Solitary metachronous small bowel metastasis from renal cell carcinoma (RCC) is rare. In contrast to idiopathic intussusception frequently occurring in children, adult intussusception is fairly uncommon and usually indicates a malignancy.We presented an 84-year-old man with small bowel intussusception and obstruction due to a solitary metachronous metastasis from RCC. Computed tomography with intravenous contrast revealed small bowel obstruction and a 4 × 4 cm intraluminal soft-tissue mass with moderate enhancement. During urgent exploratory laparotomy, a pedunculated tumor of the distal ileum was found to be the lead point of intussusception. Hence, reduction of the intestinal invagination and segmental resection of the ileum with functional end-to-end anastomosis were performed. Histological examination finally confirmed the diagnosis. The postoperative recovery was uneventful. The patient was discharged without any complications on postoperative day 6.The case report highlights the rarity of solitary metachronous small bowel metastases from RCC and suggests that life-long follow-up of RCC patients is critical due to its unpredictable behavior and the possibility of a long period of dormancy. Complete surgical resection remains the mainstay treatment for such patients. Renal cell carcinoma (RCC) is the third most frequently diagnosed cancer among urological tumors, with an estimated annual incidence of 0.4 million cases worldwide . RCC is en bloc resection of the oligometastatic disease is still a curative option for late RCC metastasis (≥ 2 years)  .Adults have intussusception less frequently than children, accounting for less than 5% of all intussusception and 1% of bowel obstruction cases . The mosFollowing the principles of the CAse REport (CARE) guidelines , we repoIn September 2021, an 83-year-old male patient presented to the emergency department with intermittent abdominal pain and distention accompanied by cessation of passage of flatus and stool for 3 days. The concurrent medical problems included type 2 diabetes mellitus and pulmonary emphysema for more than 30 years. More significantly, he underwent left radical nephrectomy for ccRCC  5 years previously. The scheduled four cycles of adjuvant therapy with tyrosine kinase inhibitor sunitinib were discontinued because of patient intolerance. Computed tomography (CT) and serum tumor marker levels showed favorable local control and no signs of distant progression during intensive surveillance. The last follow-up visit was just 6 months before this admission.; reference range, 13.0 – 17.5 g/dL) and an elevated percentage of neutrophils . CT of the chest and abdomen with intravenous contrast demonstrated small bowel obstruction due to intussusception of the distal ileum containing an approximately 4 × 4 cm intraluminal soft-tissue mass with moderate enhancement  3 and discharge on POD 6. The patient received no additional treatment due to his venerable age and underlying diseases. To date, the 1-year follow-up has been uneventful. The timeline with relevant data from the episode of care is shown in via the following immunohistochemical results: PAX-8 (+), CD10 (+), EMA (+), CK7 (-), CAIX (+), FH (+), SDHB (+), TFE3 (-), and Ki-67  , and Cochrane Central Register of Controlled Trials databases using medical subject headings and text words related to “solitary metachronous small bowel metastasis from RCC” to obtain relevant case reports published through 10 May 2022. The term “solitary metachronous small bowel metastasis from RCC” was defined as the small bowel metastasis diagnosed more than 6 months after partial or radical nephrectomy for localized RCC. The search strategy and syntax for the PubMed database are shown in the Supplementary material. Papers with non-English language, animal subjects, or unavailable full text were excluded. The general characteristics of the included studies were extracted and entered into a preplanned electronic form Table 1.Finally, 48 potential studies were included for the evaluation, in which 21 cases with solitary metachronous small bowel metastases from RCC that had been reported between 1952 and 2020 met the inclusion criteria , 36, 37.via the renal vein and inferior vena cava before spreading systemically to multiple organs (EWSR1 gene fusions are essential to confirming the diagnosis (Ileal metastases from RCC were rather uncommon in our literature review when compared to duodenal and jejunal metastases. Although some subtypes might exhibit regional lymph node involvement, RCC is notable for hematogenous metastasis . Viadanae organs . Howevere organs . Besidesiagnosis .Small bowel metastases from RCC exhibit various biological characteristics . We founThe significance of radiological screening should be emphasized in patients with atypical symptoms and an RCC history. Small bowel series is inferior to CT as a practical and effective tool in assessing suspicious small bowel lesions, especially in the emergency setting . CapsuleSystemic treatment should be considered for individuals with medically inoperable RCC . SeveralMeanwhile, complete metastasectomy of synchronous or metachronous solitary RCC metastasis could improve the patient survival . Thus, aIn conclusion, our case suggests that life-long follow-up of RCC patients is critical due to unpredictable tumor behavior and the possibility of a long period of dormancy. Complete resection of the solitary metachronous small bowel metastasis from RCC is indicated as a life-saving procedure and a curative treatment to cure metastasis-related complications. The present case report and review of the English-language literature emphasizes the rarity of this entity and recommends aggressive and tailored surgical treatment for these patients.The original contributions presented in the study are included in the article/Supplementary Material. Further inquiries can be directed to the corresponding author.The studies involving human participants were reviewed and approved by Ethical Committee on Biomedical Research, West China Hospital, Sichuan University. The patients/participants provided their written informed consent to participate in this study. Written informed consent was obtained from the individual for the publication of any potentially identifiable images or data included in this article.LY, WY, ZC, and PN conceptualized and designed the study. WY and ZC drafted the manuscript. TY and HZ were responsible for the literature search. QD, SQ, and JZ collected the clinical data. All authors contributed to the article and approved the submitted version.


Diabetic striatopathy (DS) is an acute hyperkinetic movement disorder characterized by hemiballismus-hemichorea (HBHC) due to nonketotic hyperglycemia. DS manifests a fascinating interplay between endocrinopathy (diabetes), striatal  pathology, and a dramatic neurological movement disorder, HBHC. The striking hyperintensity on imaging modalities such as computed axial tomography (CT) scan of the brain and T1-weighted magnetic resonance imaging (MRI) of the brain can mislead the clinician to an erroneous diagnosis of a cerebral hemorrhage and/or ischemic infarct, especially in an acute setting. We present an acute case of DS and outline the natural history, semiology, typical radiological findings, and therapeutic options. With careful and thoughtful analysis, an accurate diagnosis can be exacted, sparing the patient unnecessary anxiety and medical costs. Diabetic striatopathy (DS) is an intriguing acute hyperkinetic movement disorder secondary to nonketotic hyperglycemia. DS is a rare disorder with an estimated prevalence of 1 in 100,000, which is likely an underestimation due to unfamiliarity and missed diagnosis . Its sinWe present the case of 51-year-old man with poorly controlled diabetes mellitus and a two-month history of continuous and involuntary motion of the left upper and lower extremity, sparing the tongue, face, and the neck. These movements did not abate with sleep. His speech was unaffected, but the cadence and smoothness of his gait was interrupted by the incessant motion. His past medical history was significant for chronic hypertension and he had at least a 10-year history of diabetes mellitus with poor adherence to anti-diabetic medications. There was no family history of chorea, and he did not smoke cigarettes or drink alcohol. His medications included metformin, metoprolol, pravastatin, and valsartan.2. On initial inspection, his examination was notable for high amplitude involuntary dyskinesias of the axis (trunk), left upper extremity, and left lower extremity. Athetotic and torsional movements of the left shoulder, elbow, wrist, hip, and ankle were noted , and his hemoglobin A1C was greater than 14% . The basic metabolic panel was otherwise normal. Serum and urine ketones were not measured. A T-1 weighted MRI study of the brain revealed a hyperintensity signal involving the right putamen and caudate nucleus (Figure Based upon the absence of a family history of chorea, the characteristic clinical history of chronic poorly controlled diabetes, and the characteristic MRI brain findings, a tentative diagnosis of DS was rendered. The patient was referred to an endocrinologist for better blood sugar management, and a trial of tetrabenazine at a dose of 12.5 mg twice daily was prescribed to reduce the amplitude and frequency of the disabling choreoathetosis. The patient demonstrated substantial improvement of the movement disorder in one month.Acute HBHC is a defining clinical characteristic of DS. However, diagnosing DS is not always straightforward as there are many differential diagnoses of HBHC. Therefore, a structured approach should be taken to diagnose DS. We propose a two-fold method that involves running a differential diagnosis of HBHC and addressing the causes of MRI T1 hyperintensity of the striatum.The possible causes of HBHC include metabolic, infectious, inflammatory, iatrogenic, autoimmune, and neurogenetic/neurodegenerative etiologies . While tThe list of differential diagnoses can be further narrowed down by considering the causes of T1 hyperintensities of the striatum on the brain MRI. According to the biophysics of T1-weighted MRI sequences, T1-shortening leading to hyperintensities can be induced by melanin, methemoglobin in subacute hemorrhage, fat, slow-velocity blood flow, high protein content, and paramagnetic transition metals with unpaired electrons such as manganese, iron, zinc, and copper . ClinicaAs can be seen, this two-fold approach will leave high protein content as the cause of the MRI T1-hyperintensity, which confirms the diagnosis of a true DS. The high protein density is accounted for by the gemistocytes involved in the pathophysiology of DS. Gemistocytes are swollen reactive astrocytes involved in acute brain injury, and their cytoplasm is enriched with a protein hydration layer which restricts the motion of water molecules by electrostatic repulsion, thereby shortening the T1-relaxation time .DS is a rare hyperkinetic movement disorder associated with poorly controlled diabetes and characterized by HBHC. The diagnosis of DS can be complicated by the many possible causes of HBHC. A two-fold approach that narrows down the possible diagnoses based on the patient’s clinical presentation and the brain MRI characteristics can be an effective tool for the diagnosis of DS. Future research endeavors should focus on establishing a standardized protocol for DS diagnosis.


