Polyps of the uterine cervix are one of the most common benign hyperplastic lesions occurring in the female genital tract that usually arise from the endocervical canal and are believed to be the result of reactive changes due to long-standing chronic inflammation, multiparty, and foreign bodies. Cervical polyps are usually small in size (less than 4 cm) that are commonly found in adult women; however, a few cases of giant polyps and the rare occurrence of these lesions in children have also been reported. Heterotopias and malignant transformation in cervical polyps are considered to be very rare.We present the case of a 10-year-old Afghan girl with a giant pedunculated mass protruding out of the uterine cervix that was accompanied by abdominal pain and mass sensation. The mass was completely excised by surgical intervention and the specimen was submitted for histopathological evaluation. Upon gross and microscopic examination, the characteristic findings of a hamartomatous polyp with heterologous mesenchymal tissue in the form of mature cartilage were seen. To the best of our knowledge, this is the first case of a giant (17.5 cm) hamartomatous polyp of the uterine cervix in this age group.Giant hamartomatous cervical polyps rarely occur in patients below 10 years of age. The majority of these lesions are benign; however, a few cases with malignant transformation are also reported, which demands elaborate investigations into the etiopathogenesis and nature of the lesions. Polyps of the uterine cervix (UC) are one of the most common benign lesions occurring in the female genital tract that usually arise from the endocervical canal and are believed to be the result of reactive changes due to long-standing chronic inflammation, multiparty, and foreign bodies . A cerviA 10-year-old Afghan girl presented to the hospital with lower abdominal pain and mass sensation. Radiological investigation revealed a huge mass attached to the UC with well-defined borders. Upon surgical intervention, the entire pedunculated mass protruding out of cervical canal was removed and submitted for histopathological evaluation.The gross examination of the specimen revealed a well-circumscribed dark-brown firm mass with the gigantic size of 17.5 cm in diameter that showed a smooth external surface Fig. a with moCPs are relatively common benign lesions of the UC harboring focal hyperplasia of the endocervical columnar epithelium which often present as asymptomatic hyperplastic masses and are usually found in parous women aged 30–50 years , 6. In sMalignant transformation in CP is very rare with the prevalence of malignancy reported in 0.1% , 5, dyspWe present a rare case of 10-year-old girl with a giant hamartomatous CP harboring heterologous mesenchymal tissue such as mature cartilage. Although a few cases of large (greater than 4 cm) CP are reported, almost all of the them were found in adults that adds to the uniqueness of our case accompanied by the gigantic size of the polyp. The majority of CPs are benign; however, malignant transformation is also reported, which demands further investigations into the etiopathogenesis and nature of the lesions.


Background: Intimal sarcomas are rare malignant mesenchymal tumors arising from the heart and large blood vessels. Their intraluminal growth leads to vascular obstructive symptoms and peripheral neoplastic embolization. Direct infiltration of the lungs or metastases to the pulmonary system, occur in 40% of cases and extrathoracic spread is frequent, also in presentation. Intussusception is an unusual event in adults, accounting for <5% of bowel obstructions. In most cases it is caused by a malignancy and requires surgical resection.Case Presentation: We describe a rare case of a 50-year-old man suffering of bowel obstruction due to intussusception sustained by a small bowel metastasis of a primary cardiac intimal sarcoma. One year and a half before the onset of abdominal symptoms, a grade II intimal sarcoma was removed from his left atrium and consequently he followed a chemotherapy protocol. Four months later a CT scan revealed local recurrence. Eighteen months after heart surgery he referred to the ER with abdominal pain. CT scan showed an ileal intussusception and the patient was scheduled for surgery. A tract of 10 cm ileus was removed containing an intramural polypoid solid mass. Histological analyses revealed a grade II intimal sarcoma consistent with his first diagnosis.Conclusion: Primary heart tumors are late found and often partially resected, therefore metastatic pathways are to be expected. Adult small bowel intussusception is a rare event and caused by a malignancy in one third of cases. Therefore, our recommendation is to always resect the tract involved in order to perform a proper diagnosis. Primary cardiac sarcomas are extremely rare neoplasms and show a malignant behavior in 25% of cases . In the In this paper, we report an extremely rare case of small bowel intussusception sustained by an intramural polypoid mass, proven to be a metastasis from a PCIS.A 50-year-old Caucasian male patient presented to our Emergency Room (ER) with nausea and abdominal pain.At ER admission, he referred a 3-week history of recurrent abdominal pain. He also referred one episode of vomit and constipation from 2 days.The recent patient history included cardiac surgery 18 months before, aimed to remove a left atrium polylobate neoplastic mass, measuring ~8 cm, partially infiltrating pulmonary veins and the aortic valve. Self-heart transplant and aortic valve plasty were performed, but the ablation resulted incomplete . Pathology examination revealed a moderately-poorly differentiated intimal sarcoma, characterized by proliferation of atypical spindle and pleomorphic cells, with scattered, focally brisk mitotic figures and spotty necrosis. Proliferation index, measured by Ki-67, was 25–30%. Immunophenotyping showed positivity for actin, desmin, myosin, vimentin and MDM2 antibodies; no reaction was observed for the antibodies S100, ALK 1 and CD34. MDM2 gene amplification was observed by FISH analysis. After surgery, CT and PET scan, performed for staging purpose, did not show metastatic lesions. The patient underwent two cycles of chemotherapy with Doxorubicin (75 mg/mq) and Isophosphamide . Four months after surgery, CT scan showed an atrial mass consistent with local recurrence. Oncologic evaluation suggested continuing another cycle of chemotherapy, but it was suspended due to patient's intolerance. Nevertheless, patient accepted to undergo a cycle of 30 fractions of mediastinal radiotherapy (50 Gy + 10 Gy boost). Cardiac ultrasounds performed during follow-up showed no progression of the heart recurrence mass.Physical examination Abdomen was flat and soft and did not show signs of rebound tenderness, no masses were palpable and peristalsis was present.9/L, with normal hematocrit and platelet count. Hemoglobin was 14 g/dL. No other alterations were registered, included C-reactive protein which resulted negative (0.36 mg/dL).Blood test showed mild leukocytosis 11 × 10Abdominal X-rays were normal. A CT scan showed moderate small bowel distention caused by an ileal intussusception containing a 24 mm mild hypodense lesion .Patient underwent urgent laparotomy. The abdominal cavity was explored with identification of the invaginated mid-ileal tract, associated with proximal small bowel dilatation. Intussusception was manually reduced and a 2 cm lesion was palpable inside the lumen. A 10 cm-segmental ileal resection was performed, removing the tract containing the mass with its lymph nodes; consequently, digestive continuity was restored with a manual end to end anastomosis. Pathology examination revealed a malignant mesenchymal lesion composed by atypical spindle and pleomorphic cells with brisk mitotic activity; the immunoprofile was characterized by MDM2, and actine positivity . FISH analysis demonstrated MDM2 amplification. These findings overlapped the features of the previous cardiac neoplastic lesion, so that a diagnosis of intimal sarcoma metastatic to the ileal wall, with submucosal polipoid growth and mucosa ulceration, was made .Recovery was uneventful. Oral feeding was resumed 3 days after surgery, and he was discharged on 5th post-operative day. He started a radiotherapy protocol with administration of 60 Gy of radiation dosage. A chemotherapy schedule based on Doxorubicin plus Isophosphamide was started. Nevertheless, due to high toxicity and low compliance the patient underwent no other chemotherapy cycles, preferring a very strict follow-up. At present, further follow-up exams always showed negative.All diagnosis and decisions were discussed with the patient by a multi-disciplinary team, including oncologists, surgeons, cardiac surgeons and radiotherapists. The patient gave consent to both cardiac and bowel surgery and accepted radiotherapy and chemotherapy. He was also informed of the uniqueness of the case and he consented on publishing it as case report in medical literature.Primary cardiac neoplasms are extremely rare tumors. Their incidence ranges between 0.001 and 0.03%. Approximately 95% of them are soft tissue tumors, most often sarcomas, characterized by an aggressive behavior  demonstrHistologically, intimal sarcomas are usually poorly differentiated high grade malignant mesenchymal tumors characterized by hypercellular proliferation of atypical spindle cells with variable degrees of atypia, brisk mitotic activity, necrosis, and nuclear polymorphism . PromineClinical presentation of a cardiac intimal sarcoma is largely non-specific. Tumor growth can lead to congestive heart failure, dyspnea and embolism. The onset is often late: therefore, systemic embolization, conduction abnormalities, pericardial effusion and metastases (20%) can already be present . MetastaIntimal sarcoma of the heart is related to a poor prognosis. Mean survival is 3–17 months , 10. TheIntestinal invagination consists in the telescoping of a proximal segment of the bowel within the lumen of the adjacent segment. The condition is rare in adults, while it is frequently seen in pediatric population. Adult intussusception (AI) accounts for only 5% of cases, mostly sustained by a lead point that can be a benign lesion or a malignancy  , 15. An CT scan is the pivotal examination for the diagnosis of AI; nevertheless, in the case of finding an intestinal mass, it is very difficult to characterize the tumor. Moreover, at CT scan imaging, intimal sarcoma can be misdiagnosed as a GIST. In these cases, immunohistochemical analysis is crucial to rule out the difference. GISTs usually stain positive for CD34 and, in ~88% of cases, for both CD117 and DOG-1. In a recent study, a positive expression of CD117 and DOG-1 in gastrointestinal stromal tumors was shown in 95.71 and 88.57% of cases respectively . MalignaAI is responsible of 1% of all bowel obstructions  and its The main surgical strategies are primary en-bloc resection vs. initial reduction. Both laparotomic and laparoscopic approaches are described, depending on the clinical condition of the patient, the extent of intussusception and the presence of an underlying disease . In mostThe reported case shows some interesting aspects. Intimal sarcoma is the most common entity among cardiac tumors, even if extremely rare. Some clinic-pathological features show similarities with literature data, including age, site and local relapse –37. In fTwo lessons may be learned from our case. First of all, diagnosis of heart tumors is often late, compromising complete ablative surgery and therefore facilitating an expected metastatic spread. Secondly, adult small bowel invagination is a rare event, sustained in one third of cases by a malignancy and it is advisable to resect the affected intestinal tract in order to perform proper diagnosis.The original contributions presented in the study are included in the article/MC and MZ designed the study, analyzed the data, and revised the manuscript. FT designed the overall concept of the study and revised the manuscript. MB, MD, FG, and AG contributed to data acquisition and drafted the manuscript. CR, AV, and ED contributed to laboratory analyzes and draft the pathology section. UC contributed to the design the study and reviewed the discussion and conclusions. All authors approved of final the version of the manuscript to be published.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.


