Merkel cell carcinoma (MCC) is a rare neuroendocrine dermal malignancy seen in elderly light-skinned individuals, associated with immunosuppression and Merkel cell polyomavirus infection. As a neuroendocrine tumor, the recurrence and metastasis of MCC can be evaluated using positron emission tomography-computed tomography (PET-CT) with the Gallium-68-DOTATATE (Ga-68-DOTATATE) radiotracer, which has demonstrated increased sensitivity to neuroendocrine metastases when compared to F-18 fluorodeoxyglucose (FDG). Here, we present the case of a patient with known metastatic MCC with a new, abnormal focus of increased radiotracer activity in the thoracic spine on Ga-68-DOTATATE PET-CT suspected to represent a metastatic lesion. Further evaluation with MRI revealed a benign vertebral hemangioma, highlighting the limitations of this radiotracer in the setting of benign spinal lesions. Multimodality imaging findings of metastatic MCC and potential pitfalls of Ga-68-DOTATATE PET-CT staging are discussed. Merkel cell carcinoma (MCC) is a rare, deadly neuroendocrine tumor of the dermis, with 95% of cases reported in the elderly Caucasian population -3. AdditAs with most dermatologic malignancies, a comprehensive physical examination with a biopsy of the lesions typically yields samples for definitive histopathologic diagnosis. Radiologic imaging may be performed in atypical cases for cancer staging and to monitor for recurrence. A variety of radiotracers have been developed for the imaging of neuroendocrine tumors using positron emission tomography-computed tomography (PET-CT), including F-18 fluorodeoxyglucose (FDG), Gallium-68-DOTATATE (Ga-68-DOTATATE), and Gallium-68-pentixiafor (Ga-68-pentixiafor), with varying sensitivity and specificity ,6.The case presented here uniquely highlights the use of Ga-68-DOTATATE in a patient with known metastatic MCC to illustrate the clinical utility of this radiotracer, as well as the potential pitfalls during evaluation for disease recurrence. Multimodality imaging findings are discussed.Initial presentationAn 84-year-old Peruvian male with a history of hypertension, coronary artery disease, and atrial fibrillation presented for the evaluation of an enlarging lump on the posterior left upper arm. He initially noticed a painless, erythematous nodule which rapidly increased in size over a period of two months while out of the country. The patient also reported feeling a new lump in the left axilla. On physical examination, the patient’s vital signs were within normal limits. A large erythematous mass was noted at the posteromedial aspect of the left upper extremity, overlying the triceps. An additional palpable mass was identified in the left axilla. No cervical or supraclavicular lymphadenopathy was present.Initial imagingTargeted ultrasound of the left upper extremity and axilla with color Doppler was performed. The study revealed an irregularly marginated, lobulated solid hypoechoic mass measuring up to 5.2 cm in the superficial soft tissues of the left upper arm with increased color Doppler flow Figure , raisingA magnetic resonance imaging (MRI) study with a gadolinium-based contrast agent of the left upper extremity and axilla was then performed. Coronal post-contrast T1-weighted imaging with fat suppression of the left upper extremity Figure  demonstrBiopsy and pathologyUltrasound-guided core needle biopsy of the left upper extremity and axillary masses was performed. Histopathologic analysis of the sample tissues revealed a round cell tumor with focal necrosis and high nuclear/cytoplasmic ratio staining positive for CK20, chromogranin, and synaptophysin, consistent with Merkel cell carcinoma.Initial staging and treatmentA Ga-68-DOTATATE PET-CT was subsequently performed for staging purposes. Increased radiotracer uptake was demonstrated in the left upper extremity and axilla on the maximum intensity projection (MIP) image Figure , corresp2, and atezolizumab 1,200 mg/20 mL chemotherapy and immunotherapy. This was followed by targeted external-beam X-ray radiation therapy to the left upper arm and axillary region with 25 fractions of 200 cGy for a total of 5,000 cGy.Given the extent of left axillary lymph node spread, the patient was treated with four cycles of intravenous carboplatin (dose adjusted for AUC 5), etoposide 100 mg/mRestaging and MRIA repeat Ga-68-DOTATATE PET-CT was performed three months following treatment. There was complete interval resolution of the left upper arm and axillary masses with no residual radiotracer uptake in the left upper extremity or axilla. The MIP Figure , sagittaA subsequent MRI of the thoracic spine with a gadolinium-based contrast agent was performed. A well-circumscribed lesion in the T6 vertebral body demonstrating hyperintense signal on both sagittal T1-fluid-attenuated inversion recovery Figure  and shorMCC is a rare, aggressive, and deadly dermatologic malignancy of neuroendocrine origin typically seen in the elderly Caucasian population -3. AlthoMCC is characterized by a painless and rapidly growing erythematous skin nodule, as was described in the patient presented here. Diagnosis is confirmed with biopsy as tissue samples demonstrate intermediate, small-cell, or trabecular subtype histopathological characteristics along with CK20, synaptophysin, and chromogranin positivity ,8.Radiologic imaging of MCC is non-specific, demonstrating hypervascular lobulated soft tissue masses in the superficial soft tissues, as seen on the ultrasound and MRI findings presented here. Nuclear imaging, however, serves as a valuable tool for evaluating the extent of disease and disease recurrence. Ga-68-DOTATATE has emerged as a key radiotracer with a reported 97% sensitivity and 95.1% specificity for neuroendocrine tumors owing to its properties as a somatostatin receptor analog [In this patient, a new focus of increased radiotracer uptake was identified in the T6 vertebral body on the post-treatment restaging Ga-68-DOTATATE PET-CT, raising concern for metastasis. However, subsequent contrast-enhanced MRI revealed a well-circumscribed, non-enhancing intraosseous lesion corresponding to the suspicious focus with both intrinsic T1 and T2 hyperintense signal. These findings are most consistent with benign vertebral hemangiomas which, although classically enhance following contrast administration, may show variable enhancement patterns .Multiple benign spinal pathologies such as hemangiomas, meningiomas, and Schmorl’s nodes have been reported to demonstrate increased Ga-68-DOTATATE uptake ,11,12. IThese findings are an important pitfall of the use of Ga-68-DOTATATE in the staging of MCC, as increased radiotracer uptake on PET imaging can raise concern for metastatic disease. Further characterization of such foci in the spine should be performed with contrast-enhanced MRI. Reassuring imaging findings suggesting a benign hemangioma include intrinsic hyperintense T1 and T2 signal, well-circumscribed margins, absence of extraosseous extension, and minimal to no enhancement on post-contrast sequences . Recent MCC is a highly aggressive neuroendocrine malignancy of the dermis which typically presents as a rapidly enlarging erythematous nodule. Diagnosis is made with physical examination and biopsy of the skin lesion with histopathological analysis. Radiologic imaging can also be performed, primarily serving as a tool in the staging and management of this rare entity.Cancer staging and evaluation for recurrence can be performed with Ga-68-DOTATATE, a somatostatin receptor analog that demonstrates high sensitivity and specificity for neuroendocrine tumors. The case described here illustrates the utility of Ga-68-DOTATATE in staging MCC, as well as an important pitfall for clinicians to consider in the setting of benign vertebral hemangiomas which can mimic metastasis.


Adenomyoma is a rare reactive, hamartomatous benign tumor-like lesion. Although adenomyoma can occur anywhere in the gastrointestinal tract, including the gallbladder, stomach, duodenum, and jejunum, it is very rarely observed in the extrahepatic bile duct and ampulla of Vater (AOV). The preoperative accurate diagnosis of adenomyoma of the Vaterian system, including the AOV and common bile duct, is significant to appropriate patient management. However, discriminating between benign and malignancy is highly challenging. Patients are frequently mistaken as having periampullary malignancy, thereby leading to unnecessary extensive surgical resection with a high risk of complications.A 47-year-old woman visited a local hospital owing to epigastric and right upper-quadrant abdominal pain for 2 days.Abdominal ultrasonography performed in the local hospital revealed suspicious of a distal common bile duct malignancy. She was transferred to our hospital for further evaluation and management.After consulting with the patient, a multidisciplinary team, including a gastroenterologist, finally decided to perform surgery under the impression of an ampullary malignancy, and pylorus-preserving pancreatoduodenectomy was performed without any complications. She was histopathologically diagnosed with an adenomyoma of the AOV.At the 5-year follow-up, she was well and did not develop further symptoms or complications.Although adenomyoma is very rare, it should be included in the differential diagnosis of mass-like lesions of the AOV to avoid unnecessary surgeries. However, discriminating between benign and malignancy is highly challenging. Patients are frequently mistaken as having periampullary malignancy, thereby leading to unnecessarily extensive surgical resection with a high risk of complications. We report a 47-year-old woman with adenomyoma of the AOV who was treated with pylorus-preserving pancreatoduodenectomy without any complications.Adenomyoma is a rare reactive, hamartomatous benign tumor-like lesion.A 47-year-old woman visited a local hospital owing to epigastric and right upper quadrant (RUQ) abdominal pain for 2 days. Abdominal ultrasonography performed in the local hospital revealed intrahepatic and extrahepatic bile duct dilatation suspicious of a distal CBD malignancy. She was transferred to our hospital for further evaluation and management. She underwent cholecystectomy due to gallstone 20 years ago. Her personal and family medical histories were unremarkable. She complained of epigastric and RUQ abdominal pain. The following were her vital signs at the time of arrival: blood pressure, 117/71 mm Hg; heart rate, 87 beats/min; respiratory rate, 16 cycles/min; and body temperature, 37.4℃. On physical examination, mild icteric sclera and epigastric and RUQ abdominal tenderness were noted. Initial laboratory findings showed normal values for her white blood cell count, hemoglobin, platelet, and erythrocyte sedimentation rate, except an elevated C-reactive protein level of 2.23 mg/dL. The following were her serum liver enzyme levels: aspartate transaminase, 95 U/L; alanine transaminase, 165 U/L; total bilirubin, 5.83 mg/dL; conjugated bilirubin, 4.7 mg/dL; alkaline phosphatase, 317 U/L; and γ-glutamyltransferase, 650 U/L. Serum amylase and lipase levels were 24 and 181 U/L, respectively. The carcinoembryonic antigen level was 1.3 ng/mL, and the CA 19-9 level was increased to 97.6 U/mL. Abdominal ultrasonography revealed mild intrahepatic and extrahepatic bile duct dilatation with diffuse wall thickening and smooth distal CBD tapering. Furthermore, abdominal computed tomography revealed mild extrahepatic bile duct dilatation with diffuse wall thickening and enhancement (Fig. ,2 Although it can occur anywhere in the gastrointestinal tract, including the gallbladder, stomach, duodenum, and jejunum, it is very rarely observed in the extrahepatic bile duct and AOV.