Giant paratesticular liposarcoma is a rare presentation of paratesticular tumor. We present a case of the largest paratesticular liposarcoma described to date with a weight of 4,100 g and measuring 460 × 210 × 130 mm. It was initially mistaken as an inguinoscrotal hernia until a contrast-enhanced computed tomography (CECT) scan of the abdomen and pelvis revealed a huge left paratesticular tumor extending from the scrotum to the mid-abdomen. The challenge was to achieve a tumor-free margin orchidectomy due to the poor fat plane of the tumor to the external iliac artery, psoas muscle, descending colon, and anterior abdominal wall. The surgery was started with laparoscopic dissection for the intraabdominal part of tumor from the vital structure, then followed by inguinal radical orchidectomy and inguinal mesh repair. Postoperative histopathological report revealed a paratesticular dedifferentiated liposarcoma with rhabdomyosarcomatous differentiation with clear margin. The patient had good recovery post operation. Paratesticular liposarcoma is a rare malignant tumor originating from mesoderm. There are less than 200 cases reported in the English literature to date –3, with This is a case of a 73-year-old man who presented to the urology clinic with left inguinoscrotal swelling for 3 years. The swelling originated in the scrotum and gradually increased in size, ascending towards the inguinal region. It was not accompanied by any pain. He did not have any other constitutional symptoms and no family history with malignancy. It was initially diagnosed as hernia by the general practitioner and the patient did not seek further intervention until the tumor had grown into a large mass and significantly impacting his quality of life.-fetoprotein (AFP) was over 2.74 ng/ml, while serum lactate dehydrogenase (LDH) was 224 IU/L. The provisional diagnosis was left paratesticular tumor.On clinical examination, there was a huge inguinal scrotum tumor that was palpable up to the umbilical level, measuring approximately 50 × 20 × 10 cm Figure 1via inguinal incision to complete the tumor mobilization from the scrotum and delivered the tumor en bloc through inguinal incision  in addition to a background of classic well-differentiated liposarcoma. The high-grade sarcomatous component was cellular, demonstrating pleomorphic spindle cells with hyperchromatic nuclei and inconspicuous nucleoli; in areas, small round malignant cells were seen. Focal rhabdomyosarcomatous differentiation accounting for approximately 20% of the tumor was also observed Figure 5Given the aggressive nature of this tumor, the patient was advised to undergo adjuvant chemotherapy. However, because of the patient’s advanced age and concerns regarding the potential side effects of chemotherapy, they firmly declined this treatment option.At the 3-month post-operation follow-up, the patient underwent CECT imaging of the thorax, abdomen, and pelvis for surveillance purposes. During the clinic visit, the patient expressed concern about bilateral inguinal masses and swelling in the right lower limb. Unfortunately, the CECT scan revealed a recurrence of a tumor in the left scrotal wall, as well as metastases to the bilateral inguinal and abdominopelvic lymph nodes and bilateral lungs. Additionally, an ultrasound Doppler examination detected a deep vein thrombosis (DVT) in the right lower limb. To address the DVT, the patient was prescribed oral rivaroxaban at a dosage of 15 mg twice daily. After extensive discussion, the patient agreed to undergo chemotherapy and is currently receiving single-agent doxorubicin treatment for the metastatic dedifferentiated paratesticular liposarcoma Figure 6Liposarcoma is a type of soft tissue malignancy that arises embryologically from mesodermal tissue. It is the most common histopathological type of sarcoma 46%); the second most common is leiomyosarcoma 20%), followed by histiocytoma 13%) and rhabdomyosarcoma 9%). As a type of sarcoma, it can be presented at the head and neck region, extremities, and retroperitoneum. Seventy percent of the sarcomas arise from the extremities and retroperitoneum. Paratesticular liposarcoma, however, is a very uncommon condition with only less than 200 cases reported in the literature  can lead to a more specific diagnosis. Both CECT scan and MRI are the best imaging studies to detect any compression or invasion to the nearby organs. MRI, however, remains the gold standard investigation method for soft tissue tumor as it helps to characterize the lesion and delineate the degree of the local tumor invasion and also the tumor stage . In our There is no “one size fits all” approach to the treatment of liposarcoma, particularly retroperitoneal sarcoma. Surgical resection has traditionally been the only potentially curative approach. It is best to achieve resection with microscopically negative margins (R0 resection) though it can be challenging as most of the time the tumor tends to be huge. The role of radiation therapy and chemotherapy, either given preoperatively or postoperatively, continues to be debated, and there is no consensus as to the best approach for all patients.Radical orchidectomy and high inguinal spermatic cord excision remained the gold standard of treatment , 12. We The role of preoperative radiotherapy is controversial. There is no evidence-based guideline on the tumor size to consider neoadjuvant radiotherapy. Some institutions offer radiotherapy to large well-differentiated liposarcoma. Some argue that patients tend to die from local recurrence and not from distant metastasis unless in a dedifferentiated or high-grade tumor, which was shown in the randomized phase III STRASS trial . PreoperFollowing resection of the retroperitoneal sarcoma without neoadjuvant therapy, adjuvant radiotherapy was not offered for a low-grade, completely resected (margin-negative) tumor. However, sometimes postoperative RT could be considered for patients with R0/R1 resected high- or intermediate-grade tumors that are at risk for local recurrence. In this case, we decided to just observe because it is not possible to deliver postoperative adjuvant radiotherapy to such a huge area with acceptable morbidity.The benefit of adjuvant chemotherapy following surgical resection of a soft tissue