Keratoacanthomas (KA) are epithelial tumors that present as rapidly evolving nodules with a central hyperkeratotic plug and occasionally show signs of spontaneous regression. A 21‐year‐old patient strongly refused the diagnostic biopsy and insisted on a nonsurgical treatment. He was successfully treated with imiquimod 5% cream. Surgical excision is the most common treatment option for keratoacanthomas. However, the topical application of the imiquimod 5% cream is a safe and effective option, especially for patients who refuse surgical removal. The lesion rapidly progressed in size during the first 4 weeks and afterward remained stable. The clinical examination revealed a firm skin‐colored nodule, of approximately 1.8 × 1.8 cm in size, with a central non‐removable keratinous plug Figure . The cliKeratoacanthomas (KA) are epithelial tumors that present as rapidly evolving nodules with a central hyperkeratotic plug and occasionally show signs of spontaneous regression.There are no conflicts of interest to declare.FM, EL, DB, LT, G‐IV, IP, FI, CK, VT, and K‐MP contributed to the clinical data collection and prepared the case report. FM, SG, and K‐MP contributed to the design of the case report presentation and performed the final revision of the manuscript.The written informed consent was obtained from the patient for publication of this case report.


Inflammatory myofibroblastic tumor (IMT) is a very rare subtype of sarcoma, which frequently harbor chromosomal rearrangements, including anaplastic lymphoma kinase (ALK) rearrangements  and other kinase fusions such as ROS1. ROS1 fusions are present in about 10% of IMT, almost half of the ALK-negative IMT patients. Apart from radical surgery for resectable tumors, there is no standard-of-care therapy for advanced IMTs. Nonetheless, the use of tyrosine kinase inhibitors has shown promising efficacy in IMT patients with targetable genomic alterations. We report the case of a 24-year-old patient with chemotherapy-refractory metastatic IMT harboring ROS1 kinase fusion, who experienced a significant clinical and pathological response to crizotinib. This clinical case highlights the need to assess all patients with unresectable IMTs for chromosomal abnormalities and gene mutations and address them to targeted agents as well as clinical trials. Inflammatory myofibroblastic tumor (IMT), also known as inflammatory pseudotumor, is a rare mesenchymal soft tissue tumor characterized by myofibroblastic spindle-cells and a prominent inflammatory infiltrate in an extracellular myxoid/collagenous stroma , 2. IMT IMT shows a tendency for local recurrence with low risk of distant metastasis and radical surgery is the mainstay of treatment , 3, 5. FAlmost 50% of the IMTs harbors rearrangements involving the anaplastic lymphoma kinase (ALK), which seem to be associated with localized tumors and better prognosis compared to ALK-negative IMT . A substGiven the rarity of IMT, few clinical trials and case series are available on targeted therapy in ALK-positive IMT –34. NoneMoreover, very little data are currently available on the prognosis of ALK-negative IMTs and the role of ROS1 as a driver and potential target in IMT has been documented only in two single cases –20.Here we reported the clinical case of an advanced IMT patient harboring a ROS1 rearrangement who extraordinarily responds to crizotinib Figure 1rectus femoris and the tensor fasciae latae muscles, associated with enlargement of homolateral inguinal lymph nodes, radiologically suspected for reactive lymphadenopathies, but not clinically palpable for the presence of the main lesion in the proximal part of the thigh.In April 2018 a 23-year-old Caucasian man presented to the general practitioner for the appearance of a rapidly increasing palpable mass in the proximal part of the left thigh in the last month. His clinal history was negative, as well as his oncological family history. He worked as a plumber and did not report any traumatism. He was a light smoker (less than 5 cigarettes per day for 3 years) and denied the consumption of alcohol or drugs. The clinical examination revealed only a solid lesion of 6 cm of the upper third of the left thigh which was hard, fixed on the deep plane and not painful. A magnetic resonance imaging (MRI) scan was performed, showing an enhancing solid lesion of 50 × 45 × 60 mm localized between the In May 2018 a biopsy of the mass was performed, revealing an initial diagnosis of nodular fasciitis, and consequently the patient was addressed to follow up. In consideration of the clinical dimensional increase of the mass, a new MRI was performed in July 2018 confirming the increase of the left thigh lesion (54 × 64 × 75 mm). A surgical approach was, therefore, planned but the pre-operative blood tests reported an important elevation of serum transaminases values . According to the suspicion of liver metastases, a staging Total Body (TB) computed tomography (CT) scan was performed in November 2018, but no secondary lesions were shown. According to the hepatological consultation, the transaminase elevation was not amenable to a viral or autoimmune disease, but a paraneoplastic syndrome.The radical surgery was, therefore, planned in February 2019 but the pre-operative 18F-FDG-PET revealed, in addition to the main tight lesion (SUV max 23), the presence of two paravertebral lesions (SUV max 21), suspected for metastases, and a minimal uptake of the homolateral inguinal lymph nodes (SUV max 2.4), questioning the initial diagnosis of nodular fasciitis.In consideration of the different uptake compared with the main lesion, the inguinal lymph nodes were considered reactive lymphadenopathies.A new biopsy was performed in February 2019 with the diagnosis of myofibroblastic sarcoma. Considering the complexity of the case, in agreement with the patient, a second opinion from an expert pathologist was asked by the first hospital where the patient was followed.The TB CT scan of March 2019 showed the left thigh mass of 170 × 135 mm, a similar mass in the context of the left iliopsoas (40 × 25 mm) and gluteus muscles (19 × 21 mm), other two tumoral nodules in the dorsal muscles next to D11 vertebral body (37 × 30 mm) and L5 vertebral body (40 × 30 mm) 3A. The In the same month, in consideration of the rapid progression of the primary lesion  for the assessment of ALK was performed but the result was negative. A next-generation sequencing (NGS) of the tumoral tissue was subsequently performed and revealed the presence of the YWHAE1-ROS1 fusion (images not available).According to the absence of clinical and radiological response to chemotherapy 4B, the In August 2019, after 3 months of therapy, an outstanding clinical response was observed  . The neoplastic foci displayed spindle-shaped cells embedding in a loose myxoid stroma  the TB CT scan and the subsequent MRI , the experts suggest performing FISH analysis for the evaluation of ALK fusions , 36. In In 2010 the first case of ALK-positive IMT responding to crizotinib was reported by Butrynski et al., as compared with no response observed in an ALK-negative IMT patient receiving the same treatment . Other cThe CREATE trial was the most important study assessing the activity and safety of crizotinib in advanced or inoperable IMTs with and without ALK alterations . ALK-posThese data suggested the use of crizotinib only in the ALK-positive population, but it is important to notice that the mutations in the ALK-negative group were not reported and the number of patients was very small.The first case of a ROS1-positive pulmonary IMT pediatric patient responding to crizotinib was reported by Lovly et al. . SimilarIn consideration of these promising results and those observed in ROS-1-positive non-small cell lung cancer patients, we decided to propose crizotinib to our patient. This clinical case is the first case reported in the literature on an adult patient with a ROS1-rearranged IMT of soft tissues who experienced an outstanding clinical and radiological response to crizotinib.However, this clinical case is not interesting only for the rarity of the tumor and the exceptional treatment response, but also for the different clinical points in the decision-making of such a rare tumor. Considering the evident limitation due to the absence of literature data, many clinical concerns were raised during the clinical management of the patient. Every diagnostic and therapeutic choice has been taken considering these limits and was previously discussed with the patient.After the excellent clinical response to crizotinib, we opted for wide excision after 8 months of therapy for the following different reasons. Neoadjuvant or adjuvant therapies for IMT patients have been reported in few clinical cases and seemed a feasible approach –42. The In the after-surgery scenario, the main problem was to decide whether to resume the treatment with crizotinib even in the absence of evident disease or not. In the literature, very few reports have been written on neoadjuvant or adjuvant use of targeted therapy or chemotherapy in IMTs –42. NeveThe key point then was to determine for how long this therapy should be continued. A recent retrospective study published by Trahair et al. reported eight cases of ALK-positive IMT children and adolescents treated with crizotinib and surgery with different stages of disease . The medAt disease progression, after therapy discontinuation, we decided to resume crizotinib because, as reported in the literature, new responses are still possible and, moreover, in this rare disease other effective therapies are still not approved and difficult to get the access , 42–45.In fact, in ALK-positive IMT patients, after progression to crizotinib, different ALK inhibitors, such as ceritinib, alectinib and lorlatinib, have been investigated reporting promising responses , 42–45.Also in ROS1-rearranged patients, especially those with NSCLC, other ROS1 inhibitors  have been investigated and may be a possible future therapeutic option in ROS1-positive disease, including IMT –50.In this scenario, molecular assessment is recommended in ALK-negative IMT patients to identify potentially actionable mutations for which new target therapies are available . FurtherThe 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 patient for the publication of any potentially identifiable images or data included in this article.DC, FCa, and SER: conception, design, writing and review of the manuscript. FCa, MG, and GP: draft of the manuscript and analysis of the patient’s data. RB, MM, FDC, and FP: clinical management of the patient and provision of patient’s data. AG and BS: elaboration and analysis of the pathology. All authors contributed to the article and approved the submitted version.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.