Dedifferentiated liposarcoma (DL) of the ovary is a rare mesenchymal tumor which is difficult to diagnose before surgery. The diagnose of DL was depended on the morphology, Immunohistochemical stains, and fluorescence in situ hybridization after the complete surgical resection.We report a case of 63-year-old postmenopausal female who presented with a large pelvic mass and was initially thought to be an original ovarian tumor. However, it was subsequently considered to be a DL arising from the mesentery and developing in ovary.Ovarian DL.Bilateral salpingo-oophorectomy.Until now, there is no recurrence symptoms represented in the patient after surgery for 12 months.DL happening in ovary is rare. In this case, the exact origin of the mass still remains ambiguous because of the lack of morphological evidence. Although retroperitoneum is the most common area of DL origin, we for long suspect that the mass was initially derived from mesentery and developed in ovary. A long-term may help confirm the exact origin of the tumor. Complete surgical resection plays an important role in the treatment. Liposarcoma, arising from adipose cells, is the most common soft tissue sarcomas, with approximately 52% occurring in the extremities, 42% developing in the retroperitoneum (RP), and 6% deriving from the head and neck. The histological classification of liposarcoma could be divided into five groups: well-differentiated liposarcoma , marked by ring or long chromosomes; myxoid liposarcoma; round cell liposarcoma, characterized by reciprocal translocation t; pleomorphic liposarcoma, characterized by complex karyotypes; and dedifferentiated liposarcoma (DL).–5Soft tissue sarcomas are a class of rare malignant tumors originating from mesenchymal tissues.,3 Commonly, DL was reported to occur in the RP, while an ovary location is extremely rare. The clinical outcome of DL remains controversial. A previous study found that DL is less aggressive than that in other high-grade pleomorphic sarcomas. However, DL was considered as a disease with aggressive behavior and poor prognosis in recent studies. The prognostic factors of DL are also related to tumor size, anatomical location, and surgical resection.,7DL, a variant type of liposarcoma, was featured as a combination of low-grade nonlipogenic morphology, high-grade nonlipogenic morphology, and well-differentiated liposarcoma. Moreover, the transition from low-grade to high-grade nonlipogenic morphology could also be observed.We reported herein a case diagnosed as DL that occurred in ovary showing an amplification of the murine double minute 2 (MDM2) gene as well as the high expression of other DL related proteins.We present the following case in accordance with the CARE reporting checklist. The study was approved by the Ethics Committee of the Second Hospital of Jilin University . Written informed consent was obtained from the patient.A 63-year-old postmenopausal female visited the department of Obstetrics and Gynecology of Jilin University Hospital with complaints of a palpable pelvic mass found by herself 19 days prior. The patient was married at the age of 23 and had a history of gravida 2 and para 1. She suffered from a resection of brain tumor in 2000, a hysterectomy in 2011, and a resection of brain tumor because of recurrence in 2019. On gynecological and pelvic examination, an approximately 20 gestational week-sized mass, with restriction mobility, irregular form, and unclear boundary, was palpable in the meso-pelvic cavity. There were no laboratory abnormalities. The tumor markers such as Pro-gastrin-releasing peptide, alpha fetoprotein, cancer antigen 125, cancer antigen 199, and human epididymal protein 4 were within their normal ranges.The mass was suspected as an ovary-derived tumor based upon pelvic contrast-enhanced computed tomography (CT) and abdominal ultrasonography. The ultrasound revealed a hypoechoic and irregular solid tumor with blood flow signals in the edge, measured approximately 17.0 × 9.3 cm, occupying the middle of the pelvic cavity  stains for CK AE1/AE3), ER, PR, Calretinin, epithelial membrane antigen, S100 calcium binding protein B, ALK, α-inhibin, and mucin 4 were negative, while MDM2, cyclin dependent kinase 4 (CDK4), cyclin dependent kinase inhibitor 2A (P16), INI-1, BRG-1, WT1, and CD10 were positive. Some other related molecules, such as smooth muscular actin, DES, Ki67, CD34, CD99, PBH3, and CyclinD1, showed weakly positive. Part of representative pathology images were showed in Figure , ER, PR,After 15 days of surgery, positron emission tomography-CT (PET)-CT was performed to evaluate whether there are metastasis lesions or remnant tissues. The report of PET-CT demonstrated that fuorodeoxyglucose intake was slightly increased in pelvic medial abdominal wall, left pelvic peritoneum, and median abdominal wall, which was considered as postoperative inflammation. There were no metastasis lesions on the other organs. Moreover, there was no abnormal fuorodeoxyglucose and swollen lymph node in the retroperitoneal Fig. .DL is a subtype of liposarcoma, which was rarely reported to develop in ovary. In this case, because there were no characteristic symptoms and laboratory results, it is difficult for us to confirm the properties of the mass. Although preoperative ultrasound and CT revealed that the mass was located in the pelvic, its exact position and origin remained unclear. Accurate diagnosis still demands on the operation and pathology. A few cases reported that DL could be found in peritoneum, particularly in mesentery or sigmoid mesocolon. One case has reported an ovary-derived DL which, however, was subsequently suspected to be a retroperitoneal DL. In this case, the exact origin of the mass still remains ambiguous because of the lack of morphological evidence. Although RP is the most common area of DL origin, we still highly suspected that the mass was initially derived from mesentery and developed in ovary. The reasons for this suggestion are as follows: during the laparotomy, the mass seemed to adhere densely to the intestinal tube and mesentery although its smooth capsule was complete; after separation of adhesion, we found that the mass and right ovary have the same root which connected to the infundibulopelvic ligament; postoperative PET-CT did not find any suspicious metastasis or remnant lesions in the RP. However, a long-term follow-up may help confirm the exact origin of the tumor. Because there is no treatment guideline for differentiated liposarcoma happening in ovary, we just prudently referred the patient for chemotherapy and radiotherapy after the surgery. However, the patient refused our suggestion and just chosen to follow-up timely. Until now, there is no recurrence symptoms represented in the patient after surgery for 12 months.During the surgery, we thought that the mass had a close relationship with the right ovary as well as the mesentery. To previous reports, most of DLs are derived from RP and pelvic soft tissue, followed by limbs, spermatic cord, head, and neck.Actually, this case should be distinguished with well-differentiated liposarcoma with myxoid changes and myxoid liposarcoma. The basics of differential diagnosis are as follows: The differential diagnosis from well-differentiated liposarcoma with myxoid changes. After being carefully observed, neither typical well-differentiated liposarcoma ingredients nor at least piece distribution of adipose tissue and adipoblast were found in any slices. Histologically, it showed basically the same spindle cell sarcoma morphology, and mucilaginous degeneration of the stroma is considered as a nonspecific change. These characteristics did not accord with the diagnosis of well-differentiated liposarcoma; the differential diagnosis from myxoid liposarcoma. In this case, although the stroma was myxoid, there was neither adipoblastic component nor characteristic plexiform or branched vessels, which did not support the diagnosis of myxoid liposarcoma. The amplification of MDM2 gene identified by FISH in this case was not a specific genetic change of myxoid liposarcoma.,9 Immunohistochemistry and FISH are two important tools in the diagnosis of DL. The combined detection of CDK4, MDM2 and P16 plays an important role in the definite diagnosis. Identifying MDM2 amplification by FISH was considered as a golden standard for diagnosis of DL. Importantly, this case presented spindle cell sarcoma with a few pleomorphic cellular components. Although no typical well-differentiated liposarcoma component was observed, MDM2, CDK4 and P16 IHC staining were diffusely positive, and MDM2 gene amplification, identified by FISH, supported the diagnosis of DL. Considering that in some cases of DL featured as completely sarcomatous, where only few or no well-differentiated liposarcoma components could be found, the absence of well-differentiated liposarcoma components cannot rule out the diagnosis of DL. Moreover, this case has no characteristic histological morphology. After IHC staining, the possible sources of epithelial origin, smooth muscle and striated muscle origin, peripheral nerve origin, and ovarian sex cord mesenchymal origin were excluded, The other tumors were basically excluded by the results of IHC staining.Microscopically, DL was commonly formed with two major components: highly differentiated liposarcoma and dedifferentiated components. However, there is no obvious transition process and the boundary is clear.Due to the low incidence of ovarian DL, its exact origin and pathogenesis are still unclear and needed more clinical case report to further discuss. However, this case indicated that complete surgical resection still played an important role in the strategy of treatment, and could be applied in the similar patients in the future. Furthermore, the necessity of chemotherapy and radiotherapy in the disease needs more clinical cases to determine.JC, TW, MW, and SZ wrote the main manuscript text. LD and YX prepared Figures Conceptualization: Junyu Chen, Lianying Ding, Taiwei Wang, Mengqi Wang, Shuhua Zhao, Yang Xia.Data curation: Junyu Chen, Lianying Ding, Taiwei Wang.Formal analysis: Junyu Chen, Lianying Ding.Methodology: Junyu Chen.Supervision: Shuhua Zhao, Yang Xia.Writing – original draft: Junyu Chen, Lianying Ding, Taiwei Wang, Mengqi Wang, Shuhua Zhao.Writing – review & editing: Taiwei Wang, Mengqi Wang, Shuhua Zhao, Yang Xia.