sarcoma is controversial. An updated meta-analysis suggested that the use of an optimal adequately dosed anthracycline/ifosfamide-containing regimen significantly prolongs survival, but the analysis did not include the two largest trials, both conducted in Europe and both testing the value of an anthracycline- and ifosfamide-containing regimen . A poolevia a laparoscopic-assisted approach for the largest paratesticular liposarcoma described to date. The giant paratesticular liposarcoma is a rare condition and often misdiagnosed as inguinoscrotal hernia or other benign scrotal swelling, resulting in a delay in diagnosis. Margin-negative excision remains the standard of treatment.We highlight a challenging case of radical orchidectomy with high inguinal spermatic cord excision The original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding author.Ethical review and approval was not required for the study on human participants in accordance with the local legislation and institutional requirements. The patients/participants provided their written informed consent to participate in this study. Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article. Written informed consent was obtained from the participant/patient(s) for the publication of this case report.MA prepared the draft. XF conceptualized the article and was the primary surgeon. XF and IR edited and finalized the draft. YW, IR, and KS prepared the images, photos, and captions. All authors contributed to the article and approved the submitted version.


Primary pulmonary artery tumors (PPATs), originating from the pulmonary artery intima, are rare tumors characterized by pulmonary artery luminal occlusion and pulmonary hypertension. Diagnosis of this rare entity is a challenging dilemma with the need for a high expertise in the radiological and pathological identification of PPATs. computed tomographic pulmonary angiography of PPATs may show filling defects, which are easily misdiagnosed. The radionuclide scan, along with other imaging examinations, can assist with the diagnosis, but the pathological diagnosis requires a puncture or surgical resection. Most primary pulmonary artery tumors are malignant, with poor prognosis and lack of specificity in clinical manifestations. However, there is no unified understanding and standard for diagnosis and treatment. In this review, we discuss the status, diagnosis, and treatment of primary pulmonary artery tumors, as well as how clinicians can better understand and treat the disease. So far, only a few hundred cases of such diseases have been reported in the literature, and most of them are individual cases, and the vast majority are malignant tumors. Pulmonary artery sarcoma (PAS) is the most common in kinds of literature, accounting for <400 cases. Other types such as primary pulmonary artery myxoma, Primary papillary fibroma, and Primary pulmonary artery chorionic carcinoma have only been rarely reported. The main imaging manifestations are irregular soft tissue masses in the lumen, which is similar to PE. As the most common primary pulmonary artery sarcoma (PPAS), the prevalence rate reported in the literature is only 0.001% to 0.030%, considering that many cases are often misdiagnosed as PE in the preoperative examination, or there are several cases without pathological examination, and other types of PPATs are mostly sporadic cases. Therefore, the actual prevalence rate of PPATs is still unclear. The average age of onset of PPATs is 48 years old (range 13–86 years old). Some studies have shown that there is no significant difference between male and female patients, and Liu MX et al also reported that most of the patients are female (2:1). The vast majority of cases occurred in adults and a few in children. The prognosis of this kind of disease is poor, and the clinical diagnosis can only be confirmed by puncture or surgical resection of biopsy tissue for pathological examination, so its early diagnosis is extremely difficult. With the increase in the prevalence rate of PE and the improvement of clinicians awareness of it, PPATs are usually misdiagnosed as PE because of their similarity in imaging and clinical manifestations with PE.,8 That may lead to inappropriate treatments for patients, such as prolonging anticoagulation time or delaying surgical treatment, which may delay the illness. However, there is no unified understanding and standard on the clinical features, diagnosis, and treatment of PPATs. In this review, we present clinical, imaging, and histopathologic features of benign and malignant PPATs, emphasizing differentiating features from PE. We also describe available diagnostic and treatment methods for PPATs. So we hope to help clinicians better understand the disease through the relevant literature.Pulmonary embolism (PE) is the most common filling defect seen on computed tomographic pulmonary angiography (CTPA). Primary pulmonary artery tumors (PPATs) can mimic PE on imaging and clinical presentation. PA tumors, especially malignant ones, have radically different treatments and a more severe prognosis from PE. PPATs are a rare disorder of undetermined etiology, originating from the main pulmonary artery or the tissue of the pulmonary valve. The first case was reported by Mandelstamm through autopsy in 1923. thought PPATs often occur in the main pulmonary artery and the pulmonary valve, which can extend to the bifurcation of the pulmonary artery and the left and right pulmonary trunks. A few can retrograde spread to the right ventricular outflow tract through the pulmonary artery lumen, or originate from the right ventricular outflow tract, but they are extremely rare. Histologically, it can be divided into benign and malignant tumors, especially malignant tumors. There are only a few primary benign tumors of the pulmonary artery, including pulmonary artery angiomyxoma, pulmonary artery myxoma, pulmonary artery lipoma, etc. PPAS is the most common of primary pulmonary artery malignancies, but because primary pulmonary artery malignancies are extremely rare, primary pulmonary artery malignancies are limited to sarcomas. It has been suggested that PPAS may originate from mesenchymal cells in the arterial intima, and have the potential for multi-directional