Craniopharyngiomas are supra/parasellar lesions that often present with general, unspecific symptoms. Similarly, internal carotid artery (ICA) bifurcation giant aneurysms may also produce calcified, heterogeneous, parasellar expansive lesions, posing a relevant differential diagnosis due to their inherently different surgical strategies and risks. We report the case of a 54-year-old female presenting with progressive disorientation and apathetic behavior. CT and MRI reports described a suprasellar heterogenous mass with calcifications associated with an adjacent, laterally located fluid collection suggestive of a craniopharyngioma. During the surgical procedure, perfuse and unexplained arterial bleeding ensued, prompting the surgical team to review a previous contrast-enhanced CT scan. Careful inspection revealed an image suggestive of vascular pathology, with an area of continuous hyperdensity along the right ICA bifurcation. The Sylvian fissure was dissected, and an aneurysmal neck was encountered and successfully clipped. Giant intracranial aneurysms are rare but essential differential diagnoses to be considered during the workup and surgical approach toward parasellar mass lesions. This case illustrates the importance of performing a CT angiogram (CTA) for skull base lesions, even when the size is more suggestive of tumor pathology. The differential diagnosis of lesions of the sellar, parasellar, and suprasellar regions of the central nervous system (CNS) includes various lesions with varying etiologies. Not all of them are neoplastic (and more recurrent), such as the more common Rathke cleft cysts, pituitary adenomas, and craniopharyngiomas. There are granulomatous processes, autoimmune diseases, and immunocompromised state-related infections among rare cases. These cases potentially impose significant challenges in establishing the proper diagnosis and clinical conduction because of the low frequency in this anatomic region. They may present similar signs, symptoms, and radiological findings . TherefoCraniopharyngiomas are calcified, cystic, or mixed solid and cystic tumors. Embryologically, they originate from Rathke's pouch and extend into the suprasellar midline region, often directed into the third ventricle, along the pituitary stalk and the optic chiasm . Main neHowever, some lesions might not be amenable to a straightforward diagnosis due to their unusual presentation on simple imaging, and their true nature is only revealed either with a more detailed workup with vascular studies or only intraoperatively. In addition, carotid bifurcation giant aneurysms may also produce calcified, heterogeneous, parasellar expansion-masses mimicking the appearance of a craniopharyngioma, posing a relevant differential due to their inherently different surgical strategies and risks . In the We report the case of a patient presenting with a heterogeneous, calcified, parasellar/suprasellar mass lesion measuring 5.1 cm, with an apparent 6.0-cm cystic-degeneration; it was reported as a probable craniopharyngioma on a radiologist's notes, and which was revealed intraoperatively to be a giant, calcified aneurysm upon its inadvertent incision, subsequent bleeding, and successful treatment with direct clipping.A 54-year-old female patient presented to the emergency room with progressive disorientation, apathetic behavior, and bilateral lower limb weakness. She underwent a CT scan to rule out bleeding, which was followed by a contrast-enhanced CT scan when a suprasellar mass was identified. MRI showed a suprasellar heterogenous mass with calcification areas associated with an adjacent, lateralized fluid collection. Intraoperatively, further contrasted CT reconstruction evidenced a hyperdense image within the tumor wall, continuous with the top of the right carotid artery. The radiological report suggested a craniopharyngioma Figure . The patAn extended pterional incision and a fronto-orbitozygomatic approach for the resection of the tumor were planned. A middle frontal gyrus corticectomy was performed to gain access to the cystic portion of the lesion. The cyst was drained, and a firm lesion was noticed in the cavity. The lesion was dissected and debulked for volume reduction and easier maneuverability.During debulking, high-volume arterial bleeding ensued. It was not compatible with the injury of a standard, tumor-related artery. Hemostasis was achieved with fibrillar hemostats and irrigation under pressure. The high-flow bleeding and location of the lesion raised suspicion for an aneurysmal lesion of the internal carotid artery (ICA). The Sylvian fissure was dissected immediately, and an aneurysmal neck was encountered at the ICA bifurcation Figure  and clipGiant intracranial aneurysms are arterial malformations greater than 2.5 cm that represent only 2-5% of all intracranial aneurysms ,9. They As expansion lesions, giant aneurysms might present cystic, degenerated, heterogeneous, lobulated, and calcified lesions when unruptured ,8. Due tDue to limited resources and the patient being admitted with contrasted imaging suggestive of a tumor, a CT angiogram (CTA) was not performed since it was not deemed essential by the hospital's administration. However, we believe that this type of advanced vascular imaging would have helped diagnose the aneurysm before the surgery.We reported a case of a giant aneurysm presenting as a parasellar/suprasellar lesion that resembled a craniopharyngioma. Advanced vascular imaging would likely have helped with the proper diagnosis of the lesion. The radiological findings and the unspecific symptom onset point to later diagnoses in these reported cases, with their true natures being unveiled only during intraoperative dissection or during preoperative vascular assessments when available or considered. Although our case was resolved without nefarious consequences, knowing what pathology was being operated on would have allowed for more adequate surgical planning and avoided significant intraoperative hemorrhage and potential harm. We recommend that surgeons increase their efforts in requesting advanced vascular imaging when faced with lesions of the sellar/parasellar regions, as there may be more to them than meets the eye.


Gastric hamartomatous inverted polyp (GHIP) is rare, with few reports of carcinogenesis from GHIP during long‐term follow‐up. A 51‐year‐old woman was diagnosed as having a submucosal tumor (SMT) during esophagogastroduodenoscopy (EGD) in 2008. In 2016, although the size and height of the lesion had not changed, she was referred to our hospital for further investigation of the lesion. EGD depicted a gastric SMT of 20 mm in diameter in the greater curvature of the upper gastric body, and a biopsy specimen showed a well to poorly differentiated adenocarcinoma. Following successful laparoscopic total gastrectomy, histopathological examination revealed an intramucosal adenocarcinoma arising in GHIP. Gastric hamartomatous inverted polyp (GHIP) is characterized pathologically by a submucosal mass of lobulated gastric mucosa, which has a centrally located lumen and is surrounded by smooth muscle communicating with the muscularis mucosa.Helicobacter pylori antibody was negative. EGD identified a gastric SMT of 20 mm in diameter with a positive cushion sign in the greater curvature of the upper gastric body. The lesion exhibited a depression suspected as the crypt opening and dilated vessels with a branching architecture around the depression on the proximal side of the lesion  on esophagogastroduodenoscopy (EGD) first detected in 2008. The patient had undergone annual EGD to follow‐up with no remarkable findings. A biopsy specimen taken in 2011 also showed no evidence of malignancy. On presentation, she had no clinical symptoms, family history, or laboratory data abnormalities, including CEA and CA19‐9. Serum n Figure . There wn Figure . Endoscon Figure . An uppen Figure . A biopsn Figure . On immun Figure . No lympn Figure 3. No reWe encountered the rare case of a gastric adenocarcinoma arising from GHIP requiring total gastrectomy after a long and uneventful follow‐up period of 8 years.GHIP is characterized by marked submucosal glandular proliferation associated with cystic dilatation that is accompanied by dendritic proliferation of the smooth muscle bundle.It was unclear whether the carcinoma in this case had existed from the first identification of the SMT or had become cancerous during follow‐up. Considering the absence of malignant findings in previous biopsies and a report on early‐stage gastric cancer progressing to advanced gastric cancer within 34 months,Since GHIP presents as an SMT‐like lesion covered with normal gastric mucosa, assessment by EUS is useful for detecting GHIP and visualizing hyperechoic lesions, including small cystic hypoechoic spots, in the third layer.The authors declare no conflict of interest.All procedures performed in this case were in accordance with the ethical standards of the institutional committee and with the Helsinki declaration and its later amendments or comparable ethical standards. An informed consent was obtained from the patient for the publication of this case report.None.


Congenital brain tumors are extremely rare in the neonatal population, and often associated with a poor prognosis. The diagnostic suspicion is often aroused at antenatal scans or postnatally, if clinical signs and symptoms of increased intracranial pressure become evident. We present a case of definitely congenital glioblastoma multiforme incidentally diagnosed in a preterm infant, aiming to raise clinical awareness on this condition and to highlight the challenges of the related diagnostic work-up.This female infant was born at 31 weeks’ gestation after an uneventful pregnancy. No abnormalities were detected at antenatal ultrasound scans and genetic tests. Head circumference at birth was on the 25th centile. A routine brain ultrasound scan performed on day 1 revealed a large, inhomogeneous lesion in the right cerebral hemisphere, with contralateral midline shift, which was confirmed by brain magnetic resonance imaging (MRI). Eye fundus and routine blood exams, including platelets count, coagulation screening and C-reactive protein, were normal.Given the high risk of complications, surgical biopsy of the lesion was temporarily hold and a daily sonographic follow-up was undertaken. Although head circumference growth was steady on the 25th centile, progressive changes of the lesion were detected by cranial ultrasound.The repeat MRI scans showed a significant enlargement of the mass, with contralateral midline shift and signs of intralesional and intraventricular bleeding. In view of this worsening, surgical resection was performed. The histological examination of the lesion biopsy documented a GFAP+ highly cellular neoplasm, with no mutation on SMARCB1 gene. At the molecular analysis, mutations on IDH and H3F3A genes were absent, whereas MGMT promoter was unmethylated. The diagnosis was grade IV glioblastoma IDH wild-type.Congenital glioblastoma multiforme is an extremely rare but highly aggressive neoplasm. Since intralesional biopsy is not often feasible in affected neonates, knowledge of the associated clinical and neuroradiological features is particularly important, as they can also add useful information on the neoplasm behavior. Specimens from open surgical resection allow to perform a definite histological analysis and an extended molecular characterization, with relevant prognostic implications. With an estimated global incidence of 3.6 to 4.1 per 100.000 births, congenital brain tumors (CBTs) are extremely rare, accounting for only 0.5–1.9% of overall brain neoplasms in the pediatric population . AccordiCongenital CBTs can be incidentally detected at routine fetal scans as an intracranial mass, with or without hydrocephalus, whereas progressive macrocephaly is the postnatal sign most frequently observed . The estWe describe a case of definitely congenital glioblastoma multiforme, incidentally diagnosed in a preterm infant soon after birth, aiming to raise clinical awareness on the neuroimaging and pathological features associated with this condition and to highlight the challenges encountered in the related diagnostic work-up.This Caucasian female infant was born at 31 weeks’ gestational age (GA) by spontaneous vaginal delivery in a dichorionic twin pregnancy. Birth weight was 1313 g (25th pc) and head circumference (HC) was 28 cm (25th pc). While recurrent miscarriages were noted at maternal history, pregnancy history was uneventful. Antenatal ultrasound scans showed no abnormalities (last scan performed at 28 weeks’ GA) and chorionic villus sampling documented a normal female karyotype. Due to her prematurity, the baby was admitted to the local Neonatal Intensive Care Unit shortly after birth. Clinical examination at admission was unremarkable; the anterior fontanel was soft and tender. On day 1, a routine cranial ultrasound scan (CrUSS) was performed, revealing a large inhomogeneous lesion in the right cerebral hemisphere, with contralateral midline shift  scan without contrast medium was urgently performed on day 2, documenting a well-defined intra-axial right frontoparietal lesion, with evidence of intralesional hemorrhagic areas at different stages, homolateral ventricular bleeding and midline displacement due to mass effect Fig. a, right.A joint consultation between the local neonatal, neurological and neurosurgical teams was performed, followed by an off-site neuroradiological and neurosurgical consultation; given the high risk of complications related to the infant’s GA and very low weight, surgical biopsy was temporarily hold. A MRI-angiography (MRA) was thus scheduled, and a daily sonographic follow-up of the lesion was undertaken.Although the HC growth was steady on the 25th centile, progressive changes in the distribution of intralesional hypo- and hyperechoic areas and a slight increase in the size of the lesion were documented at CrUSS Fig. b, left. On this basis, a new neurosurgical consult was performed; since surgical risks were still remarkably high, clinical and sonographic follow-up was temporarily continued. The baby remained stable, with no signs of cranial hypertension nor focal neurologic symptom until day 28, when a remarkable enlargement of the lesion was observed at CrUSS Fig. c, left; The histological examination of the lesion biopsy showed a highly cellular neoplasm, composed of poorly differentiated glial cells with nuclear hyperchromasia, atypia and brisk mitotic activity Fig. a. Foci oThe molecular analysis was performed according to standard procedures with a GeneStudio S5 (ThermoFisher) NGS platform, targeting the relevant mutational hotspots of IDH1 (Exon 4), IDH2 (Exon 4) and H3F3A (Exon 1). MGMT promoter analysis was carried out according to previously reported protocols . MutatioAn oncological consultation was performed; in light of the highly aggressive features of the neoplasm and the ensuing poor prognosis, together with the infant’s prematurity and low birth weight, palliative care was undertaken in conjunction with the family of the baby, who died 17 months later.Glioblastoma multiforme (GBM) is a very rare but highly aggressive brain tumor, associated with a poor prognosis in both children and adults. Over the last 100 years, a total of 67 cases [In most of the cases previously reported, the diagnostic suspicion of congenital GBM was either aroused at antenatal scans and confirmed by fetal MRI , or raisBeing non-invasive and easily accessible, CrUSS represents the first-line investigation in the diagnostic work-up of CBTs, which appear in the differential diagnosis of unexplained parenchymal hemorrhage, intraventricular hemorrhage, or brain abscesses . TypicalMultimodal MRI is an essential milestone for the differential diagnosis of CBTs. At MRI, GBM appears as a highly vascular lesion with a cystic-solid consistency, heterogeneous signal intensity and contrast enhancement, and a restricted diffusion due to its high cellularity and mitotic activity . AlthougDespite neuroimaging findings may be highly suggestive of GBMs, lesion biopsy allows not only a definitive diagnosis of GBMs, which is based on the evidence of such histological findings as high mitotic activity, microvascular proliferation and pseudopalisading, but also an extended molecular characterization . HoweverIn the present case, IDH and H3F3A mutations were absent and MGMT promoter was unmethylated. This is consistent with Gielen et al., who reported significantly lower rates of genetic alterations in congenital and infant GBM cases compared with older children and adults , hypotheCongenital glioblastoma multiforme is an extremely rare but highly aggressive neoplasm. Knowledge of the associated clinical and neuroradiological features is particularly important, as they can also add useful information on the neoplasm behavior. Specimens from open surgical resection allow to perform a definite histological analysis and an extended molecular characterization, with relevant prognostic implications for the therapeutic or palliative management.


