO at An upper gastrointestinal endoscopy was done and showed competent cardia with bilious greenish secretions filling the stomach, normal gastric mucosa, and pylorus Figure . The finOur patient was taken to the operating room for an open exploratory laparotomy. Intraoperatively, a 3 cm GBS stone was found obstructing the distal jejunum Figure . Our opeCEF was first described by Courvoisier in 1890 . It usuaIn the literature, the CEF may present with a variety of symptoms: diarrhoea, abdominal pain, distention, jaundice, fever, sepsis, nausea, vomiting, SBO, steatorrhea, and weight loss . The majBoth our study and literature share several key points: signs and symptoms of bowel obstruction, CT findings suggesting the diagnosis, and the surgical approach and management. On the other hand, we believe that our case is unique due to many aspects: firstly, to the best of our knowledge, this is the first reported and published case of ileus gallstone performed on a 98-year-old patient. Secondly, cases of biliary duodenal fistulae are not frequently discussed in the literature. Thirdly, the obstructing GS, in our case, presented the radiological Mercedes Benz sign which does not present in all cases of GBS. Fourthly, in our case, the migrating GBS was dislodged in the distal jejunum while typically, it is dislodged in the terminal ileum. Fifthly, our patient did not have previous biliary symptoms. Finally, we were able to remove another single GBS from the second part of the duodenum during the preoperative upper endoscopy. Therefore, the clinical suspicion of CEF and gallstone ileus should be present in mind in all adult patients who present with signs and symptoms of bowel obstruction even if there is no history of GBS. The priority in surgical management should be given to addressing the bowel obstruction with or without cholecystectomy and closure of the fistula according to the patient’s status and local anatomy. In the case of cholecysto-antral fistula, the preoperative upper endoscopy may be of diagnostic and therapeutic value.Cholecysto-antral fistula and gallstone ileus are infrequent but challenging complications of gallbladder stones and should be included in the differential diagnosis of bowel obstruction, especially in elderly patients with no history of gallbladder stones. The cornerstone of treatment is the proper diagnosis and timely surgical intervention to release the obstruction by enterotomy and extraction of the stones with or without cholecystectomy, and repair of the fistula according to the patient’s condition and surgical anatomy.


Ewing’s sarcoma (ES) within the genitourinary tract are relatively unheard of and those within the external male genitalia are even rarer. To our knowledge, this is the first known case of primary ES within the paratesticular region in an adult.We present a case of a 24-year-old man with a right sided testicular mass on examination that was initially characterized as an adenomatoid tumor on ultrasound. After the patient was lost to follow up over the course of 9 months, the testicular mass grew significantly and was excised with pathology revealing primary paratesticular Ewing’s sarcoma. This rare case emphasizes the importance of elucidating between the broad differentials of paratesticular masses, including the rare presentation of primary ES and adds a review of the literature of ES in the external male genitalia.Rare differentials such as this case should be considered in patients with paratesticular masses. Further diagnostic and management algorithms for extraosseous Ewing Sarcoma, particularly in the adult population, are warranted. Primary paratesticular malignancies are rare, and sarcoma paratesticular malignancies are even more so. The paratesticular region contains the spermatic cord, testicular tunica layers, and the epididymis. Sarcomas of the genitourinary origin are exceedingly rare, and constitute approximately 2.1% of soft tissue sarcomas and 1–2% of malignant genitourinary tumors . Other rA 24-year-old Hispanic male with no prior medical or surgical history presented with a right-sided testicular mass and pain for 3 months. Upon presentation, the patient endorsed continued right-sided testicular pain and increased size of the testicular mass. Physical exam revealed an uncircumcised penis, normal phallus, bilateral descended testes with a large right testicular soft, tender, and mobile mass. Testicular ultrasound at this time showed an extratesticular 4 cm (cm) mass that, per radiologic interpretation, resembled an adenomatoid tumor. He was scheduled for surgery but lost to follow-up. Nine months later, he presented with continued pain and increased size of the right hemiscrotum with separately palpated and larger extratesticular mass on physical exam and ultrasound Fig. . Tumor mIntraoperatively, an inguinoscrotal incision was used to deliver the testicle within the spermatic fascia and a firm 8 cm paratesticular mass was palpated and concerning for malignancy. The testicle was indiscernible from the mass. A radical orchiectomy was performed.Final histopathology revealed a 7.3 cm × 6.5 cm × 6.0 cm paratesticular mass arising from the parietal tunica vaginalis of the testicle and extending into the visceral tunica vaginalis and epididymis Fig. . The tumDue to the clinically silent nature of paratesticular tumors before a noticeable mass effect, it is challenging to diagnose them. Differentials for such masses include benign lesions such as lipomas, hemangiomas, lymphangiomas, leiomyomas  and adenScrotal sarcomas are rare and are commonly liposarcomas, rhabdomyosarcomas, or leiomyosarcomas . It has Presentations of ES in the male external genitalia are rare, and a review of the literature reveals one reported case of ES within the scrotum but in a 3-year-old-boy with a 2-year history of painless growing mass . The patOn our review of the literature, other primary ES of the male external genitalia has been reported in the penis at least four times. One such case in the penis resulted in a total penile amputation in a young 32-year-old man who subsequently underwent 8 cycles of chemotherapy according to the EuroEwing 99 protocol . The patEwing’s Sarcoma is a cancer most commonly found in children, and much of its management in adults is modeled from pediatric protocols . Due to Although not the focus of this discussion, it is worthwhile to note that several cases of primary Ewing’s sarcoma in both children and adults within the bladder, ureter and kidney have been reported and treated with a combination of surgical resection, chemotherapy and radiation –23.In conclusion our case is not only the first known report of paratesticular Ewing Sarcoma in an adult, but it also represents a unique case of an initial apparent adenomatoid tumor on ultrasound that was later found to be primary ES. Although there are several reports of ES in the genitourinary system, there is ultimately one known report of ES in the scrotum and a handful of cases of penile ES. Rare diseases such as this case of a paratesticular Ewing sarcoma in an adult warrant consideration of unique differential diagnoses for scrotal masses. This disease appears to be treated best with surgical resection and adjuvant chemotherapy; however, despite treatment, prognosis is often dismal once metastatic disease develops. Diagnosis and treatment of cases in the adult population is often challenging. Current treatment post-surgical resection in pediatric and adult cases involves adjuvant chemotherapy and possible radiation, according to ES protocols per the National Comprehensive Cancer Network. Future investigation of appropriate diagnostic and management algorithms for extraosseous ES may be considered.