differentiation. Therefore, PPAS contains many different pathological types. Among them, undifferentiated sarcoma was the most common type, accounting for 34% of all PAS, followed by fibrosarcoma (21%), leiomyosarcoma (20%), rhabdomyosarcoma (6%), mesenchymal histiocytoma (6%), chondrosarcoma (4%), angiosarcoma (4%), osteosarcoma (3%), and malignant fibrous histiocytoma (2%), etc. However, these tumors have common clinical characteristics, and an overall poor prognosis.Han Y et al symptoms of hemoptysis may suggest that the tumor invades the adjacent bronchi. Palpitation is the main symptom in some patients. Some patients have non-pulmonary systemic symptoms of fever and cachexia or symptoms caused by local spread, such as tachyarrhythmia, conduction defects, pericardial effusion, inflammatory anemia, and clubbed-fingers. A few patients have no obvious symptoms in the early stage, when there is almost complete occlusion near the pulmonary artery valve and tumor growth persists, symptoms may suddenly appear, possibly manifesting as severe hypotension or shock. Given the similar clinical manifestations to PE, PPATs may be diagnosed as acute PE. It has been reported that approximately 50% of patients with PPATs were initially diagnosed with acute PE and received anticoagulation therapy. When anticoagulant therapy fails to improve the patient’s condition, it may lead to further examination. It is possible that “the thrombus” continues to grow based on adequate anticoagulation. So that PPATs are finally diagnosed after inappropriate treatment for a long time. There is currently no literature indicating whether anticoagulation affects clinical course. As with all other cancers, symptoms of local infiltration and distant metastasis may exist and vary with the scope of the disease and anatomic location, the lung is the most commonly affected area, most likely caused by thrombus, regional lymph node, liver, pancreas, brain, and kidney metastases have also been reported. There may be no obvious positive signs in the patient’s physical examination. However, when the treatment is delayed due to misdiagnosis and pulmonary hypertension (PH) and right ventricular dysfunction are combined, there may appear signs as a systolic murmur in the pulmonary valve area, cyanosis, jugular vein flaring, hepatomegaly, and edema in both lower limbs. In contrast, clinical manifestations of primary pulmonary artery benign tumors have no obvious specificity, and symptoms of dyspnea, chronic recurrent cough, and hemoptysis have been reported, which are not obviously different from symptoms of malignant tumors in general. Routine laboratory tests such as D-dimer, erythrocyte sedimentation rate, C-reactive protein, and tumor markers did not show good diagnostic value, some showed slightly elevated neuron-specific enolase (less than twice the upper limit). Electrocardiogram can usually indicate nonspecific manifestations such as right ventricular pressure elevation, right ventricular hypertrophy, and changes in the ST segment and T wave. Therefore, it is difficult for clinicians to suspect the diagnosis of PPATs based on the above-mentioned clinical manifestations and routine laboratory tests.Clinical manifestations of patients with PPATs are not specific, and the differences in clinical symptoms may depend on the size and anatomical location of the tumors. Patients with PPATs usually present with common cardiovascular or pulmonary symptoms, such as dyspnea, cough, chest pain, and hemoptysis. Dyspnea is the most common symptom. Cough, chest pain, hemoptysis, and syncope are also common,As a rare disease with a low incidence, PPATs can only be definitively diagnosed by biopsy or surgical excision. In the absence of specific clinical manifestations and laboratory tests, early diagnosis is extremely difficult. Patients with early symptoms associated with increased pulmonary vascular resistance tend to be diagnosed with common diseases. Meanwhile, PPATs are often misdiagnosed as acute or chronic PE in pulmonary augmentation CT with lung filling defects. Therefore, we should strengthen the understanding of relevant imaging examinations, such as echocardiography, CTPA, magnetic resonance imaging (MRI), and positron emission tomography/computed tomography (PET/CT), and also the understanding of immunopathology, to enhance the understanding of the disease and reduce the misdiagnosis rate Fig. . which can show right ventricular dilatation and obstruction of the outflow tract or pulmonary artery trunk. However, thrombus and tumor tissues in pulmonary trunk could not be accurately distinguished. Cardiac echocardiography is the most common examination in clinical, and being familiar with the operation and diagnostic significance of this examination can provide more clinical ideas for clinicians. Transesophageal echocardiography can also be used for the diagnosis of PPATs, clarify the physiological significance of the lesions, and evaluate the ventricular and cardiac valve functions. In addition, the application of intravascular ultrasound is helpful to evaluate the degree of pulmonary valve involvement or to distinguish between interlayer, mural thrombus, or malignant tumors of the vascular. On the basis of familiar with echocardiography, it may be possible to better assess disease involvement and severity in conjunction with other examinations.Cardiac echocardiography is essential in the diagnosis and evaluation of patients with PPATs and is used to detect obstruction of the ventricles or outflow tract, Patchy and delayed enhancement with more pronounced delayed enhancement in the venous phase (an average increase of 25HU) was observed on CT angiography, which suggested necrosis, hemorrhage, or ossification. It was found that a large number of patients with PPAS found filling defects occupying the entire lumen diameter of the main or proximal pulmonary artery on CT images, which is rare in patients with PE.,7,17 In this case, CT images may be irregularly distributed or with high-density filling defects, and the images may show lobulated or heterogeneous hilar masses starting from the pulmonary artery trunk or main pulmonary artery and extending to the peripheral arteries. The tumor vascularity, hemorrhage, necrosis, and ossification in pulmonary artery