Background: Mediastinal hemangiomas are rare, and their etiology remains unclear. Most patients affected have no pathognomonic clinical symptoms, and the diagnosis is often incidental. Due to the paucity of the available literature regarding the management of this disease, the choice and timing of treatment remains controversial. Case presentation: Herein, we report the case of a hemangioma of the azygos vein arch in a 66-year-old woman who presented with dyspnea, chest discomfort, dysphagia, and weight loss. A simultaneous right chylothorax refractory to conservative management was found. A CT-guided biopsy of the mass was performed, and it confirmed the vascular nature of the lesion. Therefore, the patient underwent an angiography followed by endo-vascular embolization. Three days later, thoracoscopic surgical resection of the mass and the repair of the chyle leakage were performed safely. The patient was discharged uneventfully on postoperative day seven, with complete resolution of all the presenting symptoms. Conclusions: Treatment of symptomatic mediastinal hemangiomas could be mandatory, but a thorough multidisciplinary approach to these rare malformations is essential. Despite the risk of intraoperative bleeding, selective endovascular embolization followed by thoracoscopic surgery allowed for a complete and safe resection with a good outcome. Mediastinal hemangioma of the azygos (MHA) is a rare tumor that accounts for only 0.5% of mediastinal masses . HistoloVenous malformations, tumors, portal hypertension, or local thrombosis could be related to the occurrence of these lesions. However, a specific etiology has not yet been identified ,4.Patients presenting with MHA are mainly asymptomatic, although clinical signs and symptoms may result from compression of nearby structures . The optThis case was reported to describe and discuss the strategy used for diagnosing and treating a rare arterio-venous capillary hemangioma of the azygos arch, with uncommon clinical presentation.A 66-year-old lady was admitted to the emergency medicine department of our institution due to worsening dyspnea associated with palpitations, dry cough, and dysphagia.Past medical history included smoking habits, hypertension, and breast fibroid.A computed tomography scan (CT) of the chest was performed. It showed the presence of a sizeable right-sided posterior mediastinal mass close to the azygos vein without a clear dissection plane, and an approximate size of 5.5 cm × 6.7 cm × 7.5 cm on axial plane. A large ipsilateral pleural effusion was also present.In the first instance, a chest drain was inserted, resulting in drainage of milky fluid, confirmed to be chyle with the laboratory tests .The radio-metabolic study with 18-fluorodeoxyglucose positron tomography (FDG-PET/CT) showed a delayed enhancement in the central mass and faint uptake in its periphery with a standardized uptake value max of 0.7. Based on this evidence, a CT-guided biopsy was performed with uneventful results, and the pathology report was compatible with venous hemangioma .Due to the severity of the symptoms and the persistence of high chylous drain output despite the fat-free diet with total parental nutrition, the case was discussed in a multidisciplinary team (MDT) setting to establish the best-tailored treatment option. The agreed strategy included an angiographic study for possible preoperative embolization treatment, followed by surgical excision.® 5 Fr. Cobra catheter and Progreat™ 2.7 Fr. by Terumo, Terumo Corporation, Tokyo, Japan), administering microparticles containing 250–500 µm of Polyvinyl Alcohol (PVA). Two platinum coils were then placed to complete the embolization (A new enhanced CT scan revealed two intercostal arterial branches supplying the MHA , and thelization .Seventy-two hours after the procedure, the patient underwent right video-assisted thoracoscopic resection of the mass using a 4-ports approach. The choice of port placement depended on the posterior location of the mass: one port at the seventh intercostal space on the anterior axillary line, and two at the eighth intercostal space on the middle and posterior axillary lines, respectively. A fourth port was placed anteriorly at the fifth intercostal space to grasp and move the lung when necessary .The esophagus was displaced anteriorly, and dense adhesions were present between the mass and descending aorta and between the mass and vertebral bodies. Several venous and arterial feeding vessels were controlled using a harmonic scalpel and titanium clip. The main pedicle connecting the mass to the azygos vein was divided with a standard mechanical stapler, and the mas was detached, preserving the azygos vein completely. The MHA was then removed into a 15 cm endo-bag through the anterior utility port .Then, the chylothorax was carefully assessed, and a leak in the thoracic duct was found in the resected bed of the mass. This was interpreted as a direct injury caused by the compression of the lesion, and it was controlled with titanium clip ligation. To prevent a recurrence, chemical pleurodesis through pleural painting with iodopovidone solution was also performed . An oxidFinal histology confirmed the diagnosis of capillary hemangioma .The patient recovered uneventfully and was discharged home on postoperative day seven.At one year follow-up, no symptoms were present, and the CT scan of the chest showed no evidence of MHA recurrence or pleural effusion .Despite the availability of reports on azygos vein arch malformations, the related subcategory of venous tumors is extremely rare, particularly capillary hemangiomas . Very feOur case report is also the first to describe refractory chylothorax secondary to a decubitus injury on the lymphatic line as a complication of mass effect. Furthermore, given the high risk of intraoperative bleeding, we are the first to report the procedure of precautionary vascular embolization of MHA before surgical treatment.The first report from Yamanaka et al.  underlinIn view of the various clinical presentation, MHA is often confused with fibromas, paragangliomas, lymphomas, and bronchial cysts ,10,11,12Preoperative tissue diagnosis by CT-guided biopsy or endoscopic ultrasonographic fine needle aspiration could validate MDT assessment in carefully selected cases when the non-invasive investigations are inconclusive ,18. In oLipiodol-based percutaneous fluoroscopic lymphangiography can demonstrate whole lymphatic channels and intragenic leaks with a technical success rate of 75% to 100% . AlthougMediastinal hemangioma is relatively benign, and therapies are still being debated. Moreover, there is a lack of guidelines or therapeutic flow charts that clearly define the indication for conservative, surgical, or endovascular treatment . TherefoSome cases of MHA have been successfully treated with embolization of the lumen with coils, covered stents implantation, or Amplatzer vascular plugs ,21,22. HInterventional radiology can undoubtedly be helpful when the resection of a deep MHA becomes dangerous due to the inability to safely control bleeding, especially if an arterio-venous malformation is present . This waThe decision on surgical approach is also affected by improved safety of hemostasis of minimally invasive surgery. Indeed, video-thoracoscopic contributed to excellent postoperative outcomes and enhanced recovery ,26,27,28In our case, the prolonged postoperative stay was caused by common reactions to chemical pleurodeses, such as fever and the permanence of pleural drainage due to a large amount of serum output. As the reports in the literature also show ,31,32, tAlthough the complete resection of MHA has a good prognosis, and the disease is rarely malignant, a follow-up is needed within at least one year not to ensure no recurrence .Treatment of vascular neoformations of the azygos vein and concomitant complications is often challenging and demanding. Preoperative planning must be thorough and discussed in an MDT to assess risks and benefits. Even in cases of complex arterio-venous MHA, thoracoscopic surgery preceded by selective vascular embolization techniques appears to be a safe and effective treatment.


Agents of platinum-based chemotherapy, such as cisplatin or carboplatin, are used in the treatment of a wide range of malignancies that affect children, such as brain tumors, osteosarcoma, neuroblastoma, hepatoblastoma, and germ cell tumors (GCTs). The Cyclophosphamide Equivalent Dose (CED) calculator for reproductive risk does not take platinum-based chemotherapy into account, despite the fact that it accounts for the majority of chemotherapy medications that are typically administered for pediatric GCTs. As a result, exposure to platinum-based drugs throughout infancy can have predictable long-term effects such as infertility, as well as other rare encounters such as lipoma formation and lipomatosis. Lipomas are the most prevalent benign soft tissue tumor subtype. They may be either solitary entities or engaged in multiple lipomatosis, which may have a familial origin or be an acquired disorder. Chemotherapy is a possible cause of lipomatosis. Chemotherapy based on cisplatin has been linked to a variety of long-term consequences, including kidney damage, neurotoxicity, and pulmonary toxicity, and may even create secondary cancers. However, lipoma development is known to occur in fewer than 1 in 100 individuals, and only a few examples of multiple cutaneous lipomatosis triggered by this therapy have been documented. Here we present a very rare case of lipomatosis in a pediatric patient with GCT under cisplatin therapy, which might be the third report of this kind affecting children. Lipomas are benign, most often encapsulated, noninvasive soft tissue tumors composed of mature adipocytes. They represent about half of all the adipocytic tumors in the pediatric population and, depending on their size and location, and they vary from being asymptomatic to causing various non-specific symptoms. Cytogenetic aberrations can often be described in these tumors, most of them affecting the chromosome 12q. The term “lipomatosis”, on the other hand, describes tumors comprised of matured adipose tissue with an infiltrative growth pattern, ill-defined margins, and a tendency toward symmetry .While the cause of these tumors remains mostly unknown, there are some etiologically and clinically described subtypes of lipomatosis, such as Madelung’s disease associated with alcohol consumption and metabolic problems; lipomatosis dolorosa, potentially associated with improper lymph drainage; or Dercum’s disease and Roch–Leri mesosomatous lipomatosis, which are considered very rare lipomatoses of unknown etiology ,5. ThereIn this case report, we present a very rare case of a 16-year-old girl diagnosed with a giant ovarian GCT of mixed histology with extension into the abdominal cavity and asymptomatic amputation of the contra-lateral ovary, who developed lipomatosis after chemotherapy with cisplatin. A thorough literature search was also performed to find similar cases, since this rare occurrence of peritoneal and liver lipomas as a possible and specific side effect of platinum-based chemotherapy is scarcely described in pediatric patients.2, being in the 95th percentile for girls her age, corresponding to class I obesity. The abdomen was distended and moderately painful upon palpation, the puddle sign was present, and percussion notes varied between tympanic and dull, raising suspicion of ascites. Lab values were significantly modified, suggesting a severe inflammatory process, as described in A 16-year-old Caucasian girl was admitted to our hospital, presenting with abdominal pain and abdominal distension, fever, and a dry cough. Upon clinical examination, the patient was found moderately pale and febrile, with a body mass index (BMI) of 28.6 kg/mIt was observed that CRP levels were 3215 mg/l, ferritin 1118 ng/mL, fibrinogen 629 mg/dL, and ESR was 92 mm/h, all of which ranged much higher than the normal threshold. Besides the severe inflammatory status of the patient, the girl was also suffering from anemia, with a red blood cell count (RBC) measured at 4.0 million, and a hemoglobin level of 10.5 g/dL. Apart from class I obesity, the adolescent patient had an elevated serum glucose (109 mg/dL), correlating with a metabolic syndrome. The serum lipid levels were also above the normal range for adolescents, although without a significant increase . The patient did not have any documented endocrinological problems.A chest X-ray found bilateral interstitial reticular coarseness and opacification of the right costophrenic angle, suggestive of pleural effusion. The patient was further investigated in our ultrasound department; the abdominal sonogram raised suspicion of an abdominal–pelvic tumor mass with mild ascites in the abdominal cavity, particularly pronounced