In addition, variations of the root canal system of premolars must thoroughly be understood. 3D radiographic evaluation of the patient should be conducted before disimpaction to establish proximity to the mandibular canal and borders of mandible. In addition, variations of the root canal system of premolars must thoroughly be understood. In this report, we describe the detailed clinical and radiographic evaluation, including considerations of key factors prior to any surgical or endodontic intervention in such a patient.Tooth impaction is a pathological process whereby the tooth does not erupt into its functional position due to physical or genetic factors.Abnormal root morphology of the maxillary premolars, although uncommon, has been reported previously in literature involving relatively rare cases such as presence of a third root of the first maxillary premolar and a two‐rooted second maxillary premolar in about 25% of cases.We hereby report a unique combination of two rare dental findings in the same individual which could pose future treatment challenges: bilateral kissing molar impactions and bilateral three‐rooted second maxillary premolars. In addition, we discuss the relationship of the molar apices to the mandibular neurovascular bundle and to the inferior border of mandible, its surgical implications and the endodontic considerations of a three‐rooted second maxillary premolar.2A 27‐year‐old African male patient presented to the Department of Oral and Maxillofacial Surgery, University of Nairobi, Kenya, with spontaneous, severe pain of left mandible for 7 days. Intraoral examination revealed bilateral Angle's class 2 molar relationship. The maxillary canines were missing (disimpactions performed 10 years ago), and carious lesions were present on 17 and 36 with both teeth tender to percussion.A panoramic radiograph revealed radiolucencies with probable pulpal involvement on the mesial aspect of 17 and 36. The maxillary third molars were impacted. Mandibular second and third molars 37, 38, 47, 48) were impacted bilaterally with their occlusal surfaces in contact and roots pointed in opposite directions . Both had divergent mesiobuccal, distobuccal, and palatal roots with corresponding root canals. Figure  The pati3KMs or “rosette formation” is a rare phenomenon first described in 1973 where the molars are in occlusion within a single, enlarged follicular space.Management of symptomatic KMs necessitates surgical intervention while that of asymptomatic KMs entails maintenance of the molars within the jaw.Anatomic variations of maxillary second premolars are well documented in the literature; however, trifurcated second premolars are rare.4A thorough clinical and radiographic evaluation is a key in ensuring adequate management. Whereas panoramic imaging is considered the gold standard, CBCT is highly recommended in order to evaluate the 3D position of such impactions and their relationship with critical structures before surgical intervention. Taking all diagnostic factors of this case into account, long‐term follow‐up was recommended due to the proximity of the molar apices to the IAN and inferior border of mandible.No conflict of interest to declare.Krishan Sarna and Ian Murithi collected the data and wrote the findings of the manuscript while Dr. Florence Opondo and Professor Symon Guthua supervised and provided critical guidance in radiologic assessment, interpretation and final preparation of the manuscript. All authors approved the final manuscript.The manuscript was prepared according to standard publication ethical guidelines.


Ganglioneuroblastoma (GNB) is a neoplasm that arises from the primitive cells of the sympathetic nervous system during childhood. The current case is very unique because of the initial primary tumor manifestation in the orbit and an adrenal tumor being observed later during follow-up.A 2-year-old girl presented to the Ophthalmology Department of our hospital complaining of swelling of the left upper eyelid for approximately one month. Orbital computed tomography (CT) revealed a left orbital mass with bone destruction. Thoracic and abdominal CT indicated no abnormalities. The mass was surgically resected, and histopathological analysis confirmed it as GNB. During follow-up, abdominal CT detected an adrenal tumor with internal calcification, a calcified nodule on the left side of the abdominal aorta, and mesenteric lymph nodes. Accordingly, primary orbital GNB and metastatic adrenal GNB were the possible considerations. We removed the adrenal tumor, and the patient underwent chemotherapy. However, the patient died 18 months after the ophthalmic surgery.Primary orbital GNB in children is easily misdiagnosed because of its rare occurrence and atypical clinical findings. Imaging methods combined with histopathological examination contribute to the detection and diagnosis of primary and metastatic GNBs. Thus, timely surgery combined with adjuvant chemotherapy and long-term follow-up is essential for controlling the metastasis of GNB and improving the survival rate of patients. GNB, fi2A 2-year-old girl was admitted to the Ophthalmology Department of our hospital with a swelling of the left upper eyelid for approximately one month. No redness in the eye or other abnormal symptoms were observed. There were no pregnancy or childbirth-related abnormalities reported by the patient’s mother. Also, no familial anamnesis of cancer existed.During the initial clinical assessment, the patient had normal growth and good nutritional status and had no abdominal symptoms such as abdominal pain and abdominal swelling. Thoracic and abdominal CT revealed no detectable abdominal masses. On ophthalmic examination, a mass on the lateral superior orbital rim of the left eye, with congestion and edema of the superotemporal conjunctiva and swelling of the lower eyelid, was detected . OrbitalTo determine the composition of the left orbital mass, the patient underwent a resection surgery under general anesthesia. During the surgery, we found that the mass was growing close to the lateral and superior wall of the orbit and showed a cord-like shape, dark red color, and an irregular surface. It adhered extensively to the surrounding tissues, with adjacent bones widely destroyed. The mass was removed as much as possible. On a general observation, the two tumor masses measured approximately 2.5 cm × 2 cm × 1.5 cm and 2.5 cm × 1.5 cm × 1 cm . Postope3Neuroblastic tumors are the most common childhood solid extracranial tumors, and GNB constitutes 20% of these tumors . ClinicaIn 60–70% of cases, metastases are present with primary lesions at the initial diagnosis . Two casSurgical resection is the mainstay treatment for GNBs, and chemotherapy is recommended for high-risk patients ,22. In tTo the best of our knowledge, this is the first reported case of primary orbital GNB and metastatic adrenal GNB. The four previous case studies associated with orbital GNB have been reported abroad, and we could not find similar domestic studies on literature review. Two of these studies reported cases with both adrenal GNB and orbital GNB. One study reported the case of a 10-month-old girl with metastatic orbital GNB and primary adrenal GNB. She underwent chemotherapy and survived after 7 years of follow-up. Another study reported a 2-year-old girl with primary adrenal GNB and metastatic lesions including the calva, orbit, and bone marrow. She underwent neoadjuvant chemotherapy, surgery, and bone marrow transplantation and survived without recurrence .4Our report highlights the importance of early diagnosis and intervention for primary orbital GNB in children with an orbital bony lesion, especially in cases with a primary orbital tumor with “raccoon eyes.” Imaging methods combined with histopathological examination can contribute to the accurate diagnosis of the primary and metastatic lesions, particularly in the adrenal gland. Furthermore, timely surgery combined with adjuvant chemotherapy and long-term follow-up is essential for reducing the recurrence rate and metastasis of GNB and for improving the survival rate of the patients.