sarcoma on CT scan show that heterogeneous enhancement is helpful for us to distinguish pulmonary artery tumors from PE disease. For benign tumors of the pulmonary artery, such as angiomyxoma, the results of the CT scan depend on the origin of the lesion. Tumors of extraluminal origin may present as nodules and invasive masses, while intraluminal growth may show intravascular filling defects. In conclusion, the differential manifestations of pulmonary artery tumors distinguished from PE by CT scan include high-density lesions, uneven enhancement caused by hemorrhage, the beaded appearance of the pulmonary artery, filling defects with soft tissue features in the pulmonary artery, and distal vascular decrease and vasodilation. In addition to contributing to the diagnosis of primary pulmonary artery tumors, CT scan can detect the local spread of the tumors to the mediastinum and parenchyma and evaluate cardiac function in patients with PPATs by indirect imaging features of right ventricular pressure overload, such as right ventricular dilatation and contrast agent regurgitation. Clinicians should be familiar with and master the CT imaging features differences of pulmonary embolism disease and pulmonary artery tumors to quickly grasp the imaging features for differential diagnosis and avoid misdiagnosis.Contrast-enhanced CT scan of the chest is usually the first imaging technique to be performed in patients with respiratory symptoms, CT scan findings support the diagnosis of PPATs rather than PE, including aspects of both morphology and density. On the plain scan, the pulmonary artery showed iso-low-density filling defects . Compared with thrombus, PPATs have a higher level of gadolinium enhancement on MRI images, and gadolinium enhancement of MRI helps to identify intraluminal involvement of the PPATs. This enhancement may be related to tumor differentiation and mucus matrix content, and post-gadolinium inversion recovery magnitude and phase-sensitive images showing heterogenous hyperenhancement of the mass consistent with malignant tumors. In the 2017 literature, Liu et al described the specific manifestation of pulmonary artery tumors on MRI, that is, the grape-shaped appearance of the pulmonary artery, which was the result of the extension of the intraluminal filling defects to the peripheral arteries, and found the signs of cardiac invasion, which had strong specificity for the diagnosis of PAS. The morphology of PPAT is usually dilated growth with corresponding pulmonary artery widening, which helps to distinguish PPAT from PE. In addition, the proximal part of pulmonary artery tumor lesions is often convex and lobulated, and the distal part is aneurysmal expansion or grape-like, suggesting that these changes may be caused by local accumulation, expansion and infiltration of tumor tissues. Soft tissue nodules or masses within the pulmonary arteries, as well as the relationship between the diseased tissue and the pulmonary valve or right ventricle, could be clearly visualized on MRI from the main pulmonary trunk to both pulmonary arteries. At the same time, it also provides opportunities to observe the tumor activity through blood flow. Since MRI enables high-resolution imaging in soft tissues, it is more advantageous when distinguished from PE, and can be used for posttreatment follow up and monitoring of patients with primary pulmonary artery tumors to assess residual or recurrent tumors.,22 Although MRI has a good ability to describe organizational features, it still has some shortcomings, including lower spatial resolution and longer breath-holding time. If we consider that most patients examined for this condition have severe dyspnea, this examination may not be chosen.,23 In short, with the popularization and application of magnetic resonance imaging technology, we should make full use of the specific manifestations of soft tissues in MRI, to help us to make the differential diagnosis of rare diseases and deepen our understanding of the diseases.With the popularity of advanced cardiac imaging technology, despite primary pulmonary artery tumors being very rare, MRI, as an advanced imaging modality, can timely and accurately diagnose or reduce the types of diseases that need to be identified. PET/CT imaging can show strong uptake of fluorodeoxyglucose in the lesion site and can also show metastatic focus. Although acute PE may also have some fluorodeoxyglucose uptake capacity, pulmonary artery malignant tumors have a higher fluorodeoxyglucose uptake capacity. Some experts have proposed that the SUVmax 3.5 is the critical point, and the sensitivity, specificity, and accuracy for identifying PPATs and PE are 100%. The study, which used 18F-fluorodeoxyglucose (FDG) PET/CT for comprehensive analysis for the first time to distinguish malignant PPATs from PE, showed that SUVmax was an accurate index for determining the nature of pulmonary artery lesions when the critical value of SUXmax was 3.3. Xi et al suggested that the sensitivity of PET/CT could reach 98.4%, but it is noteworthy that a false-negative case (SUVmax2.6) was reported in the literature. In addition, several other literatures have also stated that PAS has a poor ability to uptake FDG, which may be due to its low cell density and rich mucus-like tissue.,28,29 Therefore, the actual sensitivity may be lower than 98.4%. Except for PE, benign lesions of the pulmonary artery include other rare diseases that may present with varying degrees of FDG uptake, for example, a case of suppurative granuloma with a high uptake rate (SUVmax 4.1) was reported in the literature. Few reports suggest that benign pulmonary artery lesions have a strong uptake ability of FDG.,31 Tumor necrosis, hemorrhage, or calcification may also be associated with rare FDG uptake in tumors, with the 18F-FDG uptake rate being significantly higher in some patients than that in patients with thrombosis. The exact location of the tumors is important for complete resection of the PATs, it can help to determine the survival range of the tumors, thus optimizing the surgical plan. It often uses PET/CT for staging in other tumors, and it has been reported in the literature that PET/CT shows local recurrence of lung metastasis, which also reflects the role of PET/CT in rare