in the perihepatic and peri-splenic spaces.Surgery was considered and computer tomography (CT) was performed on the same day to better assess the extension of the tumor mass. The CT examination of the abdominal and pelvic cavities found a gigantic heterogeneous mass of 18/13/9 cm . The tum3 from the tumor originating in the contralateral ovary. The patient was transferred to our intensive care unit for post-surgery monitoring awaiting the pathology and cytology results.The major findings could be summarized as massive mixed solid-cystic tumors occupying the abdominal and pelvic cavities of suspected ovarian origin, abdominopelvic ascites, minimal right pleural effusion, and para-aortic lymphadenopathy. The patient underwent surgery on the third day of hospitalization. A midline laparotomy found peritoneal fluid that was drained and sampled for cytology and a massive septated cystic tumor occupying the abdominal and pelvic cavity with left ovarian origin. A surgical biopsy was performed along with ablation of the ovarian tumor and epiploectomy. Upon further inspection of the pelvic cavity, a calcified amputated right ovary of approximately 3cm in diameter was found, most likely a sequela of compression due to the massive volume of 1101.43mmThe cytologic examination of the peritoneal fluid identified hemorrhagic fluid with high heterogeneous cellularity of 75% neutrophils, 15% monocytes, 10% lymphocytes, reactive mesothelial cells, and several old mesothelial cells with “signet ring cell” phenotype, raising suspicion of peritoneal dissemination. Pathology findings revealed on macroscopic examination the tumor appeared as a round, encapsulated multi-cystic mass of 18.5/13.8/9.5 cm, with grey to light pink solid/gelatinous components. Immunohistochemistry (IHC) analysis found a glial fibrillary acidic protein (GFAP), alpha-fetoprotein, CD10, macro creatine kinase (MCK), and vimentin, all intensely positive, beta-HCG-negative, and KI-67-positive in 10% of solid tumor areas ,17,18.The pathology findings identified the tumor as a mixed germ cell tumor with three components, as observed in A second pathology examination was requested, and the result indicated a high-grade immature teratoma. The final diagnosis was set after careful consideration of clinical, paraclinical, histopathological, and IHC data. The tumor was labeled stage IIIC according to the FIGO staging system that identified peritoneal metastasis , and a hTherapy and follow-up: the patient underwent chemotherapy treatment (MAKEI 2005 protocol) with four PEI cycles . The chemotherapy was well tolerated with no significant events, and complete tumor remission was accomplished after the completion of the four PEI cycles. Shortly after completing the last cycle of chemotherapy, a routine MRI showed elements previously absent four months prior: a small oval peripheral intrahepatic mass of ~0.8 cm under the Glisson capsule and several smaller cysts in the perihepatic space. A PET scan was further performed and found no signs of malignancy but identified the liver mass as a possible lipoma. The tumor markers AFP and beta-HCG were within normal values; thus, a “watch and wait” approach was decided upon.2), advancing the previously diagnosed obesity to class II. No endocrine causes for the patient’s weight gain were found. The iatrogenic menopause following bilateral adnexectomy was well controlled by hormone substitution therapy, therefore the weight gain was attributed to genetics, an admittedly unbalanced diet, and a sedentary lifestyle.The routine check-ups involving physical examination, lab values, tumor markers, and imaging studies showed no signs of relapse or significant changes in the patient’s health, apart from mild hepatic steatosis and significant weight gain , they are the most common non-seminomatous tumors in adolescent girls, having the lowest 5-year relative survival rate in the 10–19 age group [In pediatric patients, a quarter of all malignant GCT presents two or more histological types, thus classified as mixed germ cell tumors ,37. In fge group .As presented in our rare case, surgical intervention was fundamental in the diagnosis and management of the large tumor, and therefore it is often the first step of the treatment in the majority of large-volume tumors. A laparotomy is usually performed, given the size and solid nature of these tumors. Since germ cell tumors occur mostly in the younger population, when possible, fertility-sparing interventions are performed in which the unaffected ovary and fallopian tube are preserved ,42,43,44While in adults any immature teratoma classified higher than stage 1, based on the FIGO system, and a lower differentiation than Grade 1A, would be treated with chemotherapy without considering prior surgical management, in pediatric patients the use of chemotherapy has been challenged, with authors suggesting that complete resection of the tumor alone is sufficient, sometimes regardless of the grading, with no significant differences in overall 4–5 year survival of these patients compared to those who receive platinum-based chemotherapy ,47,48,49Although chemotherapy treatment guidelines are clear in adult patients, they become uncertain in the pediatric population when involving ovarian GCTs of mixed histology due to the very low incidence of these cases. The use of cisplatin-based chemotherapy has been a breakthrough, not only in the treatment of gynecological cancers, but also in neuroblastomas, hepatoblastomas, medulloblastomas, and other solid tumors, drastically improving survival rates, and several studies have proven its superiority over carboplatin. This observation has led to the introduction of cisplatin in most chemotherapy schemes  in combination with etoposide, ifosfamide, bleomycin, or vinblastin . The dowThe downside of using platinum-based chemotherapy is the risk of developing secondary tumors. This theory was substantiated by research that discovered a dose-dependent impact of chemotherapy on GCTs in 96 chemotherapy-treated individuals. In larger cohort studies, a dose-dependent link between platinum-based chemotherapy and GCTs eradication has not been studied . In addiEven though the underlying process is uncertain, cisplatin-based treatment might be a causative factor, although without significant proof. Cisplatin has an alkylating-like property, binding DNA and inducing apoptosis. Even if the growth of fat cells created by an antimitotic agent may seem counterintuitive, it is vital to recognize the alterations in the body caused by cisplatin. In reality, an increased body mass index and metabolic syndrome are potential side effects that may be attributed to hormonal changes brought on by chemotherapy, such as a testosterone deficit.Quite the contrary, it has been demonstrated that cisplatin inhibits the migration and differentiation potential of human adipose-derived mesenchymal stem cells, and it can be assumed that the same mechanism is applicable to peripheral blood stem cells . AdiposeOur patient showed no signs of preexisting lipomas or abnormal growth in the affected area on the MRI performed before and after surgery prior to the start of platinum-based chemotherapy. The first signs of the abnormal growth could be seen less than a month after the last platinum agent-based cycle (cisplatin), the slight increase in size (~1–2 mm) was confirmed six months later, and surgery became necessary 18 months after the last PEI cycle C,D. AlthWhile the occurrence of lipomas after non-steroid chemotherapy regiments has not been frequently reported, chemotherapy-associated fatty depositions and liver injury is a well-known phenomenon that can manifest itself in a wide range of patterns from transient transaminitis, cholestasis, and venous occlusion to acute liver failure or neoplastic transformation. Cisplatin, one of the antineoplastic agents that our patient has received, has been noted to temporarily elevate liver enzymes, but it is rarely linked to severe cases of hepatotoxicity and fatty infiltration of the liver. However, another platinum-based agent, namely oxaliplatin, has been recently associated with the development of hepatosteatosis in adults who underwent hepatic resections due to colorectal or hepatic metastases . One stuIn the case of our patient, it would be unwise to disregard the nutritional factor that might have played a role in the development of the fatty liver. In the midst of the obesity epidemic, which has unfortunately not spared children, non-alcoholic fatty liver disease (NAFLD) has become the number one cause of chronic hepatic disorders in both children and adults . MoreoveAnother factor that should be taken into account when discussing the etiology of the lipomatosis that our patient developed is the hormonal replacement therapy (HRT) administered after surgery. Although the process is not yet known, estrogen seems to promote the subcutaneous accumulation of adipose tissue by inhibiting lipolysis in the subcutaneous deposits only. That way, intra-abdominal fat deposits, which are typically seen in the male population and post-menopausal women, are shifted predominantly toward the subcutaneous regions. After menopause, either physiological or medically induced, a redistribution of fat is observed, with fat starting to accumulate in the visceral area. HRT, among other treatments, prevents both visceral fat distribution and additional weight gain ,63. GiveThe two phenomena described as “Chemotherapeutic retroconversion” and “growing teratoma syndrome” could explain the lipomatosis that appeared in our patient after chemotherapy ,65. MatuDespite the existing evidence, lack of genetic analysis of the patients and experimental methods to determine causality leave the possible association between the use of platinum-based agents and the development of lipomatosis uncertain. The underlying mechanism by which cisplatin or other platinum agents might affect adipocytes and favor the development of lipomatous tumors is yet to be understood. This case report and literature review provides further important evidence into a possible very rare side effect of cisplatin, although it requires further research and in vitro studies. Until more data is available, we suggest that our fellow clinicians consider previous treatment with platinum agents as a possible cause in cases of rapid development and growth of lipomas when nutritional, hormonal, or familial predisposition do not warrant the clinical manifestations.


Lymphoplasmacyte-rich meningioma (LPRM) is a rare form of meningioma characterized by prominent lymphoplasmacytic infiltrates into the tumor. Report of flat growth of LPRM mimicking pachymeningitis is rare in the literature.A 55-year-old female who suffered from episodes of headache and seizures has been diagnosed with pachymeningitis for 4 years because post contrast brain MRI demonstrated enhanced carpet-like dura lesion in the left frontal lobe. The lesion kept unchanged on yearly follow-ups until a recent brain MRI found the lesion grew significantly into a mass. The lesion was resected and pathology suggested LPRM.LPRM may present as carpet-like growth pattern on MRI. Long-term follow-up in patients with pachymeningitis is necessary. Occasionally such growth pattern can also be observed in LPRMs [Lymphoplasmacyte-rich meningioma (LPRM) is an extremely rare histological variant of meningioma characterized by prominent lymphoplasmacytic infiltrates into the tumor. It accounts for 0.51% of intracranial meningiomas [in LPRMs . Herein,A 55-year-old female was admitted for a 4-year history of recurrent psychiatric problems, headache, and seizures. In June 2016, the patient developed irritation, auditory hallucination and delusion. Brain CT revealed hyperdensity in the left frontal dura matter  showed normal cell count, glucose, protein and chloride levels. CSF IgG4 was undetectable. Malignant cell was not found in CSF. The patient received left frontal dural mass biopsy, and it showed clustered nests of meningothelial cells in an abundant inflammatory and collagenated background . Lumbar puncture revealed an opening pressure of 200mmHLPRM is an extremely rare histological variant of WHO grade I meningioma, characterized by infiltration of lymphocytes and plasma cells in the tumor. On MRI, most LPRMs present as dural masses, but flat growth has been occasionally described in the literature –8. SeverThe nature of LPRM has not been fully understood. Some authors regard it as a mechanism of host immune resistance to the tumor . So, LPRIn conclusion, we report a case of LPRM with carpet-like growth pattern, mimicking pachymeningitis. Neuologists should be aware that LPRM can mimic pachymengintis in the early stage. Long-term follow-up is needed for pateints with pachymengiitis and dura matter biopsy are justifiable when the diagnosis of pachymenititis became doubtful.