–5 Hammarström et al reported that of 3131 ERCP cases, 4 patients were diagnosed with ampullary adenomyoma, and the incidence of adenomyoma of the AOV was 0.13%. Women have a 3-fold higher prevalence of adenomyoma than men, with a median patient age of 67 years.Adenomyoma is a rare reactive, hamartomatous benign tumor-like lesion.The exact mechanism of the histogenesis of adenomyoma in the Vaterian system remains unclear. The most widely accepted hypothesis is that these lesions may represent a form of an incomplete heterotopic pancreas. Another hypothesis is that the dysfunction of the muscle fibers of the AOV due to chronic papillitis may lead to muscular hyperplasia or adenomyomatous hyperplasia. Furthermore, it has immunohistochemical characteristics of cytokeratin 7 expression without cytokeratin 20 expression.Adenomyoma is histologically characterized by a dense proliferation of ducts and ductules with smooth muscle bundles and collagen fibers. Multiple lobules of the glands are mainly located in the muscular layers of the Vaterian system, thereby resulting in the hypertrophy of the sphincter of Oddi.–7Adenomyoma of the AOV grows gradually and asymptomatically for a long period. Depending on the size of the lesion, the symptoms of adenomyoma of the AOV include RUQ pain, dysphagia, obstructive jaundice with or without cholangitis, and acute pancreatitis with clinical similarities to periampullary tumors. Menzel et al reported that endoscopic forceps biopsies did not allow for reliable preoperative diagnosis of tumors of the AOV. Moreover, they recommended that in case of enlarged or suspicious papillae, endoscopic biopsies from deep and superficial layers immediately following sphincterotomy should be obtained to improve the diagnostic accuracy of endoscopic biopsies.The preoperative accurate diagnosis of adenomyoma of the Vaterian system, including the AOV and CBD, is significant to appropriate patient management. However, discriminating between benign and malignancy is highly challenging. Patients are frequently mistaken as having periampullary malignancy, thereby leading to unnecessarily extensive surgical resection with a high risk of complications, including pancreatic fistula and delayed gastric emptying. Preoperative imaging modalities, such as computed tomography and magnetic resonance imaging, could be utilized to reveal CBD obstruction or well-demarcated mass lesions in the ampullary area. Additionally, ERCP is typically performed to allow direct inspection of the periampullary region. Furthermore, histopathological examination via endoscopic biopsies may be helpful in superficial lesions although could not definitely exclude underlying ampullary malignant lesions located deeper with a low diagnostic yield.,7,9 Therefore, accurate preoperative and intraoperative diagnoses, including histological evaluation, are mandatory for a definite diagnosis and prevent unnecessary extensive surgeries as in this case.Deciding which treatment option to select for ampullary lesions before surgery is highly challenging since radiologic imaging and endoscopic investigation may not be able to discriminate between benign and malignancy in the ampullary lesion. It has been reported that endoscopic ampullectomy or transduodenal papillectomy could be sufficient for the treatment of adenomyoma of the AOV.Although adenomyoma is very rare, it should be included in the differential diagnosis of mass-like lesions of the AOV to avoid unnecessary surgeries.Conceptualization: Hyung Jun Kwon, Sang Geol Kim, Jinyoung Park.Formal analysis: Hyung Jun Kwon, Sang Geol Kim, Jinyoung Park.Writing – original draft: Hyung Jun Kwon, Jinyoung Park.Writing – review & editing: Jinyoung Park.


Ancient schwannomas are benign tumours arising from the neural sheath of peripheral, cranial and autonomic nerves. They are commonly situated in the inner ear and spine with pelvic manifestations being rare. We present the case of a 30-year-old patient, who presented with an abdominal mass. MRI imaging suggested a broad ligament fibroid and open surgery was undertaken to remove it. Subsequent histology confirmed an ancient schwannoma. This case report details the rarity of such a condition and the need for a high index of suspicion as well as outlining management options and surveillance. Schwannomas are usually benign tumours which develop from the Schwann cells surrounding nerve cells. Those located in the head and neck account for 45% of schwannomas: other common sites include the mediastinum and spine. The majority of these tumours are sporadic but can be associated with genetic conditions such as neurofibromatosis 1 and 2.Pelvic schwannomas are often asymptomatic and may be diagnosed incidentally. When they do present with symptoms, it is commonly due to the mass effect of the tumour on surrounding structures but can be vague, such as abdominal distension or heaviness, urinary frequency, and constipation. It is often difficult to determine the diagnosis pre- operatively, but it is important to use imaging to determine the size, location, and interaction with local structures to aid with operative planning.While broad ligament fibroids are not an unusual gynaecological finding, schwannomas in the pelvis are rare, accounting for 1-3% of all cases . SchwannA 30-year-old patient was referred to gynaecology with a history of worsening abdominal swelling, pressure symptoms and a known uterine fibroid. Magnetic resonance imaging (MRI) revealed an 11cm likely broad ligament fibroid and both medical and surgical treatment options were discussed. Initial medical treatment with ulipristal acetate was largely ineffective so the patient opted for surgery. A myomectomy was discussed in detail including both the laparoscopic approach  and an open procedure. The decision was made to make the final decision intraoperatively depending on the size and mobility of the fibroid. As the fibroid was immobile and fixed within the pelvis on both vaginal and abdominal examination following general anaesthetic, the decision was to proceed with an open myomectomy.A lower umbilical midline incision was utilised, and findings were of a 12cm fibroid arising wholly within the broad ligament with the ureter lying inferolateral. No obvious connection was seen to the uterus and no other fibroids were visible. Monopolar electrocautery  was utilised to open the broad ligament and a full right ureterolysis was undertaken to lateralise the ureter.Following ureterolysis, the suspected fibroid was enucleated using a myoma screw and a combination of blunt and sharp dissection. The macroscopic appearances of the mass and its behaviour during dissection were in keeping with a fibroid. The specimen was sent for histology and a routine abdominal mass closure was performed. Blood loss was 350ml and the patient made a good recovery post-operatively.The histopathology subsequently revealed an ancient schwannoma, and no malignancy was seen in the sections examined. The neoplasm showed well defined margins with degenerative cystic change and haemorrhage present.A neurology review was subsequently arranged which excluded any other schwannomas or associated genetic disorders. There was no family history of schwannomas or neuromas, or personal history of café au lait spots or hearing impairment. The neurologist proposed that it was likely the patient had a somatic mutation leading to a schwannoma causing unilateral segmental fibromatosis. The management for this included MRI of the chest, head and neck and genetic testing for neurofibromatosis followed up by pelvic surveillance every 5 years. From a gynaecology perspective, the patient recovered well with no concerns at follow-up and has had a successful pregnancy.Schwannomas are rare, benign, usually solitary, neural tumours which arise from the neural sheath; they are well circumscribed with a capsule derived from the epineurium. They are easily dissected from adjacent tissues, making laparoscopic resection possible whilst mimicking the appearance of a fibroid capsule. Once resected, the recurrence rates of a schwannoma are low and if it does recur it is likely that the resection was not radical .Ancient schwannomas are a rare variant of schwannoma. It has distinctive features seen on histology due to their slow growing nature: the large mass leads to vascular insufficiency leading to degenerative changes including cystic necrosis, fibrosis, calcification, and degenerative nuclei .Epidemiology suggests an incidence of 1.1 per 100,000. Affected sites include the spine, inner ear, gastro-intestinal tract, and mediastinum: most commonly affecting the inner ear. There is a predilection in the second and third decades of life and they are more common in women . Pelvic Schwannomas can be sporadic or occur in association with genetic conditions such as Carney complex, neurofibromatosis and schwannomatosis and tend to be multiple in the latter two conditions. Schwannomas are slow growing, with rare malignant transformation (<1%).Schwannomas of the female genitalia are scarce. Wider literature reports 63 schwannomas arising from the female genital tract  and theyImaging with MRI helps to demarcate the lesion; they appear as a clearly delineated round mass with low signal intensity on T1, heterogeneously hyperintense on T2, and intense enhancement on contrast MRI. Uterine fibroids, a differential diagnosis, on MRI characteristically have high signal intensity on T1 and low signal intensity on T2. However, fibroids with degeneration have similar appearance radiologically . The useTreatment is complete excision and as the majority are benign, enucleation is usually curative. Being neurological in origin, if suspected pre-operatively, the patient should be counselled that neurological deficits may occur depending on the location of the pathology. Nedelcu et al.  reportedPelvic schwannomas are rare, especially those in the broad ligament with very few documented in the wider literature. Diagnosis is usually made histologically as imaging is often inconclusive. A high index of suspicion is needed, especially if risk factors are present and percutaneous biopsy pre- operatively to confirm diagnosis and aid surgical planning may be helpful. Surgical excision and complete resection are recommended to reduce recurrence. Following diagnosis, neurological review is essential to exclude extra-pelvic involvement and genetic predispositions.