tumor restaging. However, the tumor burden near the right ventricle may be confused by the high uptake of FDG in the myocardium, which is limited by the low sensitivity and the potential for false positives compared to MRI, such as benign lipomatous hypertrophy is highly metabolically active. In conclusion, the specificity of 18F-FDG PET/CT imaging in the differential diagnosis of malignant pulmonary artery tumors from PE far exceeds its sensitivity in the existing literature. Thus, it may be necessary to evaluate the cardiac influences of the tumor in combination with other examination methods such as MRI.PET/CT is often used to confirm the suspicion of PE and to differentiate PATs from PE based on the uptake of radiopharmaceuticals by tumors. Pulmonary artery malignancies are more likely to be supplied by a large number of vessels and have higher metabolic activity than pulmonary embolic diseases; therefore, PET-CT, as a metabolic imaging means, can determine the degree of malignancy of the tumors.,25,35,36 and an obtuse angle with the vessel wall. In addition, approximately 90% of pulmonary artery neoplastic lesions involve more than 2 parts of the pulmonary artery, most commonly affecting the lobar pulmonary artery (85%), followed by the right pulmonary artery (71%), the left pulmonary artery (65%), and the right ventricular outflow tract (10%). In 1 study, CTPA was performed in 12 patients with PPAS, 156 patients with chronic pulmonary thromboembolism combined with pulmonary hypertension, and 426 patients with acute PE, the results showed that all patients with PPAS had “wall eclipsing signs,” while none of the patients with acute and chronic thromboembolism showed this sign. This sign is defined as the presence of the following 3 findings: a low-density intraluminal mass of the pulmonary trunk, left pulmonary artery, or right pulmonary artery with near complete occlusion; proximal protrusion of the mass toward the right ventricular outflow tract; and eclipsing of 1 or both walls of the involved artery before the lesion infiltrates beyond the artery.,39 Therefore, the “wall eclipsing sign” on CTPA is considered to be the specific manifestation of pulmonary artery tumors.,38–40 With this manifestation, it is possible to accurately differentiate PPATs from PE, which requires that both Radiologists and clinicians be familiar with the characteristics of this disease on CTPA, to improve the accuracy of the diagnosis.CTPA is currently considered to be a useful tool for differentiating PPATs from pulmonary thromboembolic diseases. Although both show filling defects of the pulmonary artery on imaging, PPATs can extend to the main pulmonary artery trunk and the right ventricular outflow tract and tend to occupy the entire lumen, with localized tumor-like dilatation, widened pulmonary artery diameter, and the possibility of forming a characteristic acute angle with the vessel wall, and tumor proximal ends usually have lobulated, raised margins, while pulmonary artery thrombosis usually has straight cup-shaped edges Most of the tissues were obtained by surgery or autopsy.,41 Given the risk of potential complications during the biopsy process, such as CT scan-guided transthoracic aspiration and transbronchial biopsy, the role of a preoperative biopsy in patients with pulmonary artery tumors is controversial. Some studies have shown that endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) can obtain a pathological diagnosis. EBUS-TBNA allows better visualization of the tumor and vascular flow via doppler ultrasonography, and the obtained cytological materials can be quickly and in-site evaluated to facilitate accurate and early diagnosis of PATs,,43 which was speculated to be a minimally invasive and feasible method for assessing intravascular lesions.,45 However, it has also been stated that PH caused by occlusive lesions increases the risk of bleeding complications.,46,47 Thus, in the diagnosis of PPATs, the limited sample sizes make the relevant literature less reported, and there is still controversy about the application of this method in the diagnosis of this disease. Another technique using the endovascular catheter-guided forceps has been able to successfully detect intimal sarcomas of the pulmonary artery in more than 75% of the patients,,48 which is an effective and safe diagnostic tool for patients with PPATs. Since the tumor tissue of pulmonary artery sarcoma is always covered with necrotic tissue or in situ thrombus, it can be repeated to obtain the actual lesion site by the intravascular catheter biopsy, intravascular catheter biopsy should be performed for early diagnosis when PPATs are suspected.,49 The endovascular catheter-guided forceps biopsies require extensive training in pulmonary arteries catheterization, as well as the use of biopsy devices with considerable catheter sizes to extract sufficient tumor tissues. It may be an interesting method for patients with inoperable or suspected disease recurrence.,51 Due to the rarity of PPATs, there is no clear consensus on the diagnosis of PPATs. However, with the advancement of medical technology, various methods for obtaining tissues are constantly being tried, and there are still disputes on safety and reliability, which requires continuous technical training, and accurate estimation and processing ability for possible risks. So that it can be more skilled and safer in the diagnosis process.As mentioned above, imaging may only indicate the possibility of diagnosis and help to determine the extent of involvement, and histopathological and immunohistochemical findings are needed to determine the final diagnosis.,25 The immunohistochemical staining for PAS revealed that vimentin was strongly expressed in all patients with PAS,,52 with positive expression of desmin and smooth muscle actin (SMA) in varying degrees, and negative expression of endothelial and epithelial markers as well as S100 protein.,52 In addition, murine double minute 2 (MDM2) is a proto-oncogene that is a negative regulator of the p53 protein. Immunohistochemical analysis showed that MDM2 was expressed in the most PASs and related to the amplification of the MDM2 gene in 12q13-14, indicating that the MDM2/p53 pathway might be a mechanism for the pathogenesis of