External root resorption is an irreversible loss of dental hard tissue as a result of odontoclastic action. Multiple external cervical root resorptions in permanent teeth are rare. The exact cause of external cervical root resorption is unclear. It is currently well established that RANK/RANKL signaling is essential for osteoclastogenesis and osteoclast-mediated bone resorption. Denosumab is an anti-RANKL antibody used for the treatment of postmenopausal osteoporosis. RANK/RANKL pathway suppression by denosumab is expected to suppress the activity of clastic cells responsible for hard tissue resorption involving both osteoclasts and odontoclasts.This case report demonstrates aggressive and generalized idiopathic external cervical root resorption that started and advanced during ongoing antiresorptive therapy with the human monoclonal RANKL-blocking antibody denosumab without discontinuation of therapy in a 74-year-old female patient treated for postmenopausal osteoporosis. The extent of resorptive defects was too large and progressively led to fractures of the teeth. The number of teeth involved and the extend of destruction excluded conservative treatment. The affected teeth had to be extracted for functional prosthetic reconstruction.This finding suggests that treatment with denosumab may be associated with severe and aggressive odontoclastic resorption of multiple dental roots despite an adequate inhibitory effect on osteoclasts in the treatment of osteoporosis. The RANKL-independent pathways of clastic cell formation are likely to be involved in this pathological process. Receptor activator of nuclear factor kappaB (RANK) is a receptor for RANK ligand (RANKL) and part of the RANK/RANKL/osteoprotegrin (OPG) signaling pathway that regulates osteoclast differentiation and activation. RANK/RANKL/OPG plays an essential role in osteoclastogenesis and osteoclastic bone resorption [External cervical root resorption (ECR) is an irreversible loss of hard dental structures that may necessitate dental treatment or even an extraction of affected teeth. Multiple external resorptions in permanent dentition are uncommonly reported phenomena. The etiology and pathogenesis of ECR is not exactly understood, but it is believed to involve the action of osteoclast-like cells (odontoclasts) originating from hematopoietic stem cells in the bone marrow . In addisorption . Histolosorption –9. Denossorption . Denosumsorption . The lonsorption . Antiressorption , 14. Thesorption . EvidencIn the present article, we demonstrate the coincidence of long-term and continuous antiresorptive therapy with denosumab and aggressive multiple external cervical root resorptions in patient with postmenopausal osteoporosis.A 74-year-old female patient presented in July 2020 with a chief complaint of occasional pain, thermal sensitivity and slightly increased mobility of her left mandibular second premolar (tooth 35). Her symptoms started two months ago. She also reported unexpected fractures of otherwise asymptomatic teeth in the previous weeks. Four weeks prior to the first examination, a crown of the first left mandibular incisor tooth 31) suddenly broke. Two weeks later, in a similar manner, she lost a crown of the second maxillary right incisor (tooth 12) because of the fracture in the cervical area of the tooth caused by chewing. She was referred by her dentist to the Department of Stomatology and Maxillofacial Surgery at University Hospital Martin with numerous sites of external cervical root resorption found on her recent panoramic radiograph  via subcutaneous injections every six months without any discontinuation until the first presentation to our department. She received the last injection of denosumab in March 2020. At the time of her presentation to our clinic, she was medicated with the following medications: denosumab, calcium, vitamin D, nitredipine, perindopril, bisoprolol, latanoprost, and timolol.No detectable abnormalities were identified on extraoral and intraoral examination. Intraoral examination revealed good oral hygiene, dentition with multiple restorations and no active carious lesions. Crowns of previously broken teeth 31 and 12 were missing, and the roots were covered with gingiva  in 2010 to 0.697 g/cm2 (T-score − 2.0) in 2020.After the examination in our clinic with the diagnosis of multiple idiopathic ECR and Generalized Stage 3 Periodontitis, the patient consulted her endocrinologist, who ordered routine laboratory tests. These test results were  within the normal range with the exception of an elevated cholesterol level of 5.89 mmol/l. In July 2020, circulating intact parathyroid hormone and serum vitamin D, calcium, magnesium and phosphorus levels were normal. Her osteoporosis treatment with denosumab, calcium and vitamin D was assessed as efficient with decreased bone turnover markers: osteocalcin level was 5.06 ng/ml , procollagen type 1 N-terminal propeptide was 9.27 ng/ml , and collagen type 1 C-terminal telopeptide was 0.051 ng/ml . Her densitometry in 2010 showed osteoporosis. During a decade of treatment, her bone mineral density increased from 0.528 g/cmBecause of the pain and mobility of tooth 35, in August 2020, the tooth was extracted under local anesthesia with subsequent suturing of the mucoperiostal flap to avoid medication-related osteonecrosis of the jaw (MRONJ). The extracted root with cervical resorptive lesion was sent for histological examination Fig. . The heahttps://www.ehealthme.com/ds/prolia/tooth-resorption/). Some reports in the literature suggest that denosumab or bisphosphonates may act protectively against the aggressive progression of ECR during treatment [External cervical root resorption is an aggressive pathologic process that may lead to loss of teeth. Multiple ECR of permanent teeth is rare condition. The first case of idiopathic external cervical root resorption was published in 1930 by Mueller and Rony; since then, several cases have been reported in the literature –21. The reatment , 21, 25.reatment . Denosumreatment . Discontreatment . The rebreatment . Possiblreatment . Becausereatment . Yet thereatment .Histological examinations of ECR lesions typically reveal the presence of multinucleated osteoclasts or resorptive (clastic) cells located within resorptive lacunas , 31. In Interesting in this case report is the coincidence of ongoing RANKL-blocking antiresorptive treatment and massive resorption of all tooth roots. Although RANKL-induced osteoclast formation is considered as major pathway, reports in the literature suggest that osteoclasts can also differentiate independently of RANKL , 36–42. This case report demonstrates aggressive and multiple idiopathic external cervical root resorption that started and advanced during ongoing antiresorptive therapy with the human monoclonal RANKL-blocking antibody denosumab without discontinuation of therapy. This finding suggests that treatment with denosumab may be associated with severe and aggressive odontoclastic resorption of multiple dental roots despite an adequate inhibitory effect on osteoclasts in the treatment of osteoporosis. The RANKL-independent pathways of clastic cell formation are likely to be involved in this pathological process.


Neuromuscular choristoma (NMC) is a rare peripheral nerve lesion that is composed of ectopic mature muscle fibers and nerve fascicles, typically involving major nerve roots or trunks, such as the cranial nerves, brachial plexus, and sciatic nerves. The onset of NMC frequently occurs in the first decade of life. Here, we present the first documented case of a case of esophageal NMC in an adult patient.A 46-year-old male patient presented in 2018 with a submucosal tumor of the esophagus. Upon presentation, the tumor was approximately 10 mm in diameter, covered by normal mucosa, and located in the left posterior wall of the esophagus in a position that was 30 cm from the incisor. The tumor was discovered incidentally during gastroscopic examination. In March 2021, endoscopic re-examination revealed no significant changes in the tumor. Endoscopic ultrasound revealed an oval hypoechoic mass with a homogeneous internal echo that originated from the muscularis propria with a maximum cross section of 13 mm × 6 mm. Resection was performed under gastroscopy. The resection specimen was 12 mm × 5 mm in size and was a well-demarcated, elastic, hard, and tough with a gray section. Histologically, the specimen consisted of an abundance of smooth muscle fiber bundles intercalated among nerve fibers, but without malignancy. Immunohistochemical examinations revealed positivity for S-100 protein, caldesmon, NSE and desmin, but negativity for CD117, DOG-1, HMB45, and Melan A. There was also aberrant nuclear localization of beta-catenin. Collectively, these findings led to a diagnosis of esophageal NMC.NMC is extremely rare, especially esophageal NMC, and is very challenging to accurately diagnose prior to resection. It is important that we can differentiate NMC from other types of tumors. Neuromuscular choristoma (NMC), referred to as a ‘benign triton tumor’, is a rare benign nerve lesion that is characterized by differentiated muscles admixed with nerve fascicles –3. The oFollow-up results relating to cases of NMC are limited and the exact characteristics and underlying mechanisms have yet to be clarified. It has previously been reported that NMC is frequently associated with desmoid-type fibromatosis (DTF) . The etiA 46-year-old male presented with a submucosal tumor of the esophagus. In 2018, the tumor was approximately 10 mm in diameter, covered by normal mucosa, and located on the left posterior wall of the esophagus 30 cm from the incisor. The tumor was discovered by accident during gastroscopic examination , Neurofilament(NF) and desmin. In addition, the lesion showed aberrant nuclear localization of beta-catenin and negativity for CD117, DOG-1, HMB45, and Melan A. Collectively, these data supported a diagnosis of esophageal neuromuscular choristoma (Fig. It has been 11 months since the resection of this esophageal submucosal tumor; the patient has no obvious clinical symptoms but has yet to be reviewed by gastroscopy.NMC is a rare peripheral nerve lesion that is composed of ectopic mature muscle fibers and nerve fascicles and typically involving major nerve roots or trunks , 13. SinPatients with intracranial neuromuscular choristoma or neuromuscular choristoma of the sciatic nerve generally undergo imaging diagnostics , 18. MagCTNNB1 gene may be a common molecular genetic anomaly in NMC and NMC associated DTF. CTNNB1 exon 3 mutations, which are identical to those observed in sporadic DTF, had been detected in 4 out of 5 NMC cases [NMC has been considered as a hamartoma that featured the aberrant proliferation of peripheral nerve with a favorable outcome . NMC assMC cases . Based oNot all esophageal submucosal lesions require endoscopic removal/resection. In our current case, the esophageal submucosal tumor was discovered by accident at the age of 43; it is not known whether the lesion originated in infancy although the size of the tumor did not change significantly over the 3-year period, thus indicating an indolent characteristic. At initial diagnosis, a doctor told the patient that he could not have the lesion removed at that time, although regular endoscopic follow-up was required. Since there have been no previous reports of esophageal NMC, the natural course of this disease still needs to be investigated in a larger number of cases.Here, we report the first case of NMC occurring in the esophagus of an adult. Although NMC is widely perceived as a benign disease, it should be included in the differential diagnosis of neoplastic lesions not only in the cranial, brachial plexus, or sciatic nerves, but also in the esophagus. Patients with esophageal NMC also need to be followed-up closely.