Case Presentation. A 75-year-old man presented to another hospital with jaundice for 1 month. Laboratory findings showed elevated total bilirubin, 10.6 mg/dL and carbohydrate antigen 19-9, 54.8 U/mL. Computed tomography showed a well-enhanced tumor located in the distal bile duct and dilated hepatic bile duct. The gallbladder wall was thickened and homogeneously enhanced. Endoscopic retrograde cholangiopancreatography revealed a filling defect in the distal common bile duct, and intraductal ultrasonography showed a papillary tumor in the common bile duct, indicating tumor invasion of the bile duct subserosa. Subsequent bile duct brush cytology revealed adenocarcinoma. The patient was referred to our hospital for surgical treatment and underwent an open PPPD. Intraoperative findings showed a thickened and indurated gallbladder wall, suggesting concurrent gallbladder cancer; thus, the patient subsequently underwent PPPD and extended cholecystectomy. Histopathological findings confirmed gallbladder carcinoma originating from ICPN, which extensively invaded the liver, common bile duct, and pancreas. The patient started adjuvant chemotherapy (tegafur/gimeracil/oteracil) 1 month after surgery and had no recurrence at follow-up after 1 year.  Intracholecystic papillary neoplasm (ICPN) is a rare tumor first classified by the World Health Organization in 2010. ICPN is a counterpart of the intraductal papillary mucinous neoplasm of the pancreas and intraductal papillary neoplasm of the bile duct. Previous reports on ICPN are limited; thus, the diagnosis, surgical intervention, and prognosis are controversial. Here, we report an extensively invasive gallbladder cancer arising in ICPN treated with pylorus-preserving pancreaticoduodenectomy (PPPD) and extended cholecystectomy.  Accurate preoperative diagnosis of ICPN, including the extent of tumor invasion is challenging. To ensure complete curability, the development of an optimal surgical strategy considering preoperative examinations and intraoperative findings is essential. Intracholecystic papillary neoplasm (ICPN) is a relatively new concept established by the 2010 World Health Organization (WHO) classification .ICPN is rare, accounting for 0.4–1.5% of cholecystectomies and 6.4% of gallbladder cancers . TherefoHere, we report a rare case of extensively invasive gallbladder cancer arising in ICPN treated with pylorus-preserving pancreaticoduodenectomy (PPPD) and extended cholecystectomy and review the previous literature.A 75-year-old man presented to the hospital with jaundice that had been present for 1 month. He had a medical history of hypertension and non-tuberculosis mycobacterial infection but no surgical history. Laboratory findings showed elevated levels of serum bilirubin and liver enzymes: total bilirubin, 10.6 mg/dL; aspartate aminotransferase, 68 IU/L; alanine aminotransferase, 119 IU/L; and gamma-glutamyl transpeptidase, 393 IU/L. Serum carbohydrate antigen 19-9 was also elevated (54.8 U/mL), though carcinoembryonic antigen was within the normal range. Enhanced computed tomography (CT) revealed a well-enhanced tumor in the distal bile duct and dilation of the hepatic bile duct . In addiThe patient was referred to our hospital for surgical treatment, and we planned to perform PPPD for distal bile duct cancer with curative intent. Intraoperative findings revealed a thickened and indurated gallbladder wall, suggesting the coexistence of gallbladder cancer; thus, we performed PPPD and extended cholecystectomy. Intraoperative frozen-section analysis of the cut end of the hepatic bile duct was negative for the tumor. A macroscopic examination of the resected specimen revealed a papillary tumor that had extensively invaded the liver, cystic duct, bile duct, and pancreas Figures . PermaneWe report the case of a patient with extensively invasive gallbladder cancer originating in ICPN treated with PPPD and extended cholecystectomy. Adsay et al. reported that invasiveness was observed in 55% of ICPN cases ; howeverMoreover, Adsay et al. reported that approximately half of the ICPNs develop in the right upper abdominal region, and the other half are incidentally found by imaging studies , which iDistinguishing between ICPN and other gallbladder tumors using imaging studies is difficult. In our review, only 15.8% of patients with ICPN were diagnosed accurately before surgery. According to previous reports, ICPN is well-defined on enhanced CT and presents high or low T2 signal intensity and high diffusion-weighted imaging signal intensity on MRI , 7. FluoThe treatment for ICPN is oncological resection; however, the selection of the optimal surgical procedure is often challenging. Simple cholecystectomy is sufficient for ICPN limited to the gallbladder mucosa without invasion. However, approximately half of the ICPN cases have an invasive component . MoreoveSome studies have reported that ICPN with or without invasive carcinoma has a good prognosis, in contrast to other types of gallbladder carcinoma , 40, 41.The optimal choice of surgical procedure, including extended cholecystectomy, bile duct resection, and pancreaticoduodenectomy is essential for achieving complete oncological resection of the tumor. In addition, close postoperative follow-up is crucial for patients with ICPN, especially those with advanced cancer arising from the tumor, in accordance with other types of gallbladder carcinoma.Accurate preoperative diagnosis of ICPN, including the extent of tumor invasion, is challenging; however, both EUS and POCS are effective tools for resolving these challenges. ICPN has been recognized as a tumor with a better prognosis compared with other types of gallbladder carcinoma; however, a recent study reported that the prognosis of these tumors is equivalent. The optimal choice of surgical procedure and close postoperative follow-up are essential for patients with ICPN, especially those with advanced cancer arising from the tumor.


Balamuthia granulomatous amoebic encephalitis (GAE) is a peculiar parasitic infectious disease of the central nervous system, about 39% of the infected Balamuthia GAE patients were found to be immunocompromised and is extremely rare clinically. The presence of trophozoites in diseased tissue is an important basis for pathological diagnosis of GAE. Balamuthia GAE is a rare and highly fatal infection for which there is no effective treatment plan in clinical practice.Balamuthia GAE to improve physician understanding of the disease and diagnostic accuracy of imaging and reduce misdiagnosis. A 61-year-old male poultry farmer presented with moderate swelling pain in the right frontoparietal region without obvious inducement three weeks ago. Head computed tomography(CT) and magnetic resonance imaging(MRI) revealed a space-occupying lesion in the right frontal lobe. Intially clinical imaging diagnosed it as a high-grade astrocytoma. The pathological diagnosis of the lesion was inflammatory granulomatous lesions with extensive necrosis, suggesting amoeba infection. The pathogen detected by metagenomic next-generation sequencing (mNGS) is Balamuthia mandrillaris, the final pathological diagnosis was Balamuthia GAE.This paper reports clinical data from a patient with Balamuthia GAE accounts for only a small proportion of intracranial infections, it should be considered in the differential diagnosis.When a head MRI shows irregular or annular enhancement, clinicians should not blindly diagnose common diseases such as brain tumors. Although  Balamuthia GAE is a peculiar parasitic infectious disease of the central nervous system, and is extremely rare clinically, with a mortality rate of nearly 90% [Balamuthia GAE to improve physician understanding of the disease and diagnostic accuracy of imaging and reduce misdiagnosis. The publication of this case report obtained written informed consent from the patient.arly 90% . CliniciBalamuthia mandrillaris . The resulting high-quality sequencing data were compared to the Microbial Genome Database (https://www.ncbi.nlm.nih.gov/data-hub/genome/?taxon=66526) to identify microorganisms and showed that the total length of the Balamuthia mandrillaris covering the genome was 1,229,795 (bp), the coverage was 1.8177%, and the average depth was 1.03 X. The consciousness was clear, and the physical examination was cooperative. The pupils were equal and round on both sides and sensitive to light reflection. There was no other cranial nerve dysfunction, and the meningeal irritation sign was negative. There was no mass or ulcer on the whole skin. Laboratory tests showed no abnormality in blood routine and serum tumor markers screening. Head CT and MRI revealed a space-occupying lesion(2.6 cm× 1.4 cm× 0.8 cm)in the right frontal lobe The lack of basic knowledge and clear understanding of the epidemic spectrum of infections in this specialty suggests that we should improve our understanding of some rare pathogens. The recognition of these waterborne diseases is helpful for the diagnosis and differential diagnosis of central nervous system lesions. (3) This case fully demonstrates the great potential of mNGS detection in infectious diseases. For unexplained encephalitis, especially encephalitis caused by rare or emerging pathogens, mNGS should be properly used to help in confirming the diagnosis when conventional bacteriological and pathological techniques fail to identify the infection. Although the mNGS detection method does not provide drug susceptibility information, it should still be used as an important complement to conventional pathogen detection. (4) When a head MRI shows irregular or annular enhancement, clinicians should not easily diagnose common diseases such as brain tumors. Although Balamuthia GAE accounts for only a small proportion of intracranial infections, it should be considered in the differential diagnosis.This case was misdiagnosed as high-grade astrocytoma before surgery, and our experience and lessons learned from the diagnosis and treatment of this case are: (1) Diagnostic reports from imaging should not be overly relied upon during treatment. Although imaging can help guide the direction of diagnosis, more detailed medical history and thorough clinical examination are required and combined with a patient’s past and epidemiological histories and other ancillary tests. We repeatedly asked for the patient’s relevant medical history in detail. It is difficult to pinpoint exactly what exposure led to this infection. The patient had previously suffered frequent scratches on the dorsal skin of the lower limbs from poultry without any treatment, and this was the most likely route of occult extracranial infection with


A spontaneous rupture of an omental vessel can cause severe intraabdominal hemorrhage. We present a case of idiopathic omental hemorrhage caused by a vascular malformation. The literature is systematically reviewed.3/mcL of white blood cells and 185 × 103/mcL of platelets. The hemoglobin value was 6.7 g/L at admission. To stabilize the patient’s condition, a unit of packed cell was administered. A double contrast enhancement abdominal computer tomography was performed, which revealed a massive hemoperitoneum. Subsequently, an exploratory laparoscopy was performed to search for the responsible pathology. But it was not successful. The surgical plan was changed to laparotomy. The hemorrhage source was not found during laparotomy. Observation revealed a massive hemoperitoneum originating in the omental vessels. A portion of the omentum located on the greater omentum at the greater curve was removed. Based on the pathological examination of the extracted tissue, vascular malformations were identified. The patient recovered uneventfully and was discharged from the hospital 7 days after surgery. Previous reports assessing idiopathic omental bleeding were systematically reviewed. A total of 14 hits were identified in PubMed and Scopus from 2015 to November 2022 for idiopathic omental bleeding.A 65-year-old Iranian man was admitted to the emergency department for 10 days with abdominal pain. His medical history was not significant. Fever, vomiting, nausea, or anorexia were not reported. However, he was suffering from diaphoresis and malaise at the time. He did not smoke or drink alcohol. During physical examination, blood pressure was 82/60 mmHg with a temperature of 36.6 °C; heart rate was 96 beats/minute and respiratory rate was 18 breaths per/minute. An abdominal examination revealed mild tenderness in the periumblical. The focused assessment with sonography in trauma examination yielded positive results. The complete blood count showed 14 × 10Presence of positive focused assessment with sonography in trauma, abdominal pain, imaging evidence of fluid accumulation, and a reduction in hemoglobin levels collectively indicate the likelihood of arteriovenous malformation occurrence. The treatment options include surgical intervention and transcatheter arterial embolization. Surgical intervention is recommended for subjects with hemodynamic instability, persistent hypotension and those whose diagnosis is unconfirmed. Idiopathic spontaneous intraperitoneal hemorrhage occurs when intraabdominal vessels rupture without an identifiable underlying cause .Omental bleeding may occur due to trauma-associated injury and irritation, neoplasia , arteriaDue to their rarity, therapeutic management guidelines are not yet established. We present a case of idiopathic omental hemorrhage caused by a vascular malformation. A systematic review of previous reports was also conducted.3/L and 185 × 103/L, respectively. Hemoglobin was 6.7 g/L at admission. One unit transfusion of packed cells was done. As soon as the patient was stable, a double contrast enhancement abdominal computer tomography (CT) was performed, which revealed massive hemoperitoneum. Through abdominal CT, we were unable to identify the source of bleeding. Hemoperitoneum was proved by ascitic tap ultrasound. During hospitalization, the