PAS.,52 The fluorescent in situ hybridization (FISH) analysis is a reliable and sensitive technique for detecting MDM2 amplification. Using FISH technology in patients with pulmonary artery sarcoma, the positive rate of platelet-derived growth factor receptor α (PDGFRA) amplification was 81%, the positive rate of MDM2 amplification was 65%, and the positive rate of the epidermal growth factor receptor (EGFR) amplification was 76%. Noteworthy, the activation of EGFR and PDGFRA usually coexists with the overexpression of MDM2, which can improve the accuracy of the diagnosis of PAS. A literature study performed the cytopathological examination of tissues obtained through EBUS-TBNA, which confirmed that tumor cells were positive for vimentin, desmin, and SMA, and chromosome in situ hybridization on the cells showed significant amplification of MDM2 gene locus, which was consistent with the above view, thus confirming the diagnosis of PAS. In a case of a primary malignant solitary fibroma, the immunohistochemical staining showed positivity for vimentin, CD34 antigen, and CD99 antigen of tumor tissue. It has also been reported in the literature that RUNX-1, nestin, WT1, and CD44 were expressed in the PAS. The pathological diagnosis of PPATs is based on the tumor’s location, morphology, and immunohistochemistry . Special cytogenetic changes, including MDM2 and PDGFRA amplification in FISH or comparative genomic hybridization analysis, can further confirm the diagnosis.,52 With the research on immunohistochemistry, it is believed that more specific markers will be found in PPATs to improve diagnostic accuracy.The histologic patterns of PPAS are heterogeneous. These can show heterologous elements like osteosarcoma or chondrosarcoma. In the most common, undifferentiated form, tumor cells are identified as exhibiting spindle or epithelioid morphologic features cytologic atypia, and pleomorphism. When compared with an organizing thrombus, the thrombus usually contains fibrin, RBCs, and lines of Zahn, as well as endothelial cell growth. In contrast, the malignant tumors of the pulmonary artery have high malignancy and poor prognosis, with an average survival rate of 17 months after diagnosis. Without surgical intervention, the average survival time after diagnosis is only 1.5 months.,14 Due to the lack of clinical trials with sample data for PPATs, there is no clear consensus on the treatment, and the treatment decision depends on the patient’s symptoms, the size of the lesion, its distant spread, and the possibility of the clinical team to determine a successful treatment. The standard treatment is early and aggressive surgery, which aims to complete surgical resection with clear margins.,14 Surgical data for PAS have been extrapolated from PATs with varying incidences.,14,56 It has been reported that the survival rate of patients with successful surgical resection can be increased to 8 to 36 months. Patients who attempted radical resection had a longer OS than patients with incomplete resection (median OS was 36.5 months vs 11 months). In line with these outcomes, in a case series of the 28 patients with PAS, 18 underwent surgical treatment , and the median OS tended to favor the surgical approach (20 months vs 9 months). Currently, the reported surgical methods include unilateral pneumonectomy, lobectomy, radical tumor resection combined with pulmonary artery reconstruction, pulmonary endarterectomy, and combined cardiopulmonary transplantation.–60 The choice of surgical approach mainly depends on the location and scope of the tumors as well as their metastasis.,56 Some experts believe that the survival time of PPAT patient with tumor resection combined with pulmonary endarterectomy is better than that of patients with tumor resection alone. For most patients with PPATs, the tumor cells originate from the proximal pulmonary artery intima and diffuse to the distal end with the intima. Compared with simple tumor resection, pulmonary artery endarterectomy can restore blood flow in the affected area of the lung, ensure adequate oxygenation, and relieve pulmonary artery pressure. As a palliative treatment, it can protect the pulmonary vascular bed and reduce the incidence of pulmonary artery pressure.,61 Since patients of PPATs with PH generally have bilateral lesions, multiple medical centers have also emphasized the simultaneous removal of tumors and bilateral pulmonary endarterectomy.,61 Even in patients with unilateral lesions without PH, tumor cells may have been planted bilaterally at the time of onset.,62 Radical tumor resection and pulmonary artery reconstruction are the most promising treatments for PPATs, which may require the resection of important structures, such as pulmonary valves or the right ventricular outflow tract. Resection of metastatic lesions distal to the implantation site showed a higher survival rate compared with distal embolectomy. Combined cardiopulmonary transplantation is also a treatment, but the success rate is very low. Survival after surgical resection seems to be related to local recurrence rather than progression through metastasis. Long-term survival data of ≥ 7 years after the initial operation have been reported, and systemic metastasis is rare. Therefore, it is recommended for reoperation or resection of metastatic lesions.,64 The majority of patients were recommended to use low-molecular-weight heparin for thromboembolism prevention for 6 weeks after the operation.Pulmonary artery benign tumors are usually treated with surgery, which can be cured by surgical resection and has a low local recurrence rate. reported the 3-year survival rate and 5-year survival rate of 31 cases of PAS are only 29% and 22% respectively. Some experts believe that the reason why surgery alone cannot achieve good results is the delayed diagnosis, it is difficult for surgeons to remove it completely with the progress of the tumor. After surgical resection, reducing the tumor load is also very important during treatment, it can be seen that chemotherapy and radiotherapy also seem to be effective treatments. However, there are no standard chemotherapy guidelines. Currently, the chemotherapeutic regimen combining adriamycin and ifosfamide was demonstrated to be effective regardless of the