Eosinophilic angiocentric fibrosis (EAF) is a rare inflammatory lesion, especially in orbit. EAF is believed to be related to IgG4-related disease (IgG4-RD), but the clinical manifestations of systemic involvement are relatively rare and easy to be confused with tumors or other inflammatory diseases. Histopathological examination is the most important way of its diagnosis and differentiation.We presented a 55-year-old female patient presented with recurrent swelling of the right lower eyelid for more than 2 months. The pathological diagnosis was EAF. Positive immunostaining for IgG, CD34, κ, and λ, while negative immunostaining for IgG4.Complete surgical resection is the preferred treatment, histopathological examination is the main diagnostic standard. Eosinophilic angiocentric fibrosis (EAF) is a rare and slow progressive inflammatory disease. The main sites of involvement are the nasal cavity and sinuses, although rare cases can also occur in the orbit, upper respiratory tract, gingiva, and brain –4. The eA 55-year-old female patient presented with recurrent swelling of the right lower eyelid for more than 2 months. She had a history of hypertension and hysterectomy. Ophthalmic examination showed binocular visual acuity of 1.0, normal intraocular pressure, and normal anterior ganglium and fundus. The tumor, which was tough, painless, and movable, was palpable near the zygomatic arch of the lower right eyelid. Laboratory examination showed the patient to be negative for syphilis antibody. Orbital magnetic resonance imaging (MRI) showed oval-like T1 and slightly shorter T2 signal shadows in the lower quadrant of the right outer orbit, with clear boundaries and significantly enhanced annular edges  . Chew etHistopathological examination is the main standard for diagnosing EAF. The typical pathological manifestations are massive infiltration of eosinophils, plasma cells, lymphocytes, and fibroblasts around small blood vessels; thickening of matrix; and characteristic rotation of onionskin-like collagen fibers and mesh proteins around occlusive blood vessels, accompanied by positive expression of IgG4 and IgG , 2. EAF Complete surgical resection is the most important treatment method for this disease. Surgery combined with glucocorticoid therapy has a certain but not strong effect . RituximIn conclusion, in this paper we report a case of localized EAF and summarize the current literature on this disease. In clinical diagnosis, EAF should be considered when a patient presents with an orbital mass. Complete surgical resection is the preferred treatment, histopathological examination is the main diagnostic standard, and close follow-up is very necessary. The relationship between EAF and IgG4-RD should be elucidated through additional case studies.


Splenic artery pseudoaneurysm is the most common of all the visceral artery pseudoaneurysms. Presentation is often variable and the condition demands immediate diagnosis and management because pseudoaneurysm rupture increases morbidity and mortality. It is associated with pancreatitis and other conditions like abdominal trauma, chronic pancreatitis, pseudocyst of the pancreas, liver transplantation, and, rarely, peptic ulcer disease. We present a case of a giant splenic artery pseudoaneurysm measuring 14x8 cm. Proximal and distal control of the vessels could not be achieved during the procedure because of local adhesions and inflammation and it was necessary to cross clamp the supraceliac aorta to control bleeding. On clinical examination, he had anemia and his blood pressure was 90/60 mmHg. After resuscitation, he underwent upper gastrointestinal endoscopy which was normal. He also had a history of hematemesis for which he was evaluated with triphasic abdominal contrast-enhanced computed tomography (CECT) . His abdOn exploration, the patient’s abdomen was filled with blood and clots, which were suctioned out. Opening the gastrocolic omentum revealed a thrombosed sac with diffuse bleeding visible from the outer wall of the sac, but no arterial spurt was identified from the sac. After opening the lesser sac, control of the supraceliac aorta was taken, but we were unable to reach the celiac axis and splenic artery proximally or distally because of dense adhesions. After obtaining supraceliac aorta control, we directly opened the sac and an arterial spurt was identified and ligated . Since tThis manuscript is in accordance with the Helsinki declaration and with local ethical guidelines. Informed consent was obtained from the patient. Written informed consent was obtained from the patient’s husband for publication of this case report and any accompanying images.Giant pseudoaneurysm of the splenic artery is a rare condition.In the index case, the patient came to an outpatient department with a history of prior pancreatitis, presenting with upper abdominal pain and two episodes of melena and gradual epigastric fullness. He was hemodynamically unstable at presentation. Up to 50-58% of patients with SAP can present in a hemodynamically unstable condition. Bleeding can occur in the lesser sac, stomach, pancreatic duct, and retroperitoneum. Ruptured SAP are associated with high rates of mortality and morbidity.,Encouraging results of endovascular treatment and minimal complications indicate endovascular procedures as first-line treatment. However, this method may not suffice in cases with giant pseudoaneurysms.10In the index case, the patient was managed with an open surgical procedure. Ideally, one must take control of the splenic artery proximally and distal to the aneurysm. However, in cases with pancreatitis, this may be difficult to achieve because of dense adhesions and sometimes the lesser sac may be frozen due to dense adhesions. In the index case, proximal and distal control of the splenic artery and celiac axis could not be achieved, so we took control of the supraceliac aorta and opened the SAP sac. The main limitation of this case study was non-availability of follow-up images.Giant pseudoaneurysm of the splenic artery is a rare condition that can occur secondary to pancreatitis. In hemodynamically stable patients, endovascular treatment modalities like coil embolization, stenting, and glue embolization can be tried depending upon the size of the pseudoaneurysm and patient condition, but when an endovascular procedure fails, exploratory laparotomy is the only option to treat the condition. However, in patients with pancreatitis, proximal and distal control may not be possible and in such cases control of the celiac axis or aorta might be helpful.


Ganglioneuromas (GNs) arise from the Schwann cells, ganglion cells, and neuronal tissues, and are extremely rare, slow-growing, benign tumors. GN has usually grown very large when it is diagnosed since no specific clinical symptoms or laboratory findings indicating GN are available, especially when it occurs in the retroperitoneal space. Total resection of the tumor is the recommended treatment.We present the imaging and pathological findings of a giant adrenal GN in a child. A 7-year-old boy suffered from nausea and postprandial vomiting for 1 week with no precipitating factors. There was no family history of any disease, and the boy did not suffer from any disease in the past. Biochemical examination showed normal results. Physical examination showed an immobilized palpable mass in the left abdominal area. Abdominal computed tomography revealed a 13 cm × 10 cm solid mass in the retroperitoneal space. The mass showed slight and heterogeneous enhancement after injection of a contrasting agent. The mass was surgically resected locally to address the embedded abdominal vessels, and the histopathological and immunohistochemical diagnosis of the mass was GN. After the surgery, the symptoms of nausea and vomiting were relieved, and no complications occurred.GN should be considered when a child presents with a giant retroperitoneal hypodense mass and the mass presents uneven and delayed enhancement. Histopathology is the golden standard for the diagnosis of GN. Currently, surgical excision is the optimal treatment. Ganglioneuroma (GN) is a rare, benign tumor. It usually occurs in the posterior mediastinum and retroperitoneum. GNs originating from the adrenal glands, neck, and paravertebral sympathetic plexus region are relatively rare. Adrenal ganglioneuroma (AGN) is a slow-growing neoplasm located in the adrenal glands and accounts for 20–30% of all GN cases . A giantA 7-year-old boy with nausea and vomiting for 1 week was presented to the gastroenterology department of our hospital. The boy was healthy and there was no past medical history or family history of any disease. During the physical examination, a left immobilized palpable solid abdominal mass of 13 cm × 10 cm was detected. Since the mass possibly originated from the left adrenal gland, adrenal endocrinological examinations were conducted. Biochemical tests revealed that the levels of cortisol were 1.36 mg/dl, 17.68 mg/dl, and 5.82 mg/dl at 0 h, 8 h, and 16 h, respectively , and that of fibrinogen was 1.9 g/l . The level of aldosterone (45.8 pg/ml) was in the normal range . Similarly, the levels of ACTH were in the normal range .Abdominal computed tomography (CT) showed a large solid mass on the left retroperitoneum measuring approximately 10.1 cm × 9.3 cm × 13.4 cm in size  Ganglioneuroblastoma: It is categorized as nodular and mixed, both of which contain neuroblastic components. The nodular type contains neuroblastic components and ganglion cells, and there are clear boundaries between the neuroma components. In the mixed type, the neuroblastic components are irregularly distributed in the Schwann cell interstitium in the form of focal or small nests. The CT scan usually lacks specific features and the diagnosis depends on the pathological examination  Ganglion. A carefBiologically, GNs are benign tumors that rarely metastasize or recur. However, in a study of 49 cases, two cases showed metastasis to local lymph nodes and one case showed metastasis to soft tissue . OccasioThe surgical method can either be open or laparoscopic tumor resection. Laparoscopic surgery is an ideal surgical method for GNs because it is associated with lesser trauma and faster recovery , 9. In oOur case findings provide a new reference for the management of giant AGNs in children and recommend laparoscopic resection in patients with giant retroperitoneal tumors.Although AGN in children is a rare and benign disease, it should be considered when CT shows a solid mass in the retroperitoneal space with delayed enhancement. The ultimate diagnosis of GN depends on histopathological examination. The optimal treatment for GN is surgical excision.