Teaching point: An inflammatory pseudotumor can occur almost everywhere in the body and has nonspecific imaging findings. Figure 1, arrows). The lesion was located on the mesentery and contained multiple central areas of non-enhancement, suggesting necrosis. Some small central intralesional calcifications were also present. Additional 18-Fluoro-Deoxy-Glucose positron emission tomography – CT showed enhanced metabolism of the lesion . No lesion suspicious for metastasis was present. An ultrasound-guided biopsy was performed, and the histopathological examination made the diagnosis of an epithelioid variant of an inflammatory myofibroblastic tumor. Laparotomy with resection of the tumor followed, which confirmed the mesenteric origin of the inflammatory myofibroblastic tumor. There was tumor invasion of the right hemicolon and terminal ileum, which were also resected during surgery. Follow-up CT three months later, revealed extensive local tumor recurrence at the level of the mesentery with associated diffuse liver metastasis and ascites . Palliative treatment was initiated.We present the case of a 73-year-old woman, referred to the gynecologist for the workup of a mass found in the lower abdominal region during clinical examination. Subsequent vaginal ultrasound was suggestive of a mass in the pelvic cavity. A body computed tomography (CT) confirmed the presence of a heterogeneously enhancing, partially ill-defined lesion in the lower abdomen with dimensions of 12 × 9 × 11 cm (An inflammatory pseudotumor (IPT) is composed of cells involved in acute and chronic inflammatory processes. Inflammatory myofibroblastic tumor is a term, which is sometimes used instead of IPT, due to the presence of myofibroblasts and histiocytes in the lesion. IPT’s can present as a single mass or multiple masses and most often occur in the orbit or lung. Nevertheless, IPT’s can be found almost everywhere in the body. A mesenteric IPT, as in our case, is an uncommon manifestation, especially since this entity tends to occur more in young adults or children, in contrast to our 73-year-old patient. A mesenteric IPT can have well-defined boundaries or an ill-defined infiltrative margin with possible associated invasion of adjacent bowel loops. The enhancement pattern can be variable, with homogeneous or heterogeneous enhancement. Associated central calcifications and areas of necrosis can be present. These non-specific and variable imaging characteristics make it difficult to differentiate from other disease entities, such as lymphoma or soft-tissue sarcomas. Finally, the clinician should provide a higher rate of surveillance for patients with an epithelioid variant of intra-abdominal IPT. These epithelioid variants of intra-abdominal IPT’s often have a more aggressive behavior, with a faster recurrence rate .


Central precocious puberty (CPP) is a condition that causes early gonadotropin-dependent sexual development; CPP is idiopathic in girls in most cases, whereas more than 50% of boys have an identifiable etiology. We conducted a qualitative systematic review following the ENTREQ  framework. A search was made in MEDLINE/Pubmed and MeSH Database using the terms “precocious puberty” AND “brain tumor” OR “posterior fossa tumor” OR “cerebellar tumor” OR “infratentorial tumor”, identifying five cases of pediatric patients with infratentorial tumors and CPP and a case of cerebellar ganglioglioma without hypothalamic−pituitary−gonadal axis involvement and/or intracranial hypertension. Our work highlights the importance of a multidisciplinary approach and extensive central nervous system imaging for patients presenting with CPP in order to detect possible tumor association. Moreover, we believe that this manuscript could contribute to stimulate other research because the exact mechanism of CPP in infratentorial brain lesions has not been understood yet. Precocious puberty (PP) is defined as the development of secondary sexual characteristics before the age of eight years in girls and nine years in boys ,2. PP maAn 11-year-old boy was referred to our pediatric endocrinology department for suspect PP (onset of pubic hair at the age of 8.5). The patient’s weight was 49 kg (75–90p), height was 156.3 cm (97p) (Tanner’s target height 165.6–176.6 cm) and testicular volumes were 15 mL (Tanner stage 4 puberty). The bone age was three years older than the chronological age (14 years old according to Greulich Pyle Atlas). Laboratory work up revealed basal LH 2.94 mUI/mL  FSH 3.58 mUI/mL , testosterone 4.23 ng/mL , free testosterone 23.15 pg/mL . After LH-FSH-releasing hormone test, a peak LH value of 35.1 mUI/mL and a peak FSH value of 9.01 mUI/mL (after 4 h) were detected. ACTH stimulation test was performed: 17 OH-P rose from a basal value 1.56 ng/mL to 6.25 ng/mL at 60 min; levels of DHEA-S, delta-4 androstenedione and cortisol remained within normal range. Late onset (non-classic) 21-hydroxylase deficiency was excluded by genetic analysis. Plasma thyroxin, thyroid-stimulating hormone, serum beta-hCG and alfa-fetoprotein concentrations were within normal range for age. One month after pediatric endocrinology consultation, brain MRI detected a mixed solid and microcystic left hemispheric cerebellar lesion growing into the cerebellomedullary and cerebellopontine cisterns, partly infiltrating the middle cerebellar peduncle, with a small extension into the jugular foramen. The solid component showed an inhomogeneous hyperintensity on T2-weighted turbo spin echo (TSE) and fluid attenuated inversion recovery (FLAIR) sequences. T1-weighted contrast-enhanced sequences showed two foci of centimetric nodular enhancement in the posterior lobe. Hydrocephalus was absent. Sagittal post-contrast T1-weighted images of the spine showed no leptomeningeal spread .The patient was diagnosed with CPP and started gonadotropin releasing hormone (GnRH) agonist treatment (triptorelin acetate 3.75 mg every 90 days). He was referred to the pediatric oncology department for preoperative evaluation.The patient did not complain of neurological symptoms and neurological examination was unremarkable.Chest x-ray, abdomen and testicular ultrasound, cardiological, neuropsychiatric and genetic consultation did not reveal any abnormality. A minimal left hypoacusia was detected by otolaryngologists. The patient was operated on via a left retrosigmoid approach on lateral position. Intraoperative neuromonitoring (IONM) was performed recording brain stem auditory evoked potentials (BAEPs), motor and somatosensory evoked potentials and V VII, IX, X, XI cranial nerve monitoring. Baseline BAEPs showed a decreased function on the left. During and after surgery no further changes were recorded. Early postoperative MRI showed a small tumor residue in the middle cerebellar peduncle, hyperintense on T2-weighted TSE and FLAIR sequences, with no contrast enhancement on T1-weighted images .Postoperative neurological examination reported a mild sensorineural left hearing loss and a transient diplopia. The boy was discharged on postoperative day 8.Histopathological features were consistent with a combination of aggregates and nests of neuronal elements, that were comprised in a background of neuropil containing benign glial elements, set in a reticulin containing stroma. Ganglion cells were large with multiple processes, nuclei were large, rounded and appeared somewhat hyperchromatic, with large nucleoli, without binucleate forms, cytomegaly, pleomorphism, mitoses and necrosis. Tumor margins were well-circumscribed, without leptomeningeal infiltration. Immunostaining for glial-fibrillar acid protein and chromogranin A were strongly positive in ganglion cells, but not in neuronal cells, which were strongly positive for synaptophysin. Ki67/MIB-1 labelling involved only glial component and was 1.0% . Final dProgression of puberty was clinically restrained, testicular volume decreased from 15 mL to 10 mL and GnRH treatment was maintained, with the indication to further evaluate biochemical response. Diplopia improved, but the auditory brainstem response confirmed left central auditory tract dysfunction. Brain MRI revealed stable findings with no evidence of tumor relapse.http://www.cochrane.de/de/LeitlinienForschungsberichte (accessed on 4 May 2021). A case-based literature search of pediatric cases of CPP associated with posterior fossa tumor was performed. The main search was conducted through PubMed and MeSH Database and the terms used were “precocious puberty” AND “brain tumor” OR “posterior fossa tumor” OR “cerebellar tumor” OR “infratentorial tumor”. Only full text papers  published between 2001 and 2021 in peer-reviewed journals were included in the study. A systematic approach to the collected data and a collegial discussion between the authors led to a final version of the manuscript.We conducted a qualitative systematic review following the ENTREQ  . Five papers describing cases of pediatric patients with infratentorial tumors and CPP were included for the qualitative analysis; 162 were found not relevant and were excluded .Four out of five tumors were in the cerebellum, two medulloblastomas ,11 and tGGs are rare primary central nervous system (CNS) tumors and represent around 5% of all pediatric CNS tumors, slightly more frequent in males ,16 WithiSeveral reports indicate that brain tumors are the most common neoplastic cause of CPP. Wendt et al.  collecteMost brain tumors causing CPP are located in the sellar-suprasellar, hypothalamic and pineal regions ,12. In sIndependently from histological diagnosis, surgical resection was the cornerstone of treatment in four out of five cases and gross total resection was a positive prognostic factor. As reported by Medina et al. , Josan eCPP associated with posterior fossa tumors are rare entities. Here we describe a unique case of CPP in a patient with a posterior fossa GG. Our work highlights the importance of prompt specialist referral in all cases of PP, regardless of clinical neurological signs or symptoms, in order to detect possible tumor association and guide management by a multidisciplinary approach.


Uterine surgery is a common predisposing factor for uterine rupture, while an invasive mole that leads to uterine rupture is a rare clinical occurrence. Here, we report a case of a 31-year-old childless woman who underwent abortion after 53 days of pregnancy. She still experienced abdominal pain and scanty vaginal bleeding after the abortion. Her levels of human chorionic gonadotropin (HCG) were high, while ultrasound and MRI results revealed an enlarged uterus and a mass in the myometrium. During preparation for treatment, the gynecologist ruptured the uterus of the patient, leaving her shocked. Eventually the patient's uterus was removed the uterus and pathologically diagnosed as result is the an invasive mole. An invasive mole refers to a common manifestation of gestational trophoblastic neoplasia (GTN) that originates from hydatidiform. In the uterine myometrium, invasive moles can grow in the uterine muscular wall and cause massive bleeding in the abdominal cavity . Previou3, with evidence of a lesion (measuring 12 × 8 cm2) in the myometrium  63 times/min. Physical examination revealed that all her abdominal muscles were tense and tender, and this was accompanied by rebound tenderness, albeit with no vaginal bleeding. We suspected uterine rupture and immediately subjected the patient to surgical treatment. Intraoperative exploration revealed that the anterior uterine wall had ruptured about 8 cm, and there was evidence of botryoid tissue on the surface. Estimated blood loss was 2,600 ml . UterineAll invasive moles originate from a hydatidiform mole, and most of them occur within a half year following mole removal. During this period, patients may experience irregular vaginal bleeding and exhibit high HCG levels in their serum. Approximately 15% of all invasive moles metastasize to the lungs or vagina . ChemothIn this case, the patient still experienced irregular vaginal bleeding 53 days after the abortion. Her serum HCG was higher than 10,000 mIU/ml, and images from b-ultrasound and MR examination revealed a mass that invaded the myometrium, but with no distant metastasis. According to the clinical features, the patient was diagnosed as GTN. High-risk GTN, which is defined as stages II–III   of diseaHowever, uterine lesion resection combined with subsequent chemotherapy for young women with fertility requirements may be a feasible and safe therapeutic strategy , especiaThrough this case, we believe resection of lesion is feasible in management of GTN. In this case, the lesion is too large in the myometrium and invaded the serosa of the uterus. When to choose lesion resection and whether to remove a lesion is the most serious difficulty. Even if chemotherapy is performed first, uterine rupture is likely to occur during chemotherapy. If surgery is performed, the lesion may not be removed entirely. Again, the case was systematically reviewed. The patient had fertility requirements and no distant metastasis. We should try to remove the lesion, and the n chemotherapy after operation. If there are still lesions that cannot be eliminated by chemotherapy, second lesion resection can be considered. In the treatment of GTN, we should consider individualized treatment.For this case, we recommended the following: (1) After abortion, specimens for pathological examination should be collected, and serum hC G levels monitored until normal and (2) we should be more involved in chemotherapy-assisted surgical therapy for this patient with fertility requirements if the lesion invaded the myometrium.The original contributions presented in the study are included in the article/supplementary materials, further inquiries can be directed to the corresponding author.Written informed consent was obtained from the individual(s), and minor(s)' legal guardian/next of kin, for the publication of any potentially identifiable images or data included in this article.All authors listed have made a substantial, direct, and intellectual contribution to the work and approved it for publication.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.