hemoglobin value decreased by 2 units in 5 days. Subsequently, an exploratory laparoscopy was performed, searching for the responsible pathology. Approximately 2 L of blood was suctioned. But it was not successful. So, the surgical plan was changed to laparotomy. During laparotomy no active hemorrhage source was found. There was only a slight change (the lesion-like hematoma was 3 mm in size) in apparent omentum. Our decision was to take a biopsy of the omentum located on the greater omentum at the greater curve and send it for pathology analysis. Pathological assessment of the extracted tissue pointed to abnormally dilated blood vessels—an arteriovenous malformation (AVM)  examination yielded positive results. The complete blood count (CBC) reported white cell and platelet counts of 14 × 10PubMed and Scopus (2015–2022) databases were searched for case reports of idiopathic omental bleeding. Screening was conducted on all abstracts published in the English language. Data on patient characteristics, including age, diagnosis, and treatment, were extracted.n = 7) or transcatheter arterial embolization (TAE) (n = 4), and one patient underwent both. Five patients underwent omentectomy and three had ligation, all of which achieved hemostasis. One patient was managed non-operatively as a conservative case [Out of the 12 articles, one of them contained three cases of idiopathic omental bleeding, which we have included in our study –16. The ive case .Table 1ROmental bleeding, with a mortality rate exceeding 30%, is a serious condition . The morFAST is frequently employed to expedite the prompt identification of life-threatening hemorrhage in patients. The majority of patients with positive FAST results require laparotomy .Idiopathic omental bleeding requires aggressive treatment, regardless of its underlying cause. Omentectomy or ligation are routine surgical procedures for idiopathic omental hemorrhage. Most reported cases, however, involved emergency surgery. The surgical option is suitable for patients with persistent hypotension and unconfirmed diagnoses. The reason why surgery is often needed is that few cases are diagnosed correctly before treatment .Vascular malformations (VMs) are treated with surgery and embolization. Embolization through endovascular means is less invasive and recommended in most cases. Life-threatening conditions can arise due to VMs because of their unpredictable clinical evolution and manifestations .The cases presented in this manuscript are spontaneous, with no history of trauma, coagulopathy, or comorbidities, except for two , 7. AbdoOmental hemorrhage commonly presents in male patients with abdominal pain and occasionally with nausea, vomiting, or diarrhea. However, some of our included cases may not have had any other abdominal symptoms , 8–13.Ultrasonography, computed tomography scan with contrast, chest x-ray , angiogrOptimal diagnostic and therapeutic evidence for spontaneous intraperitoneal hemorrhage remains controversial, based on the available literature. Through our case analysis and literature review, it has been observed that the prevailing diagnostic model for arteriovenous malformation primarily relies on the presence of positive FAST, abdominal pain, imaging evidence of fluid accumulation, and a reduction in hemoglobin levels, all of which collectively indicate the likelihood of AVM occurrence. CT angiography (CTA) may have false negative results, however, due to short acquisition times when bleeding is not obvious at time of scan . It has A laparotomy or laparoscopy coupled with an omentectomy or a simple artery ligation is recommended. Laparoscopic surgery or transcatheter arterial embolization have been used more frequently in recent years as minimally invasive interventions , 22.As described in the report, a patient presented to our hospital with sudden, non-specific abdominal pain, which was diagnosed with idiopathic spontaneous intraperitoneal hemorrhage caused by a vascular malformation in the omentum.A spontaneous intraabdominal hemorrhage without any antecedent trauma should begin with volume resuscitation to stabilize the patient’s circulatory parameters. Coagulation studies and platelet function analysis should be considered. Hemorrhages of the omentum, however, are infrequent, and patients’ conditions are often unstable. In cases where no source of bleeding is found, a high index of suspicion should be kept. Presence of positive FAST, abdominal pain, imaging evidence of fluid accumulation, and a reduction in hemoglobin levels collectively indicate the likelihood of AVM occurrence. The venous hypertension, which can be caused by factors such as consuming food or engaging in extensive sports, can lead to reduced perfusion pressure in the surrounding tissues. For both unconfirmed and definitive diagnoses and treatments, emergency surgery is recommended. To rule out underlying malignancy or vascular disease, omentectomy is preferred to ligation or transcatheter arterial embolization. Rebleeding should be eliminated from these patients by omentectomy as a definitive therapy.


Pheochromocytoma is a neuroendocrine tumor, and its treatment is dependent on surgical resection. Due to the wide availability of cross-sectional imaging, pheochromocytomas are commonly seen as small tumors less than 10 cm in size and are mostly treated with minimally invasive surgery. Their concomitant presence with horseshoe kidney or other anatomical and vascular anomalies is rare. Herein, we present a surgically complex giant pheochromocytoma case who underwent an open left radical adrenalectomy.A 41-year-old Hispanic female presented with a 12 × 8 cm left hypervascular adrenal mass, pelvic horseshoe kidney, and severely dilated large left retro-aortic renal vein which branched into a left adrenal vein, congested left ovarian vein, and left uterine plexus. She was managed with insulin and metformin for uncontrolled diabetes with an A1c level of 9% and doxazosin for persistent hypertension. Clinical diagnosis of pheochromocytoma was confirmed with elevated urine and serum metanephrine and normetanephrine. The pre-operative ACTH was within normal range with a normal dexamethasone suppression test and 24-hour urine free cortisol. The adrenalectomy of the highly aggressive adrenal mass was performed via open approach to obtain adequate surgical exposure. Due to the large size of the tumor and its significant involvement with multiple adjacent structures, coordination with multiple surgical teams and close hemodynamic monitoring by anesthesiology was required for successful patient outcomes including preservation of blood supply to the pelvic horseshoe kidney. The histopathological diagnosis was pheochromocytoma with negative surgical margins. The patient was followed at 1, 4, 12, and 24 weeks postoperatively. She had a normal postoperative eGFR and was able to discontinue antihypertensive and antidiabetic medications at four weeks. She had transient adrenal insufficiency, which resolved at five months. The horseshoe kidney was intact except for a minimal area of hypo-enhancement in the left superior renal moiety due to infarction, which was significantly improved at six months.Our patient had a giant pheochromocytoma with anatomical variations complicating an already surgically challenging procedure. Nonetheless, with multiple provider collaboration, detailed pre-operative surgical planning, and meticulous perioperative monitoring, radical resection of the giant pheochromocytoma was safe and feasible with successful postoperative outcomes. Pheochromocytomas are neuroendocrine tumors originating in the adrenal medulla. The tumors may be sporadic, though many carry mutations which have been inherited in an autosomal dominant pattern, and the latter is frequently found with other genitourinary anomalies and associated with increased risk of neuroendocrine renal tumors . SymptomA 41 year-old Hispanic female with known uncomplicated type 2 diabetes mellitus for about 8 years treated with metformin presented to the emergency department with complaints of chest and abdominal pain, dyspnea, intractable nausea, and vomiting. She was in acute distress with tachycardia and hypertension, found to be in diabetic ketoacidosis, and diagnosed with an active COVID-19 infection prompting hospitalization. She was admitted to ICU and treated with continuous insulin infusion and IV fluids. She also acknowledged longstanding complaints of headaches, diaphoresis, and palpitations started several years ago prior to this presentation. Given chest pain at initial presentation, and elevated serum troponin level, she underwent further evaluation with CT chest, which revealed a 12 × 8 cm adrenal mass and a horseshoe kidney. The clinical diagnosis of pheochromocytoma was confirmed with elevated urine and serum metanephrine and noremetanephrine. Her diabetic ketoacidosis improved, and her COVID-19 infection was asymptomatic during her admission. She was discharged with long-acting insulin, and an alpha-blocker (terazosin), and the initial treatment plan was to proceed with resection of her adrenal mass. Nonetheless, her planned surgery was cancelled a few times due to patient’s insurance problems, and incompliance with use of phenoxybenzamine.About 22 months after initial diagnosis of pheochromocytoma, the patient was ready for surgery. The pre-operative assessment revealed normal levels of ACTH with a normal dexamethasone suppression test and 24-hour urine free cortisol. The serum renin, aldosterone, and DHEAS were in the normal range which excluded co-secretion from the pheochromocytoma. Further workup with CT chest abdomen pelvis and PET/CT Copper Cu 64 Dotatate scans were negative for metastasis and additional paragangliomas. CT angiogram for surgical planning revealed the large hypervascular adrenal mass, pelvic horseshoe kidney, severely dilated left retro-aortic renal vein which branching into an enlarged left adrenal vein, congested left ovarian vein, congested uterine plexus, and a minimum of three prominent arteries supplying the mass branching from the abdominal aorta, left renal artery, and L1 lumbar artery , diagnostic of adrenal insufficiency. The adrenal insufficiency was asymptomatic and required no treatment. Her blood glucose normalized, and insulin was discontinued. The patient was discharged on postoperative day six. Postoperative CT scan confirmed that the horseshoe kidney was preserved with some hypo-enhancement due to infarction in the left superior renal moiety Fig. . This inThe histopathological examination revealed a pheochromocytoma, 12.6 cm in greatest dimension, with lymphovascular and capsular invasion Fig. .Originally derived from the neural crest, pheochromocytomas are neuroendocrine tumors of the adrenal medulla that arise from chromaffin cells. These tumors may be sporadic or hereditary, manifesting as part of a genetic syndrome such as Multiple Endocrine Neoplasia type 2, Von Hippel-Lindau disease, and Neurofibromatosis Type 1. The annual incidence of a pheochromocytoma is 2 to 9.1 per million adults and, when sporadic, commonly become apparent at 30–50 years old [Although only estimated to occur in 24% of pheochromocytoma patients, the tumor’s secretion of catecholamines give rise to the classic triad of palpitations, diaphoresis, and headache . This paAdrenal tumors are commonly benign. Conversely, adrenocortical carcinoma is a rare malignant adrenal mass that produces steroids and androgens. It has also been described in few cases to mimic pheochromocytomas . In addiMinimally invasive surgery with laparoscopic adrenalectomy is preferred for smaller pheochromocytomas due to lower rates of intraoperative hemodynamic instability, less intraoperative blood loss, and shorter postoperative hospital stays . HoweverThe additional challenge of adrenalectomy for pheochromocytoma is perioperative hemodynamic instability in the form of both severe hypertension and hypotension. Hypertension is observed with manipulation or palpation of the pheochromocytoma. Conversely, extreme hypotension arises upon interruption of the vessels leading to a sudden decrease in catecholamine secretion. Hypotension is further exacerbated by decreased vascular volume by intraoperative blood loss. In a study of 67 patients who underwent surgery for pheochromocytoma, changes in blood pressure greater than 30% of the patient’s pre-operative mean arterial pressure were observed in 97% of patients, with 36% experiencing hypertension and 93% experiencing hypotension . Five orThorough pre-surgical planning and close coordination among urologist, anesthesiologist, endocrinologist and other surgical teams are essential to prevent severe perioperative complications. Moreover, Abdou et al. reported a challenging case of a 5.5 cm left adrenal mass and horseshoe kidney, which failed surgical resection twice previously. The first attempted laparoscopic surgery was aborted due to severe adhesions, whereas the second attempted open surgery was aborted due to severe bleeding . Only afTo our knowledge, this is the first case report describing a giant pheochromocytoma with a concurrent horseshoe kidney. She also had several additional anatomical variations, including the retro-aortic left renal vein, and congested left ovarian vein, complicating an already surgically challenging procedure. Wide resection of the highly aggressive adrenal tumor was required due to the symptomatic mass caused by catecholamine overproduction and the possibility of malignancy. The surgical principles of gentle retraction of the adrenal mass and early control of adrenal vein to prevent bursts of catecholamine release may not be easily feasible for complex large pheochromocytoma cases with anatomical anomalies. Nonetheless, with detailed anatomic studies, thorough pre-operative planning, multiple provider collaboration, and meticulous intraoperative monitoring, it is possible to minimize complications and achieve successful outcomes.