histological subtype of these tumors. The use of other agents, including carboplatin, epirubicin, cyclophosphamide, gemcitabine, dacarbazine, etoposide, and vinorelbine, have also been reported in some types of PPATs with certain effects.,13,65 Some experts believe that when some patients are unable to undergo surgery with complete tumor resection, neoadjuvant chemotherapy is recommended to improve the prognosis of patients with locally advanced soft tissue sarcoma and make surgery easier. It has been reported that neoadjuvant chemotherapy has led to operation on patients who were initially inoperable and had a positive effect on survival. Adjuvant therapy for high-risk tumors with a depth or height of more than 5 cm has been recommended in the literature. Some authors emphasized the use of chemotherapy and/or radiotherapy on an individualized basis, while others emphasized that adjuvant therapy was recommended whenever possible to prolong the remission period.,56,60,61 Available data confirm that the median survival rate of patients receiving adjuvant therapy is improved than that of patients undergoing surgery only, which indicates that the treatment is effective.,68 Many case reports have been published with successful results in favor of chemotherapy in the postoperative setting, especially in patients with residual lesions with doxorubicin-based chemotherapy.,70 Delayed chemotherapy for relapse was also demonstrated to be successful in a patient who was treated with amrubicin and ifosfamide. Adriamycin-based chemotherapy remains the most effective strategy for soft tissue sarcoma in the presence of tumor metastasis. The aggressive nature of PPATs and the pulmonary complications associated with surgery have led to the limited treatment of metastatic patients.Although surgery can prolong the median survival time of patients, the long-term prognosis is still poor. Mussot et al and the indications for PPAT still need to be further explored. Secondino et al thought that more than 80% of patients with PPATs received adjuvant therapy, whether 6 or 4 cycles of a chemotherapeutic regimen combining adriamycin and ifosfamide, followed by radiation therapy of 60GY. The median survival time for both groups was 26 months with a range of 10 to 21 months and 4 to 55 months for the chemotherapy alone or concomitant chemoradiotherapy respectively with also a trend towards better survival in patients who received additional postoperative therapy. In addition to its effective role in the postoperative environment, radiotherapy alone can also prolong the survival time in some cases where chemotherapeutic drugs are resistant. Complete regression of tumors after surgery followed by chemoradiotherapy was also reported. In conclusion, multiple safe and effective treatment methods should be integrated as far as possible for patients with postoperative recurrence of PPATs who cannot completely remove the tumor or have a high-risk of postoperative recurrence. However, it is worth noting that surgical treatment remains the preferred option despite the positive effects of chemotherapy and radiation on the patient’s survival rate, chemotherapy and radiotherapy only play a supplementary role, allowing inoperable patients to operate after adjuvant treatment. Therefore, the comprehensive application of multiple treatment regimens may play a positive role in improving the prognosis  and serine/threonine kinase (AKT) signaling pathways. Van Dievel et al treated 4 patients with metastatic PPAS with PDGFRA mutation with imatinib, of which 1 patient obtained partial remission. Due to the clonal heterogeneity of PPATs and the coexistence of multiple genetic changes , targeting multiple tyrosine kinase inhibitors becomes a necessity and an innovative approach in these tumors to improve the oncological outcomes of these dismal tumors. Pazopanib, a multitargeted tyrosine kinase inhibitor including PDGFR, is approved for the second-line therapy of advanced non-adipocytic soft tissue tumors after doxorubicin and ifosfamide failure. A retrospective analysis by Kollar et al of 2 patients with endometrial sarcoma, out of 52 patients with vascular tumors, showed partial remission in both patients in the second-line metastatic disease after receiving pazopanib treatment, but there were no relevant reports of patients with PPATs. Further studies are needed on the efficacy and safety of possible targeted therapeutic agents, and the exploration of more biomarkers for PPATs and therapeutic targets may be the direction in the future.With the discovery of potentially targetable genetic alterations PPATs are rare in the clinic and have an extremely poor prognosis, early diagnosis is difficult, and it is easy to be misdiagnosed as PE. Therefore, it is important to consider PPATs in the differential diagnosis of PE to improve the survival rate of patients. When considering that there are persistent vascular filling defects and long clinical durations after adequate anticoagulant therapy, it is recommended to conduct a rapid and comprehensive examination by echocardiography, CT, MRI, and PET/CT as soon as possible in order to improve the early diagnosis rate and finally provide a clear pathologic diagnosis by biopsy. Surgical treatment can effectively improve the survival rate and prolong the survival time of patients. The combined application of chemotherapy, radiotherapy and other treatment methods may have a better therapeutic effect on the PPATs. It still needs continuous research and exploration of more safe and more effective treatments to improve the survival rate and prognosis of patients with PPATs.We are most grateful to all participants in the present study.Formal analysis: Xiuqing Liu, Xuhan Liu, Ruirui Li.Investigation: Xiuqing Liu.Methodology: Xiuqing Liu.Writing – original draft: Xiuqing Liu, Xuhan Liu, Ruirui Li.Writing – review & editing: Weihua Zhang.