In this study, we assessed the diagnostic yield of CEUS LI-RADS and AFs in hepatocellular carcinoma (HCC). We retrospectively included patients with risk factors for HCC and newly diagnosed focal liver lesions (FLL). All lesions have been categorized according to the CEUS LI-RADS v2017 by an experienced sonographer blinded to clinical data and to the final diagnosis. From a total of 143 patients with 191 FLL, AFs favoring HCC were observed in 19.8% cases as hypoechoic rim and in 16.7% cases as nodule-in nodule architecture. From the total of 141 HCC cases, 83.6% were correctly classified: 57.4%- LR-5 and 26.2%- LR-4. In 9.21% cases, CEUS indicated LR-M; 2.12% cases- LR-3. The LR-5 category was 96.2% predictive (PPV) of HCC. LR-5 had 60.4% sensitivity and 93.6% specificity. PPV for primitive malignancy (LR-4 + LR-5) was 95.7%, with 88% sensitivity, 89.3% specificity and 88.4% accuracy for HCC. LR-4 category had 94.8% PPV and 26.2% sensitivity. CEUS LR4 + LR5 had 81,8% sensitivity for HCCs over 2 cm and 78.57% sensitivity for smaller HCCs. CEUS LR-5 remains an excellent diagnostic tool for HCC, despite the size of the lesion. The use of AFs might improve the overarching goal of LR-5 + LR-4 diagnosis of high specificity for HCC and exclusion of non-HCC malignancy.Clinical utility of ancillary features (AFs) in contrast-enhanced ultrasound (CEUS) Liver Imaging Reporting and Data System (LI-RADS Liver tumors are a heterogeneous pathology with multiple variables, which are not uniform globally, making it difficult to establish a system of accurate diagnosis and prognosis.Hepatocellular carcinoma (HCC) is among the first malignancies in terms of grim prognosis, being one of the neoplasms with a growing incidence . HCC devTherefore, the optimization of minimally invasive, imaging and laboratory methods, which provide better tumor characterization, improve early diagnosis and prognosis of patients, is necessary. However, there is currently no single method to meet these conditions, despite multiple studies suggesting various combinations of serum biomarkers and imaging tests.Contrast-enhanced ultrasound (CEUS), as a non-invasive imaging technique of describing focal liver lesions (FLL) according to features of their microvascularization, has proven to be an accurate diagnostic technique lately, comparable to contrast-enhanced magnetic resonance imaging (MRI), but faster and with a better safety profile . The conCEUS has dramatically increased the capability of ultrasonography for the detection of FLL and has the potential to be incorporated into the diagnostic algorithm for malignant FLL .Unfortunately, controversies such as the differential diagnosis of HCC and ICC with CEUS still exist . Another®) represents the most comprehensive system for imaging diagnosis, providing guidance on all imaging-related aspects of HCC, from technique for acquisition, reporting, assessment of treatment response and management ) [All lesions have been categorized according to the CEUS LI-RADS described by The American College of Radiology scheme (Contrast-Enhanced Ultrasound Liver Imaging Reporting and Data System [LI-RADS®]) . The intThe AFs were taken into account. CEUS AFs in favor of benignity were size reduction or stability >2 years of the tumor. The malignancy aspects were: definite growth, nodulein nodule architecture and mosaic architecture, which are patterns that favor HCC in particular. The evidence of one or more AFs in favor of benignity downgraded by 1 category the LI-RADS score, while one or more AFs of malignancy upgraded by 1 category up to CEUS LR-4.Lesions suggesting a cyst/hemangioma or hepatic fat deposition/sparing were classified as LR-1. Distinct isoenhancing solid nodule <10 mm standed for LR-2 group. LR-3 category included nodules with iso- or hypoenhancement in the arterial phase without late washout, regardless of their size or, in the instance of the occurrence of mild and late washout, only if <20 mm in size. LR-4 category also included lesions ≥10 mm with arterial phase hyperenhancement , but without any washout. LR-5 category comprised nodules ≥10 mm with arterial phase hyperenhancement  followed by washout appearance that was mild in degree and late in onset. If the lesion could not be categorized due to image degradation or omission, it was classified as LR-NC.Histopathological assessment was required in patients with unclear imaging diagnosis or with inconclusive findings at CEUS, CT and MRI imaging. It was performed either on a tissue sample of the lesion obtained by surgical resection or percutaneous US-guided biopsy whenever it was possible. The biopsy sample was obtained by using an 18-gauge core needle with a 16 mm or 22 mm throw .The IBM program- Statistical Analysis Software Package (SPSS) version 20 was used for data processing and descriptive analysis. The program was also used to calculate the diagnostic performance of CEUS rendered by the following parameters: sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), likelihood ratio and accuracy.This retrospective single-centre study included 143 patients with a total of 191 liver nodules examined from a total of 823 consecutive patients with FLL detected by standard abdominal ultrasound. The flow chart of the patients’ enrollment is shown in The presence of AFs favoring HCC was observed in 38 patients (19.8%) with a hypoechoic rim and in 32 patients (16.7%) with nodule-in-nodule architecture, respectively .From a total of 191 lesions, the malignancy rate was 83%, representing 159 lesions. The distribution of diagnosis was as follows: 73% HCC (141 patients), 2% intrahepatic cholangiocarcinoma , 1% mixed tumor HCC/ICC (3 patients), 5% metastases from other primary sites and one malignant transformation of a hepatocellular adenoma (0.5%).The majority of HCCs  were greater than 2 cm in size, while only 14 HCCs (7.3%) were smaller than 2 cm from all nodules .Furthermore, 32 benign tumors were diagnosed (16%): 19 regenerative liver nodules (9.9%), one focal nodular hyperplasia—FNH (0.5%), 6 liver hemangioma (3.1%) and 6 complex cysts (3.1%), respectively.Imaging features of all liver lesions on ultrasound are listed in HCC cases, 118 of them (83.6%) were correctly classified as HCC diagnosis, as follows: 81 tumors (57.4%)—definitely HCC (LR-5) and 37 tumors (26.2%)—probably HCC (LR-4). In 13 cases (9.21%), CEUS characteristics indicated malignant lesions, but not necessarily HCC (LR-M). Only 3 cases (2.12%) were classified as intermediate probability of malignancy (LR-3) and none of them was incorrectly diagnosed as benign  were classified as not categorizable (LR-NC) because of the image degradation and, consequently, they were excluded from the The final diagnosis and the rates of different cellular types of nodules according to LI-RADS classes are listed in None of LR-1 (definitely benign) or LR-2 (probably benign) lesions  were fin84 nodules (43.9%) were established as LR-5 and 81 of them (96.4%) were accurately diagnosed as HCC . The othThere were 37 lesions from 39 cases (94.8%) classified as probably HCC (LR-4) that were finally diagnosed as HCC .There were 29 lesions classified by using CEUS as probably or definitely malignant but not HCC specific (LR-M); 13 of them (44.8%) were HCC, 3 of them (10.3%) were ICC, 3 (10.3%) mixed tumor HCC/ICC and 10 (34.4%) metastases .Sensitivity, specificity and accuracy for LI-RADS categories and overall diagnostic accuracy of LI-RADS patterns for HCC are reported in Individually taken, the LR-5 category was 96.2% (95% CI: 89.4–98.7%) predictive (PPV) of HCC, with one case of misdiagnosis for cholangiocarcinoma. CEUS LR-5 sensitivity for HCC was 60.4% and specificity was 93.6%.PPV for primitive malignancy (LR-4 + LR-5) was 95.7% (95 CI%: 90.7–98%), 88% sensitivity, 89.3% specificity and 88.4% accuracy for HCC (95CI%: 82.8–92.6%).Regarding the LR-4 category, PPV was 94.8%, with only 26.2% sensitivity, whereas for LR-3 the PPV was 41.02%, with only 2% sensitivity.CEUS LI-RADS L4 + L5 had 81.8% sensitivity for HCCs over 2 cm (n = 127), and 78.57% sensitivity for small HCCs less than 2 cm (n = 14).Histological diagnosis was available in 48 (25%) lesions. Thus, of the 36 cases of HCC, CEUS correctly classified 27 cases (81.8%) as probably (LR-4) or definitively HCC (LR-5). All 3 cases of ICC, 3 cases of mixed tumor (HCC/ICC) and 4 biopsied metastases were correctly defined by CEUS as lesions probably or definitely malignant but not specific HCC (LR-M).To date, CEUS is considered to be a second-line imaging technique in the characterization of FLL, having the advantage of a low cost. Beyond contrast-enhanced MRI as the method of choice for characterizing FLL, there are studies concluding that there are no statistically significant differences between CEUS and MRI or CT ,13,14. HStudies focused on AFs in MRI and there is little evidence on their use in CEUS or on the differences between the use of different contrast agents. To our knowledge, this is the first blind evaluation of CEUS performance by highly experienced in abdominal ultrasound and CEUS by using the latest LI-RADS algorithms and AFs.This study highlights that the CEUS LR-5 pattern by using this new LI-RADS algorithm remains an excellent diagnostic tool for HCC. Sensitivity and specificity for diagnosing HCC of CEUS LR-5  were quite similar to the estimates of CT / MRI LR-5 (sensitivity 67% and specificity 93%) . As for Nevertheless, Schellhaas et al. suggested the combination of the categories LR-4 and LR-5 for the diagnosis of definite HCC to improve the CEUS LI-RADS v2017 algorithm . TherefoInterestingly, higher sensitivity than estimated for the diagnosis of HCCs smaller than 2 cm was achieved for CEUS LR-4 and LR-5 (78.5%), which was similar to CEUS sensitivity for HCCs over 2 cm (78.9%) in another study . TherefoPPV for LR-3 category for nodules at intermediate risk of HCC was 41.02%, which was close to 50% considered as the middle risk and to Terzi et al.’s results (47%), but with a lower sensitivity of only 2% . Anyway,It is still controversial whether CEUS can make a specific diagnosis of HCC because of the potential risk of misdiagnosis in the case of ICC which manifest global arterial phase hyperenhancement followed by washout at CEUS, leading to a misdiagnosis of HCC in approximately 50% of the cases ,21,22. TAll LR-M from our cohort were confirmed as malignancies, therefore with better accuracy than that of the CT/MRI LR-M (93%) category reported in a previous meta-analysis . HCCs reTo date, there are no subgroup analyses based on major and ancillary features of the CEUS LI-RADS because of insufficient data .From all the benign tumors that were diagnosed correctly, only one lesion was a focal nodular hyperplasia, FNH (0.5%), in a female patient, 65 years old, with alcoholic liver cirrhosis. The appearance of the nodule was characteristic, filling with contrast medium from the center to the periphery, originating from a central arterial blood supply and we graded it as LR-2. FNH on liver cirrhosis is rarely reported. Despite the fact that this aspect could be a feature in favor of benignity, it is not recognized as a LI-RADS AF yet and it is considered of little value in daily practice, except for patients who are at risk for HCC due to hepatitis B without cirrhosis ,31.Therefore, the accuracy of blinded evaluation of CEUS by taking into consideration AFs was similar to other studies where prior to performing a liver CEUS study, the patient’s clinical history, laboratory data and any imaging findings were reviewed. Also, the sensitivity was not influenced by the size of the nodules.The use of tumor size reduction as an AF in favour of benignity is questionable, as diameter reduction could happen secondary to the resorption of haemorrhage or the development of fibrosis . Size chOne limitation of this study regarding the assessment of AFs could be the fact that CEUS was perfomed by using only one contrast agent (SonoVue), while other agents could provide additional Kupffer phase images ,28. FutuIn conclusion, CEUS could be accepted by unanimous consent in international guidelines as a useful non-invasive tool with high accuracy to rapidly orientate liver tumor diagnosis, especially in high-risk patients, whenever it is available and performed by experienced sonographers. Given that CEUS LI-RADS and CEUS AFs are newly introduced concepts, more prospective multicenter cohort studies which assess the usefulness of this algorithm will bring important information for possible improvements in the future.