Mesenchymal chondrosarcomas are extremely rare and aggressive tumors that primarily affect patients between the ages of 20 and 30. These neoplasms are typically found in the lower limbs and cranial region. Their occurrence within soft tissues is exceedingly rare, and the initial presentation often includes immediate metastatic dissemination. Given the extraordinarily low prevalence of extraskeletal mesenchymal chondrosarcoma, treatment approaches remain non-standardized. Surgical resection combined with neoadjuvant chemotherapy or radiotherapy is the most commonly favored strategy by medical teams. In this case report, we present the case of a 72-year-old patient with no specific medical history, who presented with a non-metastatic extraskeletal mesenchymal chondrosarcoma located in the popliteal fossa. The therapeutic intervention encompassed surgical resection followed by adjuvant radiotherapy. After 18 months of follow-up period, there was no evidence of local recurrence or distant metastases. The disparity between the patient's clinical characteristics and the existing medical literature may provide new insights into understanding this neoplastic entity. Mesenchymal chondrosarcoma is an extremely rare, highly malignant tumor, accounting for less than 10% of all chondrosarcomas. It usually involves bone  -4. The mA 72-year-old female patient, with an unremarkable medical history or prior treatments, presented with a painless soft tissue mass of the posterior region of the distal thigh, which increased progressively in size. She was referred by the radiologist who discovered the lesion to the multidisciplinary tumor board meeting. Physical examination revealed a palpable, mildly tender, firm, and immobile mass in the posterior portion of the distal thigh region, with normal overlying skin. No other palpable masses were found in other sites, and there were no abnormalities in the neurological and vascular examinations. Other general and systemic examinations were normal, and the results of the blood workup were unremarkable.A CT scan and MRI were performed in December 2021, revealing a deep soft tissue mass measuring 7 cm in its longest dimension in the posterior region of the distal thigh.Regarding the MRI findings Figures -1C, theyThe differential diagnosis included calcified soft tissue lesions such as extraskeletal osteosarcoma, extraskeletal myxoid chondrosarcoma, synovial sarcoma, myositis ossificans, hematoma, hemangioma, and calcific myonecrosis . BecauseIn January 2022, an ultrasound-guided biopsy was performed, revealing a tumor characterized by monotonous spindle cells with a high N: C ratio associated with a staghorn vascular pattern Figure . ImmunohThe patient's case was discussed in the multidisciplinary tumor board meeting, and it was decided to conduct an extension assessment for staging with cerebral MRI and thoracoabdominal-pelvic computed tomography (TAP-CT), followed by performing a wide surgical resection with short margins around the popliteal vessels and then followed by adjuvant radiotherapy.The staging assessment did not reveal any metastases. The surgical procedure took place in March 2022. Macroscopy examination revealed a firm tumor measuring 6.5 cm and containing calcified areas. Pathological examination confirmed the presence of a tumor composed of round or spindle cells with a hemangiopericytomatous vascular pattern admixed with bone tissue areas and only focal islands of cartilage Figure . The resThe patient received adjuvant radiotherapy that covered the surgical field using the external beam radiation therapy (EBRT) technique (60 Gy in 30 fractions), which was administered five weeks postoperativelyA surveillance plan was established, consisting of contrast-enhanced MRI combined with thoracic CT scans, alternated with TAP  CT scans every four months for a period of three years, then biannually until five years postoperatively.In 18 months of postoperative follow-up, there have been no signs of local recurrence or distant metastasis.EMC is an extremely rare malignant tumor and represents less than 1% of all chondrosarcomas . The lesDue to the high degree of malignancy, it is not uncommon for the clinical presentation to be aggressive, with metastasis evident from the outset. The lesions are primarily found in the lungs ,3,5. TheSeveral atypical locations have been described in the literature, including the uterus, orbit, kidney, or pancreas ,5-7. LocCharacteristic imaging reveals a tissue mass with calcifications ,4. At thHistologically, the tumor is usually characterized by the presence of small round cells with a staghorn vasculature and a variable proportion of islands of well-differentiated cartilage ,6,17,18.Regarding treatment, the gold standard remains wide surgical resection. Adjuvant chemotherapy, such as doxorubicin is recommended by most teams for mesenchymal chondrosarcoma involving bone. However, there is no standardized protocol, specifically when presenting in soft tissue ,3,4,18. A mesenchymal extra-articular chondrosarcoma is an extremely rare tumor, typically found in young individuals. This case, presenting with a tumor localized in the popliteal fossa, non-metastatic in a 72-year-old patient, and showing no recurrence after treatment, is highly unusual. The treatment approach, which lacks consensus, often involves resection with adjuvant chemotherapy. In our case, given the advanced age of the patient, resection with adjuvant radiotherapy was chosen to optimize treatment tolerance. The most recent follow-up, 18 months after treatment, demonstrates an absence of recurrence. These atypical findings, in light of the existing literature, could contribute to a better understanding of the characteristics of this rare tumor.


Desmoplastic round cell tumor, though rare, must be taken into consideration as a differential diagnosis, thus aiding in early evaluation and changing the trajectory of the natural history of the disease condition, and improving the prognosis of patients.Desmoplastic small round cell tumor is a rare, aggressive tumor of mesenchymal origin with an incidence of 0.74 cases per million. We present a young adult with a periumbilical mass who was diagnosed as a desmoplastic round cell tumor and later was treated with exploratory laparotomy and resection of the tumor with no recurrence during a 6‐month follow‐up period. Histopathological image of desmoplastic small round cell tumor. Since there is no established standard management protocol for the care of DSCRT, treatment modalities entail cytoreductive surgical debulking, high‐dose alkylator‐based chemotherapy regimens, or immunotherapy as a possible alternative treatment modality.We present a young adult with a desmoplastic small round cell tumor who presented with a periumbilical mass and was treated with exploratory laparotomy and resection of the tumor with no significant recurrence during a 6‐month follow‐up period.2A 25‐year‐old male presented to our center for the evaluation of a periumbilical mass for 5 months, which was insidious at the onset, gradually increasing with a rapid increase in the last 2 months, and associated with mild dragging‐type pain for the last month. He was initially addressed at the local level with the diagnosis of gastritis, but in contrast, this was not resolved. Thereafter, he was under traditional treatment for the last month. Furthermore, he provided a history of progressive weight loss of approximately 4 kg in the last month. The patient denied any history of serious medical conditions in the past or a significant family history of cancer. He was a non‐smoker and non‐alcoholic.An abdominal examination revealed a firm, smooth‐surfaced, intraperitoneal, mobile mass that measured approximately 18 × 15 cm in the umbilical, hypogastrium, right lumbar, and iliac regions. However, other systemic examinations revealed no significant abnormalities.The CECT abdomen Figure  revealedBased on these findings, exploratory laparotomy with excision of omental mass plus total omentectomy along with right‐sided peritonectomy under general anesthesia was performed. A large mass  of the omental mass Figure  revealedFurthermore, the immunohistochemistry (IHC) customized panel; WT1, EMA, Desmin, CK, Myogenin, SMA, Vimentin, CD99, NKX2.2, Chromogranin, CD45, and Ki‐67, as well as FISH to assess for EWSR1‐WT, were done, among which PanCK (AE1/AE3) and Desmin (GM 007) showed positive status   that resulted from the fusion of the Ewing's sarcoma gene's (EWS) N‐ and C‐terminal domains with the Wilms tumor suppressor gene's (WT1), creating the EWSR1‐WT1 gene.IDSRCT is frequently detected in a late stage. At the time of diagnosis, there may be even enlarged lymph nodes and distant metastases.The diagnostic test, fluorescent in situ hybridization (FISH), shows chromosomal translocation t  , which causes the EWS and WT1 genes to fuse.For the treatment of DSCRT, multimodal therapy is being considered. The disease can be controlled by combining high‐dose chemotherapy with extensive surgery and tumor debulking. Irradiating the entire abdomen may marginally increase long‐term survival.4DSRCT must be taken into consideration while making a differential diagnosis for young individuals with abdominal tumors. Early diagnosis can help in improving the prognosis of the condition.Sujan Bohara: Conceptualization; resources; writing – original draft; writing – review and editing. Pinky Jha: Conceptualization; resources; writing – original draft; writing – review and editing. Pawan Singh Bhat: Data curation; supervision; writing – review and editing. Srijan Malla: Data curation; supervision; writing – review and editing. Samikshya Karki: Resources; writing – review and editing. Saroj Kumar Jha: Resources; writing – review and editing. Sunil Basukala: Supervision; writing – review and editing. Sushil Bahadur Rawal: Supervision; writing – review and editing.All authors declare that they have not received any grants or funding for this manuscript.All the authors declare that they have no conflicts of interest.Not required for the publication of this manuscript.Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.The Abstract has been submitted to the 5th International Cancer Congress held on March 17–18, 2023, in Janakpurdham, Nepal.