Congenital left atrial appendage aneurysm (LAAA) is a rare cardiac anomaly with a variety of presentations, from being asymptomatic to potentially serious complications such as systemic thromboembolism and atrial tachyarrhythmia.We report a case of congenital giant LAAA in a 35-year-old man presenting with acute massive cerebral infarction and atrial fibrillation (AF) with rapid ventricular rate. The AF was refractory to conventional antiarrhythmic agents, such as amiodarone and electrical cardioversion, but restored and maintained sinus rhythm after surgical resection of LAAA. The patient remained free of events and was in sinus rhythm during half-year follow-up.Giant LAAA has the potential causing serious complications and should be managed surgically in most cases. Left atrial appendage aneurysm (LAAA) is a rare entity that can be congenital or acquired in etiology , 2. CongA 35-year-old man who had no previous medical history and cardiac history suddenly lost consciousness and was referred to our hospital 10 h after the onset of the stroke. At the time of admission, his coma was rated 7 on the Glasgow Coma Scale (GCS), and he displayed left-sided hemiplegia. He had sinus rhythm with paroxysmal AF  and normvia a broad neck  origin from an otherwise normal atrial chamber; (2) well-defined communication with the atrial cavity; (3) location within the pericardium; and (4) distortion of the LV by an aneurysm , 27. TheAccording to the above criteria, imaging exams are essential tools for diagnosis and precise evaluation of LAAA, which are important for the subsequent treatment . ImagingThe early surgical intervention seems to be the standard of treatment once the diagnosis is established in the current literature, even in asymptomatic cases, aimed to prevent the occurrence of serious complications , 31. HowThe recommendation of surgical occlusion in symptomatic patients is based on non-randomized or observational cohort studies  as it cavia median sternotomy and cardiopulmonary bypass was used. A full maze procedure was not adopted in the patient, but sinus rhythm was restored right after the operation and maintained as of the time of writing.Various successful approaches to aneurysmectomy with or without cardiopulmonary bypass have been described, such as median sternotomy, left thoracotomy, mini-thoracotomy, and endoscopy , 38. RecIn our opinion, surgical intervention should be taken in symptomatic LAAA or in secondary prevention of thromboembolic events. In asymptomatic cases or in primary prevention, risk stratification for thromboembolic events is the key for the choice of management strategies. The early surgical intervention should be taken in cases with a high risk of thromboembolic events. However, in addition to spontaneous echo contrast and thrombi in the aneurysm, no other reliable indicators can predict embolic complications . A studyThis is only a case report with a literature review, and such a study has inherent limitations and may not be able to provide the true perspective of the disease. Again, the LAAA is a very rare disease and a case series study is hard to perform in the real world. Also, this study does not allow us to establish a cause–effect relationship between LAAA and massive cerebral infarction.We described here a unique case with a giant LAAA that presented with acute massive cerebral infarction and refractory AF with very rapid heart rate, which has never been described before in the literature. LAAA was detected initially by TTE and thereafter confirmed by contrast-enhanced echocardiography and TEE. Surgical resection was performed promptly after frequently occurring AF with rapid heart rate, and the patient had favorable outcome. Giant LAAA has a high risk of causing significant morbidity as in our case and should be managed by surgical intervention in most cases.The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author.Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.RL wrote the report, performed the literature research, and took the pictures. RL and FM performed echocardiography, including TEE and contrast-enhanced echocardiography. LL performed surgery. HG and YP provided CT images. HW revised the report and performed the literature research. DW reviewed the manuscript. All authors read and approved the final manuscript.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Intravestibular schwannomas are rare tumors within the intralabyrinthine region and involve different management considerations compared to more common vestibular schwannomas. In this report, the authors review a case of a 52-year-old woman who presented with hearing loss and vestibular symptoms and was found to have a left intravestibular schwannoma. Given her debilitating vestibular symptoms, she underwent microsurgical resection. In this video, the authors review the relevant anatomy, surgical technique, and management considerations in these patients.https://stream.cadmore.media/r10.3171/2021.7.FOCVID2187The video can be found here:  In this surgical video, we describe the management of a patient found to have an intravestibular schwannoma and review surgical technique as well as relevant anatomy.The patient is a 52-year-old female who presented with profound left-sided hearing loss, tinnitus, ear fullness, and imbalance leading to falls. Other past medical history included a distant diagnosis of colon cancer which had been resected and hyperthyroidism.Her physical examination revealed significant left-sided hearing loss, but she was otherwise neurologically intact. Otomicroscopy performed bilaterally was unrevealing.Audiogram results demonstrated sensorineural hearing loss on the left side with no response at 90 dB and 0% word recognition. Right-sided hearing was preserved.An MRI was obtained demonstrating a small mass within the left vestibule projecting into the horizontal and superior semicircular canals concerning for an intravestibular schwannoma, as demonstrated on these T1 postcontrast and FIESTA sequences. The mass did not extend into the internal auditory canal or cochlea.Management options for intravestibular schwannomas can include microsurgical resection, radiosurgery, and conservative management with serial imaging. Given the patient’s hearing loss and ongoing debilitating vestibular symptoms, microsurgical resection of the mass was recommended via a translabyrinthine approach. Recent meta-analyses in the literature suggest that the majority of patients treated with microsurgery will experience improvement in their vestibular symptoms on follow-up. If surgery is pursued, cochlear implant placement can be performed at the time of resection. As there is a risk of cochlear ossification on follow-up which can prevent cochlear implant placement, our group typically proceeds with placement at the time of tumor removal or within 3–6 months prior to ossification. In this case, consideration was given to placing a cochlear implant at the time of surgery, but the patient elected for tumor resection only.She subsequently was taken to the OR for tumor resection. The patient was placed in the supine position with the head turned to the right. Facial nerve monitoring was utilized for intraoperative localization and preservation.2:41 A semicircular postauricular incision was made down to the temporalis fascia. The mastoid periosteum was incised and elevated. Next, a mastoidectomy was performed. The anterior, posterior, and inferior borders of this presigmoid approach correspond to the external ear canal, the sigmoid sinus, and the mastoid tip, respectively.3:02 The microscope was then brought into the operating field. Using a cutting burr, a mastoidectomy was performed. Drilling proceeded through the air cells of the mastoid bone until the antrum was visualized. During this approach, the bone overlying the tegmen, sigmoid sinus, and ear canal was kept intact.3:21 A diamond burr was then used to define the horizontal canal and open the antrum wider to define the incus, which can be seen partially exposed within the antrum in this operative video.3:35 Using the diamond burr, air cells were further removed until the dense bone of the horizontal canal was visualized and blue-lined.3:46 At this portion of the case, the horizontal canal can be seen blue-lined. Drilling proceeds along the center of this line or superior to it to avoid injury to the facial nerve which is located inferiorly.4:02 Once the facial recess had been defined, the posterior semicircular canal was drilled using a diamond burr. Here, the posterior canal has been blue-lined.4:14 The superior semicircular canal was also further defined at this point in the case. The horizontal semicircular canal was then entered, making sure to avoid injury to the facial nerve located inferior and slightly lateral.4:30 The tumor was noted along the anterior limb of the horizontal canal. This was followed into the vestibule.4:48 Once the tumor was exposed, microinstruments were used to dissect the tumor out of the vestibule.5:11 Here, the component of the tumor extending into the superior semicircular canal was dissected and pulled down toward the main component within the vestibule.5:30 During this time, the facial nerve with overlying bone stimulated at 0.5 mA.5:43 A gross-total removal of the mass was performed without changes in facial nerve stimulation threshold.6:01 For reconstruction, a bone pate was placed over the vestibule and exposed semicircular canals. A fat graft was not used to simplify approaches to this region if cochlear implant placement was considered in the future. There was no CSF leak during the surgery. The rest of the closure was performed in layers in a typical fashion. There were no complications.Pathology demonstrated a WHO grade I schwannoma. Postoperatively, the patient had no facial weakness. Her vestibular symptoms improved shortly after the surgery, and the patient is currently undergoing planning for a left cochlear implant.6:40 Intralabyrinthine schwannomas are rare tumors of the vestibular or cochlear nerves that can result in hearing loss, vertigo, and tinnitus, often presenting with Ménière’s-like symptomatology. Typically, high-resolution images of the affected temporal bone can be obtained to help with diagnosis and tumor localization. Prior studies have categorized these tumors based on specific location and involvement of either the cochlea or vestibule and based on presence of extension into the internal auditory canal.7:10 Here are tumor locations within the labyrinth that have been previously defined. Intravestibular lesions may or may not involve extension into the semicircular canals.Hearing preservation is not possible with surgical approaches to these tumors, and thus intervention is often reserved for patients with nonserviceable hearing and growth on imaging. Additional surgical indications include intractable vestibular symptoms, which often respond well to intervention. Radiosurgery may be another treatment option but may exacerbate hearing loss. Furthermore, it is unclear of the impact of this treatment modality on long-term vestibular outcomes. Overall, evidence from the literature suggests that symptoms such as vertigo improve in the vast majority of patients after microsurgical resection and remain stable for patients treated with SRS.Dr. Limb reports being a consultant for and receiving research support from Advanced Bionics Corporation, Med-El Corporation, and Oticon Medical; and being the chief medical officer of and a stock shareholder in Spiral Therapeutics.Primary surgeon: Theodosopoulos, Limb. Assistant surgeon: Xu. Editing and drafting the video and abstract: Theodosopoulos, Morshed, Haddad, Raygor. Critically revising the work: Theodosopoulos, Morshed, Haddad, Raygor, Limb. Reviewed submitted version of the work: all authors. Approved the final version of the work on behalf of all authors: Theodosopoulos. Supervision: Morshed.


We report the challenging case of a 6-year-old boy with precocious puberty related to histologically proven Leydig cell tumor.−3 mm2/s and a lower ADC value of the non-tumor parenchyma compared to the contralateral testis (ADC = 800 × 10−3 mm2/s vs 1100 × 10−3 mm2/s), attributed to the spermatogenesis induced by hormonal impregnation.Multiparametric ultrasound and magnetic resonance imaging (MRI) was performed. Interesting findings were scarcely or never reported in children and differed from adults Leydig cell tumors s such as the hyperechogenic halo surrounding the lesion and the dominant central vascularization using ultrasensitive Doppler. MRI revealed an enlarged testicle with strong enhancement of a tumor, a tumor apparent diffusion coefficient (ADC) of 600 × 10We illustrate multiparametric US and MRI findings of a pediatric Leydig cell tumor, including the imaging changes attributed to local hormone secretion, which may be helpful in similar cases. Precocious puberty (PP) has a major impact on the psychological and physical development and well-being of children, with an annual incidence over 0.24 per 10,000 boys , and areN < 35 pmol/mL). US examination  showed an enlarged left testicle (estimated volume: 1.5 mL vs 0.6 mL on the right side), with a nodular ovoid-shaped hypoechoic lesion at its upper pole. The tumor exhibited a peripheral hyperechoic ring, with smooth margins and a shaped demarcation with adjacent pulp, the whole measuring 7.4 × 6 mm. color Doppler showed a hypervascular tumor compared to the surrounding tissue, with predominant central vascularization at ultrasensitive Doppler. Shear-wave elastography of the tumor, surrounding parenchyma, and contralateral testis indicated a mean stiffness of 11.7 kPa, 6.1 kPa, and 5.4 kPa, respectively , and estradiol level was slightly increased  and hypo-intensity on apparent diffusion coefficient (ADC) maps (ADC = 600 × 10−3 mm2/s) . No intraoperative frozen section was performed as lesion size was inferior to 7 mm after surgical removal. [Scrotal MRI using a 1.5-T scanner and a surface coil indicated that the tumor had a low-intensity signal on a T2-weighted image  and an iso-intensity signal on a T1-weighted image. Fast, strong and homogeneous enhancement was observed after dynamic gadolinium injection with a type 2 time–signal intensity curve without any significant wash-out, compared to the normal contralateral testis. MRI also showed slightly restricted diffusion on high removal. . No signLCTs are rare. Typical US findings in adults have been described as an isolated hypoechoic well-delimited mass with predominant peripheral hypervascularization . In adulTesticular adrenal rests tumors (TART) described in congenital adrenal hyperplasia is the main differential diagnosis in case of PP associated with testicular mass. TARTs may be hypoechoic or hyperechoic at US, mainly located near the mediastinum and bilateral, and showing a multilocular pattern, and those signs helped to differentiate them from LCTs . Their vTo conclude, the predominant central vascularization on ultrasensitive-Doppler and the hyperechoic rim were the two major features to discriminate an LCT from a unilateral TART on US scan. On MRI, both the enhancement kinetic curve and the low ADC of the lesion as well as the testicular parenchyma helped to cement our preoperative hypothesis.Diagnostic management of PP related to testicular mass may be challenging. This case illustrates the contributions brought by multiparametric US and MRI findings, including imaging changes caused by local hormone secretion, which may be helpful to distinguish an LCT from a malignant tumor or a TART, the two main differential diagnoses in PP.