Candida infective endocarditis (CIE) is a feared complication of candidemia, associated with high mortality rates. It requires prolonged hospital stays for medical and, often, surgical management. We report a case of a massive intracardiac Candida mass in an adult with native valve CIE.  Coupled with the increasing use of indwelling vascular catheters and prosthetic cardiac valves is an uptrend in sepsis secondary to fungemia. An insidious onset often shrouds the initial diagnosis, contributing to poor outcomes. Candida glabrata (C. glabrata) and Candida dubliniensis (C. dubliniensis). Transthoracic (TTE) and transesophageal echocardiography revealed a large mobile right atrial mass (4 cm × 6 cm × 2.5 cm), extending to the right ventricular outflow tract. Since he was a poor surgical candidate, management with micafungin was initiated and continued for 8 weeks. He responded well to the regimen with resolution of the fungal mass on follow-up TTE 3 months later. In anticipation of the future need for TPN, he continues on lifelong suppressive oral fluconazole.  A 51-year-old male on chronic total parenteral nutrition (TPN) because of bowel resection presented with fevers, night sweats, and unintentional weight loss. He was febrile and tachycardiac on admission, with a benign physical examination. Laboratory workup showed elevated inflammatory markers and an acute kidney injury. Extended blood cultures showed growth of  CIE may be an insidious complication of indwelling central venous catheters, necessitating a high index of suspicion. Conservative management, with antifungal therapy, can yield favorable outcomes in poor surgical candidates. Candida infective endocarditis (CIE) is a dreaded complication of candidemia and is associated with exceptionally high mortality rates, ranging from 30–80% [Candida species causing over half of all fungal endocarditis cases [Candida masses has been reported in the neonatal population but remains a rare occurrence in adults with native valves [Candida mass in a patient with native valves as a complication of total parenteral nutrition (TPN), with a favorable outcome after conservative medical management.m 30–80% , 2. A riis cases , 3. Indwis cases , 2. CIE e valves . There i3, elevated ESR: 99 mm/hr, CRP: 139 mg/L, and creatinine: 3.56 mg/dL (baseline: 0.9 mg/dL). Urinalysis and chest X-ray were unremarkable for infection, and standard blood cultures displayed no growth of microorganisms. Electrocardiography (EKG) showed normal sinus rhythm without any conduction system abnormalities. Broad-spectrum antibacterial coverage with vancomycin and piperacillin-tazobactam was initiated, while micafungin was added with suspicion of fungemia given the history of TPN. Extended blood cultures from admission showed growth of Candida glabrata (C. glabrata) and Candida dubliniensis (C. dubliniensis); both species were sensitive to amphotericin-B (minimum inhibitory concentration: 0.2 mcg/ml) and micafungin (minimum inhibitory concentration: 0.12 mcg/ml). The PICC was subsequently removed; cultures from the catheter tip also grew C. glabrata and C. dubliniensis. A transthoracic echocardiogram (TTE) showed an echogenic large mobile right atrial mass (4 cm × 6 cm × 2.5 cm), extending from the superior vena cava into the right atrium and prolapsing into the right ventricle and right ventricular outflow tract. There was no evidence of outflow tract obstruction. These findings were concerning for an intracardiac fungal mass versus a large thrombus. Transesophageal echocardiogram (TEE) revealed similar lesions, with an additional aortic valve vegetation (1 cm × 0.5 cm) , tachycardiac (HR 106 bpm), but hemodynamically stable (BP: 130/86 mm Hg) and saturating well (96%) on room air. Physical examination revealed no erythema or tenderness at the site of his peripherally inserted central catheter (PICC), through which he had been receiving TPN. No significant cardiac murmurs or sequelae of infective endocarditis were observed, and the remainder of physical examination was benign as well. Admission laboratory workup revealed a white cell count of 12,000/mmFigures 1.The increasing use of indwelling vascular catheters, intracardiac devices, and prosthetic cardiac valves has contributed to an upsurge of candidemia and CIE, countered to an extent by the approval of echinocandins in 2003, which are now recommended as first-line agents . Most caCandida CIE.Cardiac involvement may result in worsening or new-onset congestive heart failure due to outflow tract obstruction or valvular compromise, depending on the size or site of the mass. Other complications involve paravalvular leaks, intracardiac abscesses, and septic embolization; the latter may result in endophthalmitis, cerebrovascular accidents, osteomyelitis, etc. Fungal vegetations tend to be larger in size and carry an increased risk of embolization compared to bacterial vegetations . IntracaCandida mass in our case and inability to undergo surgical management, antifungal medications yielded a favorable outcome.Available data for the treatment of fungal endocarditis, particularly involving intracardiac fungal masses, is based primarily on case reports, case series, and observational studies. The recommended practice per the Infectious Diseases Society of America involves adjunctive surgery wherever possible to achieve source control along with an appropriate antifungal regimen . Recent Initial treatment regimens consist of parenteral amphotericin-B or an echinocandin. Animal models and prospective studies have shown both regimens to be equally effective , 9. PatiThe diagnosis of CIE requires a high degree of clinical suspicion owing to its insidious onset and high possibility of initial misdiagnosis. In poor surgical candidates, a prolonged course of antifungals followed by chronic suppressive therapy with fluconazole can yield favorable outcomes, even without surgical management. Clinicians must remain vigilant against drug resistance as a possible drawback of chronic antifungal therapy.


Anorectal melanoma (ARM) is a rare, aggressive disease. Given that it presents with local symptoms that resemble other common benign anorectal conditions, ARM is often low on the differential diagnosis. Delayed diagnosis and nonconsensus of treatment options lead to poor prognosis. Here, we report the case of an 85-year-old woman with a history of Irritable bowel syndrome who presented with altered bowel habits and bleeding per rectum. CT revealed a rectal mass with metastatic lesions to the bone, liver, and lungs. Immunohistochemical staining was positive for Human Melanoma Black-45, melanoma antigen recognized by T cells, and SRY-related HMG-box 10. A final diagnosis of ARM was made. Lower GI bleeding is a common presentation in the emergency department and clinic settings. Common etiologies are often grouped into benign conditions  or malignant causes . However, for a broad differential diagnosis, other less common etiologies may be considered including uncommon malignancies , infiltrative pathologies (amyloidosis), and vascular abnormalities (Dieulafoy’s lesions).Anorectal melanoma (ARM) is a rare disorder, with a 0.5-4% incidence rate of all malignant tumors in the region ,2. MucosAn 85-year-old woman presented to the outpatient clinic with bright red blood per rectum that occurred with and without bowel movements. She reported having intermittent rectal bleeding for a month that became persistent. In addition, she complained of diarrhea alternating with constipation for several months with varying frequency, along with a feeling of pressure in the pelvis with defecation. Physical examination was unremarkable. A computed tomography (CT) scan of the chest/abdomen/pelvis revealed a distal rectal mass, with severe circumferential mucosal thickening extending over 8 cm and multiple enlarged lymph nodes in a pattern highly suggestive of rectal carcinoma. Metastatic lesions in the liver, lungs, and bone were detected. Colonoscopy showed a mass with erythematous, congested, and friable mucosa that appeared fungating Figure . It partMelanomas originate from melanocytic cells which can be found throughout the body, most commonly in the skin. In the rectum, these cells are located around the dentate line, and when malignant, they spread submucosally. Diagnosis of ARM can be challenging as it can resemble other adenocarcinomatous malignancies on histopathology . MoreoveTreatment of ARM is still debatable because of the rarity of the condition and the few studies comparing different modalities of treatment. Surgery, chemotherapy, and radiotherapy have been implicated. While surgery remains the mainstay of treatment, which varies from wide local excision to abdominoperineal resection, the therapeutic benefit of these procedures remains questionable ,18. UnfoThe high vascularity of the anus contributes to the aggressive nature of ARM, therefore, most patients present at an advanced stage. Presentation is often nonspecific and varies from common symptoms such as altered bowel movement to red flags such as anorectal mass and rectal bleeding, which makes the diagnosis challenging. We encourage physicians to widen their differential diagnoses and have a lower threshold for malignant etiologies in high-risk individuals, particularly in individuals with previously stable or mild IBS who present with new GI symptoms. There is no consensus regarding the treatment plan for ARM. Surgery, chemotherapy, and radiotherapy are treatment options. Further studies are recommended to decrease both the morbidity and mortality rates and achieve better care for patients.