Canal of Nuck masses is a rare occurrence that can cause swelling in the abdominal-inguinal region of females for various reasons. This condition arises due to an abnormal persistence of the processus vaginalis opening. Although Canal of Nuck pathology is not widely known among general surgeons or gynecologists due to its rarity, it has been associated with significant morbidity and requires further research. In this comprehensive review, we aim to summarize the embryology and anatomy of the Canal of Nuck, followed by a description of the various types of masses that can occur in this region. We discuss the clinical presentation and diagnostic workup of Canal of Nuck masses, including imaging modalities and differential diagnoses. Next, we review the surgical management of these masses, including open and laparoscopic approaches. Finally, we discuss the potential complications and long-term outcomes associated with Canal of Nuck pathology. This review aims to compile the presently accessible literature on anomalies occurring in the Canal of Nuck in females, with a particular focus on describing their pathological nature, diagnosis, and management. In summary, this review provides an up-to-date understanding of the pathology, diagnosis, and management of Canal of Nuck masses and aims to raise awareness of this under-recognized surgical challenge among healthcare providers. The canal of Nuck was first identified by Dutch anatomist Anton Nuck in 1691 [The inguinal canal is a narrow passage situated in the lower abdominal wall, measuring a mere 4 cm, and connecting the peritoneum to the perineum -7. It ruThe inguinal canal's embryology involves the gubernaculum and the processus vaginalis. The gubernaculum, a muscular and fibrous tissue cord, forms between 8 and 12 weeks during fetal development . In femaThe most common clinical sign of canal of Nuck pathology is the presence of a mass or swelling in the groin or labia, which may be accompanied by pain. These masses encompass various differential diagnoses, including lymph node, cyst, inguinal hernia, infection/abscess, inguinal gonad, endometriosis, benign tumors, and neoplasia. Unfortunately, many clinicians, including surgeons, remain unaware of this anatomical variation and the potential pathology it may present. It is crucial for clinicians and surgeons to be knowledgeable about the masses in the canal of Nuck, as they can mimic other pathologies in the inguinal region. A thorough understanding of these masses' anatomy, clinical presentation, and imaging characteristics can help avoid misdiagnosis and inappropriate treatment. By making an accurate diagnosis, unnecessary biopsy or surgical intervention can be avoided, leading to better patient outcomes and reduced healthcare costs. Surgical intervention is considered the gold standard for managing symptomatic masses in the canal of Nuck. This involves carefully dissecting the mass and surrounding structures to minimize the risk of injury to adjacent nerves, vessels, and organs. In cases where malignancy is suspected, wide local excision with negative margins is recommended to achieve complete tumor removal. However, it is important to note that surgical management should be tailored to the individual patient and their specific clinical scenario. In some cases, conservative management with close observation may be appropriate, especially in asymptomatic or low-risk lesions.MethodsWe systematically searched PubMed and CENTRAL databases using specific keywords related to the canal of Nuck, masses, and anomalies in female patients. The search was limited to articles published within the last 10 years (2012-2022). We used a combination of the following keywords: 'canal of Nuck,' 'masses,' 'anomalies,' 'females,' 'women,' 'girls,' and 'pediatric.' Our inclusion criteria were studies that examined masses or anomalies in the canal of Nuck in female patients, regardless of age. We excluded studies that did not specifically focus on the canal of Nuck or masses/anomalies in female patients.We initially retrieved 215,240 articles, which were then screened for relevance. After carefully reviewing and eliminating duplicate articles, we identified 1510 potentially relevant articles. We then used the inclusion and exclusion criteria to select the final articles for our review. We included studies that reported original research, case reports, or case series that specifically investigated the canal of Nuck, masses, or anomalies in female patients. We excluded studies that were not in English or did not report original data.After applying these criteria, we identified 1010 relevant articles. We selected the final 44 studies from these articles for our qualitative review. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) flow diagram , Type 2 is a communicating hydrocele, and Type 3 is a bilocular hydrocele .The most common type of canal of Nuck hydrocele is Type 1, which occurs due to incomplete occlusion of the canal. In this case, the caudal portion of the canal remains open while the cranial portion is occluded. This occlusion progresses from the deep to the superficial inguinal ring. Since there is no connection between the peritoneal cavity and the canal of Nuck, fluid accumulates in the canal, leading to the development of a cyst. A cystic dilation may be caused by a discrepancy between the absorption and secretion of fluid, an alteration in lymphatic drainage, trauma, or infection, or it may be idiopathic .It should be noted that a canal of a Nuck cyst is different from an inguinal hernia sac in that it contains fluid instead of adipose tissue or intestine, and it is not connected to the peritoneal cavity. Type 2 of the cyst, a communicating hydrocele, is characterized by its continuity with the peritoneal cavity. As a result, the size and shape of the cyst may change during the Valsalva manoeuvres . The thiSarkar et al. reported a case of encysted hydrocele in a three-year-old female . Sala etCanal of Nuck HerniaA patent processus vaginalis increases the likelihood of acquiring an indirect inguinal hernia. In this situation, when any intra-abdominal organ herniates through the deep inguinal ring, lateral to the inferior epigastric artery, following the round ligament and ultimately reaching the labia majora, the hernia is categorized as "indirect" . HerniasThese types of hernias have a high morbidity rate due to the high risk of incarceration and torsion, which can lead to necrosis. Choi and Baek reported a case of a 15-day-old girl with an ovarian hernia that was incarcerated in the canal of Nuck . Sadiqi Ultrasonography (USG) is generally the first imaging modality to diagnose hernias in children, while computed tomography (CT) scans are often used in adults with an acute abdomen. However, surgeons must exercise caution when dealing with adult hernias, particularly if they are painful or complex. These can be associated with a higher risk of complications such as intestinal blockages or ischemia. In severe cases, these blockages can lead to symptoms such as nausea, vomiting, abdominal pain, and constipation. Surgeons must remain vigilant for signs of complications and consider prompt intervention to minimize the risk of serious harm ,33.Inguinal GonadsHernias in the inguinal canal of females can contain either the ovaries alone or the ovary in conjunction with other reproductive organs. Inguinal hernias containing the ovary are more common in newborns than adolescents or adults, who encounter them relatively less frequently. These types of hernias may be more prevalent in preterm newborns, as the processus vaginalis fuses within the first year of life, leaving them with a patent processus vaginalis. Inguinal hernias in females can also be indirect or direct, with indirect hernias being more common and often associated with a patent processus vaginalis. Regardless of their type, hernias in the inguinal canal can be a significant source of morbidity and require prompt diagnosis and treatment.Infectious or inflammatory conditionsInflammatory or infectious diseases of the canal of Nuck can arise from the hematogenous spread of an existing phlogistic process into a pre-existing cyst in the canal of Nuck. Alternatively, they may be linked to an abdominal infection that spreads into the canal due to its communication with the peritoneal cavity. This clinical manifestation is typically marked by pain or discomfort, feverish illness, an inguinal lump, and reactive immunological tests.Cysts of Canal of NuckAlthough rare, cysts in the canal of Nuck are the most commonly reported pathology . A cyst Due to their elongated and narrow form, cysts in the canal of Nuck are often referred to as having a sausage-like appearance. In one case, Ferreira et al. reported a 23-year-old girl with a sausage-shaped lump in her right labia majora, which was a cyst of the canal of Nuck .On the other hand, if fluid accumulates within the cyst, it can expand and become more spherical. Similar to cysts in other parts of the body, a Nuck canal cyst can be simple or complex with internal septations and debris. In a case report by Lai et al., a complex cyst was reported to have formed in the canal of Nuck .Surgery is the only successful treatment for a cyst in the canal of Nuck. Many patients with symptomatic cysts undergo surgery to treat their symptoms or because a hernia was assumed to be the underlying condition. Simple, asymptomatic cysts can be treated with percutaneous aspiration, but recurrence has been documented .Infection/AbscessGiven its anatomical relationship with the peritoneal cavity, infections such as abscesses are typical of intra-abdominal origin. However, pathologies within the canal of Nuck, such as cysts, may also become superinfected via hematogenous dissemination, leading to an abscess within the canal. Clinical manifestations of such abscesses may include fever, discomfort, the presence of an inguinal mass, and/or elevated levels of inflammatory markers, including leukocytosis. Similar to the management of abscesses in other areas of the body, treatment may involve the use of antibiotics and drainage.EndometriosisEndometriosis is a condition that frequently occurs in the ovary, pelvic portion of the parietal peritoneum, pouch of Douglas (POD), and uterosacral ligament . There aThe most common type of endometrial implant seen in the canal of Nuck is a solid, fibrotic endometrioma, which is more frequently identified on the right side . SurgicaVascular conditionsCommonly, this condition involves hematomas, varicoceles, and, in rare cases, post-traumatic arteriovenous fistulas, aneurysms, and varices of the great saphenous vein.HematomaAn inguinal hematoma typically presents as soreness and swelling in the affected area. In addition to post-traumatic or postsurgical causes, anticoagulation therapy, catheter administration, and malignancy can also cause hematomas . HematomThorgersen et al. reported a hematoma in the inguinal canal of a 42-year-old male patient previously treated with warfarin . To our VaricoceleGenerally, when the term "female varicocele" is used, it refers to pelvic congestion syndrome. Nonetheless, a varicocele can occur in the canal of Nuck, which is located near the round ligament . These vNeoplasmsNeoplasms can originate from various types of cells, such as fat, muscle, blood vessels, connective tissue, neural tissue or lymphoid tissue, all of which are structural components of the inguinal canal. Similar to other body parts, these neoplasms can be benign or malignant. Among the benign lesions, a lipoma, a tumor composed entirely of fat, is the most prevalent . HoweverVarious benign masses can appear in the canal, including neurofibromas, desmoid tumors, and leiomyomas. Desmoid tumors are fibrous tumors that are both aggressive and benign. Although many of the structures in the canal of Nuck can give rise to primary malignant neoplasms, it is extremely rare for benign conditions to transform into malignant forms. For instance, an endometrial implant can lead to endometroid cancer . The canSofia et al. reported a single instance of pseudomyxoma peritonei that affected the canal of Nuck. The case involved a 38-year-old woman who suffered from infertility and chronic pelvic pain . On the ResultsThis review article aims to identify the various pathological entities that arise in the inguinal canal of females, focusing on the canal