Peyronie's disease (PD) usually affects middle-aged men. This condition is characterized by fibrosis and plaque in the tunica albuginea that results in deformity of the penis and makes sexual intercourse difficult. We report a case of a 52-year-old male who presented with complaints of curvature of the erect penis and erectile dysfunction. Based on our imaging findings, the patient was diagnosed with PD. ultrasonography (USG), elastography, and CT findings are described. Peyronie's disease (PD) is characterized by penile deformity caused by fibrous tissue plaques within the tunica albuginea of the corpora cavernosa of the penis . The conA clearly palpable tunica albuginea plaque that is easily distinguishable from the induration of the cavernosal tissue is seen in the majority of patients with PD. However, imaging is frequently needed to support the clinical diagnosis, assess the severity of the condition, and choose the best course of action for the patient. When evaluating patients with painful penile induration, ultrasonography is frequently the first imaging technique employed in conventional clinical practice . ManagemPatient information:A 42-year-old male presented to the outpatient department with chief complaints of curvature of the erect penis and erectile dysfunction for one year. There was no history of any associated trauma or urethral instrumentation.Clinical featuresOn clinical examination, a curve in the distal end of the penis was noted Figure .A surgical opinion was obtained and the patient was provisionally diagnosed with PD. The patient was advised penile ultrasound.Diagnostic assessmentOn B-mode ultrasound examination, a few linear calcific plaques having posterior acoustic shadowing were noted along the dorsal aspect of the shaft of the penis and involving the anterior aspect of the corpora cavernosa muscle Figure . Along tAdditionally, on ultrasound strain elastography, a strain ratio of 3.8 was noted, which indicated dark blue color suggestive of increased stiffness Figure .Further evaluation using CT was done to evaluate the exact location of the plaques and for other deeper plaques that could be missed on ultrasound. CT revealed calcified plaques along the distal end of the shaft of the penis, which further directed us closer to our diagnosis Figure .Based upon the above imaging features, a diagnosis of Peyronie’s disease was made.Therapeutic interventionsThe patient was started on intralesional interferon alfa-2b injection and the patient responded well. Antibiotic coverage  was given to prevent any secondary infection.Andreas Vesalius and Gabriele Fallopio, who were famous anatomists initially reported PD, also known as "Induratio penis plastica," but François de la Peyronie clinically classified it as a disorder causing anejaculation in 1743 [Peyronie's disease (PD) leads to the inability to perform penetrative intercourse and related emotional distress . It affeUSG, MRI, CT scans, radiography, and penile color Doppler and elastography are imaging techniques that aid in the diagnosis and treatment of PD .Due to its accessibility, low risk, and capacity to scan and quantify both the calcified and soft tissue components of PD, US is the preferred imaging technique. If necessary, the vascular state can also be evaluated. Additionally, it identifies tiny lesions that are non-palpable and can also assess the degree of fibrosis . When peSono-elastography is another modality that helps evaluate non-palpable lesions that cannot be seen on ultrasonography. Real-time strain elastography helps evaluate the elasticity of tissue by applying compression to the tissue and measuring the differences in pre and post-compression vibration. Shear-wave elastography identifies the place of maximal curvature and a region of enhanced tissue stiffness that can be utilized to target intralesional injection treatment .CT is useful, and it is possible to clearly evaluate the calcified plaques; however, CT is not useful in clinical practice and should only be used for research owing to the cost of the examination and the radiation exposure .MRI is useful in determining the location, size, and involvement of other tissues like the corpora and septum. It can also be used to make a diagnosis and choose a course of therapy. The low sensitivity for calcified visualization and the high cost, time, and resource requirements of the MRI are its limitations .Overall disappointing results of medications used in the treatment of Peyronie's disease have been noted. Anti-inflammatory drugs are used in conservative management; they help relieve the pain but do not treat the deformity. Intralesional collagenase injection corrects the deformity, though it is more costly. Significant bending or shortening of the penis with sexual difficulty or partner discomfort are indications for surgical correction of penile curvature .Numerous imaging modalities are used in evaluating PD but ultrasonography is the modality of choice since it is non-invasive and determines the severity of erectile dysfunction. Additionally, it identifies the location, thickness, and size of the plaque and can also detect calcification within the plaque. Ultrasonography along with clinical history and examination in the form of penis palpation directs toward the diagnosis of PD. This information is sufficient to guide the surgeon in the management of the disease.


Primary duodenal cancer (PDC) is rare, especially signet-ring cell carcinoma (SRCC) of the duodenal bulb, and it is commonly misdiagnosed as an ulceration. Here, we report a rare case of SRCC of the duodenal bulb presenting with gastrointestinal hemorrhage in an 82-year-old man.An 82-year-old man was admitted for gastrointestinal hemorrhage. Physical examination revealed upper abdominal tenderness and pale appearance, but was otherwise unrevealing. Laboratory workup was significant for anemia. Imaging showed no abnormalities. Two endoscopic evaluations along with interventional embolization were attempted and, unfortunately, adequate hemostasis was not achieved, resulting in distal subtotal gastrectomy, including the duodenal bulb. SRCC of the duodenal bulb was diagnosed based on pathology after surgery. Post-operatively, the patient experienced persistent gastrointestinal bleeding. Family declined further intervention and the patient eventually died one month post-resection.SRCC in the duodenal bulb is difficult to diagnose. For those with high-risk factors, endoscopic examination and biopsy are recommended. For patients who can receive radical tumor resection, pancreaticoduodenectomy (PD) is considered a first-line option. Early diagnosis and resection have been shown to improve prognosis. Primary duodenal cancer (PDC) is rare and accounts for just 0.3–0.5% of gastrointestinal cancers . Most PDAn 82-year-old male was admitted to the Department of Gastroenterology in November 2020 with intermittent melena for a duration of 2 months and hematemesis over the past 10 h. He had a history of hypertension, coronary artery disease after stenting, gout and diabetes mellitus, and he had been treated with clopidogrel hydrogen sulphate tablets since stenting. The patient was treated with analgesics that he could not describe before admission because of epigastric discomfort. His brother died of lung cancer. In 2014, he received endoscopic treatment due to a gastric ulcer complicated with hemorrhage in our hospital, and pathology revealed chronic active gastritis of the gastric mucosa. Two weeks before admission, the patient had a large amount of melena, and endoscopy was performed, which revealed multiple lesions of ulceration in the descending part of the duodenum. Gastroenterography showed the image of barium protruding out of the lumen in the descending part of the duodenum, which was considered a diverticulum. No biopsy was performed.The patient was tachycardic, but vital signs were otherwise normal. Physical examination revealed tenderness in the epigastric region and a pale appearance, but was otherwise unrevealing. Laboratory values indicated anemia (hemoglobin of 94 g/L) and renal dysfunction (blood urea of 25.4 mmol/L and creatinine of 352 μmol/L). Tumor makers, such as carbohydrate antigen 19–9 (CA19-9), alpha-fetoprotein (AFP), carcinoembryonic antigen (CEA) and carbohydrate antigen 12–5 (CA12-5) were normal. Computed tomography (CT) and abdominal ultrasound showed no abnormalities. During hospitalization, the patient received two endoscopic hemostasis procedures and one interventional embolization. The first endoscopy revealed a 1.0 × 1.0 cm lesion with ulceration and active bleeding in the duodenal bulb. No active bleeding was observed after multipoint injection with a solution consisting of lidocaine, hypertonic saline and epinephrine (L-HS-E)  originating in the gastrointestinal tract was considered. Antibiotic treatment and other symptomatic therapies were given, but the symptoms did not improve. As time went by, the patient also suffered from an inflammatory process and gradually had a fever. The situation worsened, and a jejunal nutrient tube was placed instead of enteral nutrition. The patient died one month postoperatively.PDCs are rare tumors and are mainly located in the third and fourth portions of the duodenum 45%), followed by the second part of the duodenum (40%) and the duodenal bulb (15%) . Abdomin%, followThe development of advanced endoscopic and imaging methods, as well as an accurate histological examination, allows a better preoperative characterization of duodenal cancer. At present, the main clinical diagnostic methods for duodenal cancer include endoscopy, ultrasonography, magnetic resonance imaging (MRI) and enhanced CT . EndoscoSurgery is the only radical treatment for SRCC in the duodenum. It has been reported that the 5-year survival rate after radical resection of PDC is approximately 37%-55% , 14. HowHistologically, there are several theories about the origin of SRCs. One theory suggests that SRCs originate in ectopic gastric mucosa found in the duodenum, while another theory suggests that SRCC arises from gastric type metaplastic epithelium –21. In SThe prognosis of SRCC is poor, which may be due, in part, to its more advanced stage. Reports have shown that over 80% of SRCC diagnoses present with advanced disease regardless of the site of origin , 20. In Whereas SRCC is thought to be less chemosensitive than non-SRCC, it may have a specific sensitivity profile and be more sensitive to taxane-based chemotherapy or antiangiogenics –30. BangIn the present case, the tumor was considered to be primary duodenal bulb cancer due to several reasons. (1) Duodenal ulcer was indicated by endoscopy 2 months ago, and no other lesions were found in the stomach. The patient received a proton pump inhibitor (PPI), but his symptoms did not improve. (2) According to the pathology, the tumor was limited to the mucosal layer and submucosal duodenal gland. The proximal margin was negative, while the distal margin was positive. (3) SRCC was not detected in the stomach histopathologically, and no mass was found elsewhere during the operation. Because of the extensive vascular network that increases the risk of bleeding and the thin, deep muscle layer leading to high perforation rates, duodenal biopsy is not routine, and an experienced endoscopist is critical when performing endoscopic evaluation . For patIn conclusion, we report a rare case of SRCC in the duodenal bulb. The diagnosis of PDCs is difficult due to mild and nonspecific presenting symptoms, normal or only minimally elevated tumor markers, and low sensitivity of biopsies. For patients who can receive radical tumor resection, PD should be considered as the first choice. The prognosis of PDC is potentially improved through early diagnosis and radical surgical resection.