Epithelioid angiosarcoma arising in schwannoma is an extremely rare mesenchymal tumor that accounts for only 1 to 2% of all sarcomas. This type of tumor occurs in all parts of the body, most often in the skin and soft tissues and rarely in the mediastinum. The present study describes the case of an asymptomatic, 58-year-old male who presented with epithelioid angiosarcoma in the posterior mediastinum during a physical examination. Enhanced computed tomography of the chest revealed a 3.5 × 3.1-cm mass in the posterior mediastinum. Thoracoscopic mediastinal mass resection was performed under general anesthesia due to the possibility that the tumor was malignant. Pathological examination revealed the presence of angiosarcoma and schwannoma components. Immunohistochemical staining for cluster of differentiation (CD) 31, CD34, early growth response (EGR), vimentin, Sry-related HMG box (SOX)-10 and S-100 was strongly positive. The patient recovered and was discharged on postoperative day 5. Two months postsurgery, the patient returned for evaluation, and no evidence of tumor recurrence was observed. Epithelioid angiosarcoma arising in schwannoma has been described in a variety of anatomic locations, including the vagus nerve, sciatic nerve, and the adrenal gland, which was initially described by Trassard . The majA 58-year-old, asymptomatic male presented to our hospital  for a physical examination. Written informed consent was obtained from the patient for the publication of the present study. The patient had no history of cigarette smoking, hypertensive disease, diabetes mellitus, hepatitis or tuberculosis. Routine blood, liver function, renal function, blood glucose, coagulation function and serum electrolyte test results were all within the normal range. Enhanced computed tomography (CT)  of the chest showed a 3.5 × 3.1-cm mass in the posterior mediastinum, and no other lesions were identified in the thoracic cavity .A contrast-enhanced CT scan showed an enhanced mass with homogeneous internal density and clear tumor boundaries. A preoperative diagnosis of schwannoma was considered due to the enhanced CT features. Right-side video-assisted thoracoscopic surgery (VATS) was performed under general anesthesia due to the possibility that the mass was malignant. The 4th intercostal incision in the right axillary front was ~3 cm wide and was used as the operating hole; the 7th intercostal incision in the right midaxillary line was ~2 cm wide and was used as the observation hole. Intraoperatively, the tumor had clear boundaries, no invasion of surrounding tissues, and slight toughness. There was no evidence of intraspinal extension of the tumor. The boundary of the tumor was carefully separated with an ultrasonic knife, and the tumor was completely excised. The operation time was 20 min, and the bleeding volume was 20 ml. A chest tube was placed and removed 2 days after the operation. The surgical margins of resection were microscopically negative. Pathological examination revealed the presence of angiosarcoma  and schwRelevant studies and literature regarding angiosarcoma arising in a schwannoma were searched in databases (PubMed and Web of Science) from January 1980 to January 2020. The text words and MeSH terms “angiosarcoma” and “schwannoma” were used. Tumor characteristics, clinical features, therapeutic strategies and survival outcomes were reviewed, and these data were tabulated. Fifteen articles were identified from database searches, and a total of 21 patients were included  Table 1Table 1. Schwannoma is a benign peripheral nerve sheath tumor that occurs in patients of any age with a slight predilection for adults . It is wThe patients' symptoms are complex and depend on the location and size of the tumor. When the tumor is small, it is often asymptomatic and is detected by physical examination. When the tumor grows, it will compress or invade surrounding tissues, causing pain and difficulty breathing. X-ray and B-ultrasound have no obvious advantage for the early detection of this tumor. CT scans of the chest can detect these tumors early, but the pathology cannot be determined. Whether PET-CT is applicable for such diseases is unclear, and further studies are needed. Benign lesions were considered before surgery in this patient, and PET-CT examination was not performed.The differential diagnosis of these extremely rare cases includes numerous malignant and benign tumors, including epithelioid malignant peripheral nerve sheath tumors, fibromas, neurofibromas, and hemangiomas. Immunohistochemistry is helpful for differentiating between these tumors. For example, pathological examination revealed the presence of angiosarcoma and schwannoma components. The angiosarcoma cells CD34-, CD31- and EGR-positive, and the schwannoma cells were SOX-10- and S-100-positive.Surgery is still the preferred treatment for mediastinal tumors, whether they are benign or malignant. Capsular penetration of the tumor or invasion of adjacent tissues are poor prognostic factors because they make complete surgical resection more difficult, if not impossible . There iIn conclusion, the present study described a rare case of epithelioid angiosarcoma arising in a schwannoma in a male patient. This study revealed four important findings. First, epithelioid angiosarcoma arising in a schwannoma can occur in people of all ages and with no sex predominance. The male-to-female ratio was 1.0:0.5. Second, complete surgical resection presents a safe and effective treatment. Third, preoperative and postoperative chemoradiotherapies are still controversial. Fourth, to the possibility of malignant transformation during the long-term follow-up of patients with schwannoma.The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author/s.YX and LY drafted the article. XL, FZ, and XZ performed the surgery. ZW made critical revisions for important intellectual content. All authors read and approved the final article.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.


Case Report. A 67-year-old woman presented to the emergency room with two brief episodes of dizziness and diplopia for 2 minutes. She had a medical history of provoked upper extremity DVT after a fracture, hypothyroidism, hyperlipidemia, and soft tissue sarcoma. The sarcoma was initially diagnosed in, and subsequently resected from, the right triceps muscle. During posttreatment surveillance, a second lesion was discovered in the left upper pulmonary lobe, and this was also completely resected 9 months following initial diagnosis. We present a case of a woman with a tertiary (cardiac) site sarcoma that presented with embolic stroke.  Cancer and ischemic stroke are associated with significant morbidity and mortality. Hypercoagulability, disseminated intravascular coagulation, venous-to-arterial embolism, and non-bacterial thrombotic endocarditis are among recognized mechanisms. Emboli to the brain, or to other organs, are known to occur as a consequence of liberated thrombotic debris originating from the thrombogenic surface of intracardiac neoplastic entities. The most common primary malignancy of the heart is sarcoma; however, masses that occur in the heart are 20 to 40 times more likely as a consequence of metastasis from other sites.  Our case highlights the benefits of multimodality imaging, heart-team approach with oncology support to define anatomy, thereby enable surgical treatment, of a complex intracardiac lesion. Cardioembolic debris is a well-established source of ischemic stroke that has a robust clinical presence in everyday hospital practice. Of those presenting with ischemic stroke, a cardioembolic source is implicated in 15-20% of the cases , and theCancer and ischemic stroke are associated with significant morbidity and mortality . HypercoA 67-year-old healthy appearing woman experienced dizziness and 2 episodes of brief diplopia leading to an acute inpatient evaluation. The patient had a history of provoked upper extremity deep vein thrombosis (DVT) after a fracture, hypothyroidism, hyperlipidemia, and soft tissue sarcoma of the right triceps. Physical exam, including gross neurologic function, was normal at time of presentation. Laboratory analysis was normal. Magnetic Resonance Imaging (MRI) of the brain demonstrated cerebral hemispheric and cerebellar foci which were felt to represent embolic lesions.18 months prior to presentation, the patient had undergone complete resection of a sarcoma that had arisen in the right triceps muscle. 9 months after the initial resection of the triceps sarcoma, surveillance imaging revealed a newly developed left upper lobe pulmonary mass. This lung lesion was completely resected and was a metastatic sarcoma. 9 months after the pulmonary resection, the patient presented to our institution with two episodes of acute neurologic change manifested as two brief episodes of dizziness and diplopia for 2 minutes. Subsequent imaging of the brain demonstrated two foci of acute or subacute ischemia in the superior right frontal lobe and left cerebellum, reflective of embolic lesions. Peripheral vascular imaging with carotid ultrasound did not demonstrate vascular abnormalities. Transthoracic echocardiography (TTE) revealed a large mobile mass based of the interventricular septum within the left ventricular cavity . TransesThis case posed multiple diagnostic challenges that were relevant to, and ultimately defined her treatment options. Our patient presented with symptoms of a neurological event which prompted brain imaging. Cardiac tumors can be asymptomatic . Our patTumors occurring on the left side of the heart can produce symptoms due to direct contact with the systemic arterial circulation. Neoplastic or thrombotic emboli from the tumor nidus can lead to stroke or another systemic organ damage .The driving impetus in the management of our patient was the prevention of further cerebrovascular embolic event. Given the relatively brief disease-free interval from her sarcoma diagnoses, as well as an initial surface echocardiographic finding suggestive of a large sessile left ventricular-based intracardiac mass, the initial clinical impression was to offer the patient a chemo-radiotherapy treatment option. Confidence in the ability to offer resection of the mass only occurred after further anatomical characterization of the lesion with transesophageal echocardiography and cardiac MRI. Ultimately, intraoperative analysis of the anatomical extent of the lesion mirrored our estimations from imaging, and a complete resection with negative margins was achieved. This treatment modality, in contrast to the nonsurgical treatment options that were proposed prior to anatomical delineation of the mass, offered removal of the cardioembolic nidus and the hope of completely resecting the malignancy.For patients presenting with echogenic structures located inside the heart, multimodality imaging is often required. Initial TTE is useful; however, TEE is frequently required for comprehensive and accurate assessment . The mulOur case represents a rare tumor with an unusual course with three different sites involved over the clinical course of many months. It highlights the importance of multimodality imaging, a heart team approach, and oncology support to delineate a successful surgical resection and an optimal clinical result.