of Nuck. The most common abnormalities detected in this canal include hernias and hydroceles, infections or inflammatory conditions, vascular conditions, and neoplasms.The canal of Nuck hydrocele is indicated by swelling along the route of the inguinal canal or inside the labia majora. There are three categories of the canal of Nuck hydrocele depending on the degree of fluid distension, including Type 1 cyst of the canal of Nuck, Type 2 communicating hydrocele, and Type 3 bilocular hydrocele. Type 1 is the most common type of canal of Nuck hydrocele and is caused due to incomplete occlusion of the canal, leading to fluid accumulation in the canal .Hernias of the canal of Nuck is relatively more common in preterm infants, as the canal of Nuck typically closes within the first year of life. The ovary may herniate through the inguinal canal in tubal and ovarian hernias, specifically in the pediatric population, when the gubernaculum is not attached to the uterus. These types of hernias have a high morbidity rate due to the high risk of incarceration and torsion, which can lead to necrosis .Ultrasonography (USG) is generally the first imaging modality to diagnose hernias in children, while computed tomography (CT) scans are often used in adults with an acute abdomen. Surgeons must exercise caution when dealing with adult hernias, particularly if they are painful or complex. These can be associated with a higher risk of complications such as intestinal blockages or ischemia . Overall, recognizing the significance of the location of these abnormalities within the canal of Nuck is important for accurate diagnosis and effective treatment. The review article provides a comprehensive overview of the various pathological entities that arise in the inguinal canal of females and highlights the need for prompt intervention to minimize the risk of serious harm.In conclusion, canal of Nuck masses is a rare but clinically significant condition that requires timely recognition and appropriate management. Diagnosis can be challenging, but a thorough understanding of the anatomy and diagnostic workup is crucial to accurately identify and differentiate these masses from other pelvic lumps or swellings. Once diagnosed, canal of Nuck masses management often involves surgical intervention, with both open and laparoscopic approaches available. With advances in imaging technology and surgical techniques, the prospects for successfully managing these masses have significantly improved in recent years. It is important to raise awareness of this under-recognized surgical challenge among healthcare providers to ensure patients receive timely and appropriate management.


Intramedullary myxopapillary ependymomas are rare spinal cord tumors primarily affecting young adults. Grade 2 tumors are associated with a higher proliferative index and potentially more aggressive behavior compared to grade 1 tumors. We present a case of a 30-year-old male who presented with a three-month history of progressive unilateral lower back pain that was refractory to analgesics. Neurological examination revealed bilateral lower limb weakness and sensory impairments in the L2 region. MRI confirmed a well-defined, enhancing intramedullary lesion at the L2 level, causing cord enlargement and edema. Diagnosis of grade 2 intramedullary myxopapillary ependymoma was made. Complete surgical resection was performed, confirming a grade 2 myxopapillary ependymoma. Postoperatively, the patient demonstrated significant improvement in lower limb function and sensation, with no tumor recurrence during long-term follow-up. Rehabilitation therapy was initiated, while close monitoring for complications and tumor progression was maintained. This case explores the etiology and features of intramedullary myxopapillary ependymomas and underscores the importance of early recognition, accurate diagnosis, and aggressive surgical management. Ependymomas are rare neuroepithelial tumors derived from ependymal cells within the central nervous system . They arAccording to the World Health Organization (WHO), ependymomas can be classified into four histopathological subtypes based on their grade of anaplasia. This aids in determining prognosis, and categories include (i) subependymoma, which is grade I, (ii) classic ependymoma, which is grade II, (iii) anaplastic ependymoma, which is grade III and associated with rapid growth and (iv) myxopapillary ependymoma (MPE), which was recently moved to the grade II category . HoweverMPEs of the spine are extremely rare with an incidence of 0.01 per million people . They prThe optimal management of ependymomas poses a challenge for physicians; however, surgical resection is the most commonly employed treatment modality . SurgicaA 30-year-old Asian male, school teacher, presented to the outpatient neurology department of a tertiary care hospital with a three-month history of unilateral (left) lower back pain that was initially non-radiating and relieved by analgesics. Subsequently, for one month, the pain extended to the contralateral lower extremity, growing in intensity and becoming refractory to analgesic therapy. Furthermore, two weeks later, the patient presented with bilateral weakness in the lower limbs and sensory impairments in the L2 region involving light touch and pinprick sensations. He did not experience any urinary and fecal incontinence. His vital signs were normal. The patient's medical history was unremarkable, with no significant prior illnesses, weight loss, or current use of medications. He confirmed that he did not consume alcohol and was a non-smoker. Subsequently, he was admitted to the neurology ward for further assessment and the baseline investigations including complete blood count (CBC), liver function tests (LFT), renal function tests (RFT), electrolyte levels, and vitamin B12 assessment were carried out. Table Upon a detailed neurological examination, the patient exhibited normal mental status and intact cranial nerve function. Motor examination revealed predominant motor weakness, with decreased strength noted in the hip flexors, knee extensors, ankle dorsiflexors, and toe extensors, graded as 4/5. His motor assessment suggested dysfunction in the descending corticospinal tract, indicating possible upper motor neuron involvement. Sensory examination revealed impaired sensation in light touch and pinprick testing, indicating decreased sensation to tactile stimuli, suggesting a disruption along the ascending spinothalamic tract. Deep tendon reflexes were slightly diminished, graded as 2/4, while Babinski reflex was present bilaterally. The Hoffmann test was negative, indicating no flexion or twitching of the thumb and adjacent fingers. The patient displayed a preserved anal reflex and there were no saddle paresthesia symptoms observed. Notably, the absence of saddle paresthesia, intact anal reflex, and negative Hoffman's sign suggested relative preservation of the sacral segments and associated nerve roots. Table Based on the extensive assessment of the patient's neurological examination, which encompassed motor, sensory, and reflex functions, along with a thorough review of the patient's medical history, there was a strong suspicion of an intramedullary tumor at the L2 level. In order to confirm this diagnosis and to exclude alternative potential differentials, such as demyelinating disorders like multiple sclerosis, inflammatory conditions such as transverse myelitis, B12 deficiency, Guillain-Barré syndrome (GBS), and central cord syndrome, a spinal magnetic resonance imaging (MRI) was conducted. The MRI revealed a well-defined, enhancing intramedullary lesion at the L2 level, as well as cord enlargement and edema. The tumor exhibited hyperintensity on T2-weighted imaging and isointensity on T1-weighted imaging, with varying enhancement on contrast-enhanced imaging. This is highlighted in Figure The MRI also revealed evidence of compression of the spinal cord at L2. Based on his physical examination findings, clinical presentation, and confirmatory imaging with MRI, the diagnosis of an intramedullary ependymoma at the L2 level was established.Following the diagnosis of an intramedullary ependymoma at the L2 level on MRI, a complete surgical resection was advised due to the lesion's limited dimensions. The surgery was a complete success with no intraoperative or immediate postoperative complications. Further microscopic examination of the specimen revealed the presence of neoplastic tissue organized in clusters and elongated processes. The neoplastic cells exhibited a cuboidal to elongated morphology and were conspicuously arranged around myxoid cores. Special staining with Periodic Acid-Schiff (PAS) with or without Alcian Blue (AB) demonstrated the presence of mucin within the myxoid cores. Immunohistochemical staining was performed, revealing positive reactivity for glial fibrillary acidic protein (GFAP) and focal positivity for epithelial membrane antigen (EMA). These findings are consistent with a diagnosis of intramedullary spinal myxopapillary ependymoma, grade II.Following the surgery, there was a noteworthy improvement in the patient's lower limb strength and sensation, enabling him to walk independently. The patient was under close observation for any possible signs of neurological impairment or adverse outcomes. There were no observed instances of new neurological impairments. An MRI was conducted postoperatively to verify the complete removal of the tumor and to evaluate the presence of any remaining or recurring tumor. Figure The patient also began a rehabilitation program to facilitate the recovery of both motor and sensory function as well as mitigate any potential complications such as deep vein thrombosis and pressure ulcers.Sustained surveillance is imperative for individuals with spinal cord tumors to detect any possible relapse or advancement of pathology, so a long-term follow-up was carried out using MRI, which was routinely performed at fixed intervals, usually every six to 12 months for three years following the surgery, with less frequency after the first year. Additionally, it was recommended that he follow up with his neurologist and receive occupational therapy and psychological assistance in order to enhance his recuperation and overall well-being.The underlying etiology of ependymoma development is not well known. These tumors are heterogeneous, exhibiting various subtypes and variations in cellular and clinical characteristics . MoleculSymptoms commonly associated with MPEs include lower back pain and lumbosacral radiculopathy due to compression of nerve roots . In thisThorough neurological evaluation and the use of imaging tests aid in the diagnosis of spinal ependymomas . Spinal MPEs have a high rate of local recurrence, with reports of post-operative recurrence ranging from one to eight years . MarchesIn the presented case, the patient had a small intramedullary ependymoma, and his symptomatic presentation, diagnostic imaging findings, and histopathological features all pointed to MPE. Spinal MRI confirmed the diagnosis and complete gross total resection and subsequent physical rehabilitation led to the complete resolution of his neurological symptoms. Close follow-up with MRI at regular intervals proved effective in ensuring the absence of tumor recurrence.Spinal myxopapillary ependymoma is a rare grade II CNS tumor characterized by perivascular degeneration and stromal mucin secretion. It is heterogeneous in nature and has a variety of clinical presentations including lumbosacral radiculopathy, bladder and bowel dysfunction, and cauda equina syndrome. Swift and appropriate diagnostic and therapeutic management are crucial to prevent permanent neurological consequences. Physicians should consider conducting a spinal MRI as the gold standard diagnostic tool when presented with symptoms associated with MPEs. It is worth noting that small, well-encapsulated tumors with mild preoperative neurological features are associated with a better prognosis and improved functional outcomes. Given the elevated recurrence rate of MPEs, strict post-resection surveillance with spinal MRI may be beneficial. Post-operative spinal MRI screenings can facilitate early detection and treatment of relapsed MPEs. Further research is needed to investigate the long-term benefits of MRI surveillance and its impact on patients' quality of life.


Uterine leiomyoma should be considered when a female patient reports symptoms of abdominal pressure and abnormal vaginal bleeding. However, the symptoms of a uterine leiomyoma are vast and overlap with other possible diseases that are difficult to distinguish even with imaging studies. This is why it is important for physicians and healthcare providers to keep an open mind and have a broad differential diagnosis. In this case study, we present a 61-year-old postmenopausal female patient who presented to the emergency department with complaints of pelvic and abdominal pain, as well as vomiting and diarrhea. She was admitted for observation. A complete blood count (CBC), comprehensive metabolic panel (CMP), and urinalysis revealed no abnormalities; a pelvic ultrasound and CT scan reported possible adnexal torsion. The patient remained stable and the pain had subsided when she was seen the next morning by her gynecologist (GYN) who discharged her to follow-up in the office. Subsequent examinations that aided in the diagnosis included, but were not limited to pelvic and transvaginal ultrasounds, an abdominal and pelvic CT, and a pelvic MRI. In this case, the MRI revealed an 11-cm mass that could represent a torsioned pedunculated necrotic fibroid originating from the uterus. Radiology recommended surgical removal. Upon removal and review of the pathology of the mass, it was revealed to be a torsioned, partially necrotic fibroma that had originated from the ovary and not from the uterus, as imaging had originally suggested. Ovarian fibromas are solid tumors that appear in ovaries, usually of benign histology (neoplasm), that are generally diagnosed in premenopausal and menopausal women . OvarianOvarian fibromas are usually asymptomatic and grow until detected during gynecological examination; in a retrospective study of 23 patients with ovarian fibromas, the average size was 14 cm . SometimThe differentiation of ovarian masses from uterine leiomyomas can be accomplished by localizing a mass. If the mass is found to be detached from the ovaries or adjacent to round ligaments, then ovarian etiology is improbable. In addition, the presence of bridging vascular signs, which are essentially vessels that extend from the uterus and supply pelvic mass, is indicative of uterine leiomyoma . In a prOccasionally, an ovarian fibroma may be pedunculated resulting in partial or complete twisting/rotation of the mass around its pedicle, restricting the arterial and venous flow . MRI finLaparoscopy is the standard procedure for dealing with benign adnexal masses that involves less hospital stay, quicker recovery time as well as less postoperative pain . HoweverA 61-year-old postmenopausal woman presented to the emergency department with complaints of intense pelvic and abdominal pain radiating to her lower back, vomiting, and diarrhea. The patient also had symptoms of nocturia, recurrent urinary tract infections, frequent urination, and female urinary stress incontinence. She had a family history of breast cancer, which her maternal aunt had in her 50s and her cousin had in her 40s. Her social history showed that she was a former smoker but was unremarkable in regard to exercise, diet, and stress levels. Her past medical history was significant for osteopenia and her surgical history was significant for a right kidney malignant mass removal in 2015 and a kidney stone operation in 1995.A complete blood count (CBC), comprehensive metabolic panel (CMP), and urinalysis revealed no abnormalities. The patient was admitted for observation by her gynecologist. A transabdominal pelvic/kidney ultrasonogram was negative for stones but revealed uterine fibroids, the largest of which measured 10.1 × 9.7 × 11.5 cm. The patient was feeling better in the morning and CBC, as well as vital signs, were stable. Further, because of a previous ultrasound, done 15 days prior to the emergency room (ER) visit, in which both ovaries were visualized, it was deemed that there was a low risk of ovarian torsion and that surgical intervention was not indicated  a few days after her emergency room visit with complaints of severe pain again in her lower abdomen, right groin, right buttock, and with occasional nausea. The decision was made to further explore and reevaluate the patient’s symptoms through supplementary testing. The patient underwent additional pelvic and transvaginal ultrasound, an abdominal and pelvic CT, and a pelvic MRI with and without intravenous contrast. The MRI findings revealed a solid mass measuring 10.5 × 9.5 × 11.3 cm anterior to the uterine fundus with numerous internal non-enhancing cystic spaces. The mass slightly abuts the anterior uterine fundus but appears to cause mass effect on the uterus. Immediately inferior to this 11 cm mass, a tubular cystic lesion measuring 3.2 × 5.2 × 5.3 cm was found. The uterus was also found to be anteverted, slightly anteflexed, and normal-sized. The findings also included a right ovarian cyst measuring 1.1 cm. The 11 cm solid mass was presumed to represent a pedunculated partially necrotic fibroid that had torsed and disconnected from the uterus. Radiology recommended surgical resection.The preoperative diagnosis was uterine leiomyoma. The patient was scheduled for a total abdominal hysterectomy with removal of tubes and ovaries, excision of adnexal mass, uterosacral colposuspension, lysis of ovarian cyst, and an exploratory laparotomy. The procedure was conducted under general anesthesia. Visualization of the pelvic organs showed a partially torsioned right adnexal mass, measuring 14 × 10 × 10 cm with many areas of cystic degeneration and hemorrhage as well as a cyst with two pieces of smooth hemorrhagic friable tissue measuring 2.5 × 2 × 0.5 cm  with symptoms of minimal vaginal bleeding for five days . A pelviAs with most cases involving abdominal pain, ultrasonography imaging is the initial emergent examination for diagnosis  but ofteMRI is widely used in the diagnosis of gynecological disorders because it depicts a higher resolution relative to CT and ultrasound . In testIn this case, MRI incorrectly identified the fibroma as originating from the uterus instead of the ovary, whereas additional CT scan suggested an ovarian fibroma, which was confirmed in surgery. Therefore, CT scans are also accurate in the diagnosis of the origin of ovarian fibromas. CT is preferred over MRI because it is often more available and quicker. MRI and CT share a similar staging accuracy of 70-90%, but given MRI’s high cost and relatively long examination times, CT is the recommended imaging modality of choice for staging ovarian cancer . CT is eAlthough this case did not use a coronal reformation, CT was still able to detect a twisted pedicle and consequently accurately diagnosed the mass as an adnexal torsion originating from the right ovary. It is evident that the accuracy of these scanning methodologies varies, and MRI and CT are both useful while the results of the ultrasound are often inconclusive and ambiguous. A combination of initial ultrasound and further MRI and CT may be used for the diagnosis of ovarian fibromas but, because of the varying accuracy of these scans, only after exploratory surgery can a definitive conclusion be made.This study described the symptoms, pre and postoperative diagnosis, and the various treatments correlated with ovarian fibromas. It specifically discusses a female who presented to the emergency department with what appeared to be symptoms of a degenerating uterine leiomyoma but ultimately ended up being diagnosed with a torsioned ovarian fibroma. In order to avoid adverse outcomes which can lead to further complications, it is important for physicians to consider various differential diagnoses in order to properly treat the patient for any and all ailments the patient may present with. While surgery is not used as an initial diagnostic method, it is clear that imaging only provides a certain level of accuracy. Due to these limitations, only after surgery can a definitively accurate diagnosis be established.


Encephaloceles are relatively uncommon in western countries. Most of the reported cases involve occipital encephaloceles. Open frontal encephaloceles comprise a rare entity. Most of them will be detected during early prenatal diagnostic, whereas the majority of the pregnancies will be terminated after the consent of the parents. Open frontal encephaloceles pose a great challenge to neurosurgeons as well as anesthesiologists, as these infants present with a microcephaly, non-physiological intracranial anatomy, and low birth weight, thus making the infant prone to excessive blood loss, hypothermia, and death. Neonates born with an incomplete cutaneous coverage are exposed to an imminent threat to life due to the risk of meningitis, necessitating surgical repair in the first days of life. We represent a rare case of an open nasofrontal encephalocele managed surgically in the first day of life. Surgery did not influence the neurological outcome of the patient. Encephalocele is usually a congenital type of neural tube defect (NTD), where a sac containing brain/meninges/cerebrospinal fluid (CSF) forms outside the skull through a bone defect. On occasions, acquired, encephaloceles may result from trauma, tumors, or iatrogenic injury. If the sac is formed by protrusion of meninges and CSF, it is appropriately called a meningocele, but it is called an encephalocele when it contains brain tissue . EncephaSurgical repair of the encephalocele does not require always an immediate surgical intervention. In the case of an incomplete cutaneous coverage, a rapid surgery is required due to the high risk of meningitis , 4–8. ApFrontoethmoidal encephaloceles are extremely rare, with an incidence of 1 in 35,000 live births , 5, 12. Our male patient was born at 35 weeks + 6 days. During the fetal malformation scan, the family was informed about the encephalocele and decided to continue with the pregnancy. The delivery was performed by a caesarean section. The baby was immediately transferred to our University Hospital and the defect was secured in sterile covers. The patient was admitted in a stable, awake condition. The pupils were fully dilated and irresponsive to light. There was no apparent neurological deficit. We performed preoperatively an MRI and MR-angiography Figs.  and 2, rWe present the second case of an open frontonasal encephalocele with pre and post MRI imaging. According to the pathogenesis, encephaloceles could be divided to primary and secondary . Although cases have been recorded since the sixteenth century, there is still a scarcity of knowledge on the exact causes and factors associated with the development of the disease. Many studies have determined these to be caused by a combination of genetic and environmental factors . As mentWe present the second case of an open nasofrontal encephalocele, urging to a surgical intervention within 24 h after birth. Despite the early surgical intervention as well the uneventful postoperatively course, the detrimental neurological developmental delay could not be influenced.