Parapelvic cyst (PPC) is a rare cyst that arises from the renal parenchyma and usually does not cause symptoms. However, PPC may cause compression of the pelvicalyceal system resulting in obstructive symptoms. Ultrasonography may not be sufficient to diagnose PPC since they are often misdiagnosed with hydronephrosis, thus computed tomography of the renal is required. In this case, we present, a 27 years old male with a 3 years history of partial urinary retention presented with intermittent loin pain. Hydronephrosis was found on ultrasonography but failed to identify the source of obstruction. A CT scan was conducted and a PPC was confirmed. Nephrostomy was scheduled for ureteral dilation and the patient responded well 4 months after surgery. In this case, we highlight the role of radiology in identifying and differentiating the PPC. Renal cyst, including pararenal cyst, is the most common lesion found in the kidney. Renal cysts affect approximately 40% of all individuals undergoing renal imaging that can be unilateral/bilateral, focal/multifocal, or acquired/congenital Several studies have demonstrated the imaging of PPC. A PPC at times may cause compression of the pelvicalyceal system resulting in hydronephrosis. Commonly, PPCs do not communicate with the collecting system and are believed to be lymphatic in origin. A PPC is a nongenetic cyst with pathological changes; the incidence is about 1%-2% of total renal cysts A 27-year-old male with a 3-year history of partial urinary retention presented with intermittent loin pain. History of fever, stone passage through urine, and hematuria was denied. His vital signs were within normal limits. Physical examinations were within normal limits. Laboratory examinations including hematology, renal function, and urinalysis found no abnormalities. Initial ultrasonography was performed and found pelvicalyceal dilatation and the proximal ureter was dilated, indicating pelvico ureteric junction obstruction (PUJO) .Fig. 1LoRenal ultrasound was unable to identify the source of obstruction, thus further imaging was conducted. A renal computed tomography (CT) CT scan was performed and showed the normal size of the right kidney measuring 10.44 × 4.68 cm. The Parenchyma structure is homogeneous, pelvocalyceal system is widened. There was a hypodense lesion in the renal pelvis, extending to the proximal ureter that was not filled with contrast measuring 5.03 × 5.36 × 5.98 cm .Fig. 2CoThe proximal right ureter was dilated. The cyst presses the right renal pelvic system and right proximal ureter causing PUJO, causing hydronephrosis grade III. He was advised to undergo a nephrostomy for ureteral dilation followed by double J stent placement. A percutaneous approach for cyst removal was deemed very difficult in this patient, thus nephrostomy was conducted using an 8-8.9 Fr nephroscope. To be able to remove the cyst, the cyst wall had to be very thin and well-visualized to avoid renal parenchyma injury. However, in our case, the cyst wall was too thick and poorly visualized, thus cyst removal was not conducted. Cyst removal failed, thus we proceeded to perform ureteral dilatation. The double J stent was inserted and bleeding was checked. Four months after surgery, the cyst remained, but the patient was asymptomatic.PPC is a relatively rare disease with an incidence of 1%-2% of total renal cysts. PPCs do not communicate with the collecting system and are believed to be lymphatic in origin. While other evidence suggests that PPCs are embryological remnants of the developing mesonephric duct. The mechanism of formation and tissue structure of PPC are almost the same as the simple kidney cyst. Generally, this disease is either caused as a result of congenital dysplasia or acquired obstruction in the kidney. The resulting anatomical disturbance in patients with PPC may cause chronic inflammation and local expansion of pelvic lymphatic vessels. This mechanism was thought to be responsible for the associated history of urinary tract obstruction and infection in patients with PPC. The association between PPC with other anatomical anomalies remained unknown. However, it is possible that the associated anomaly within the urinary tract contributes to UTI which may increase the risk of PPC Marret et al found that the histology within the PPC consists of a single flat epithelial cell layer, suggesting that PPC and the urinary tract are structures of independent origins. A communication between the cyst and the collecting system has never been shown in the literature. They are usually asymptomatic unless the cyst obstructs the ureter, causing PUJO. Due to its rarity, there are only a few studies reporting the case of hydronephrosis due to PPC. In this case, we highlight the role of radiographic imaging in PPC.Most renal cysts are symptomless, but they can occasionally cause lumbar discomfort, hematuria, hypertension, and hydronephrosis due to obstruction by the cyst. However, the possibility of a PPC must be kept in mind when the asymptomatic hydronephrosis is noted by ultrasonography with unknown causative obstructive lesion, since PPCs are often misdiagnosed as hydronephrosis. In our case, the patient reported intermittent loin pain without significant urinary tract symptoms due to the hydronephrosis caused by the cyst Only a few studies reported the radiologic features of PPC. PPC can mimic hydronephrosis and so can be confused with pelvi-ureteric junction obstruction (PUJO) as the ureter in both conditions is not dilated. This could be well differentiated at the excretory phase of the CT-IVP study (CT urography) that demonstrates stretched (but not dilated) collecting system by the cyst in contrary to PUJO at which the contrast will be pooled within the dilated collecting system. It can cause pelvicalyceal system dilatation by its mass effect A case report by Mao et al.  reportedSymptomatic or complicated PPC mandates corrective intervention. Various treatments for simple renal cysts have been proposed with varying outcomes, including sclero- therapy, laparoscopic unroofing, and percutaneous ablation. Compared with the treatment of simple renal cysts, the treatment of PPC can be more difficult because of the location near the renal hilum and renal vessels. Sclerotherapy is considered to be contraindicated in the treatment of PPC because it can provoke perirenal inflammation and subsequently, ureteropelvic junction obstruction. Percutaneous aspiration of PPC has been condemned in view of grave complications like retroperitoneal leakage of sclerosant and perirenal inflammation and the recurrence rate is 22.8%-30%. Percutaneous nephroscopy-guided resection and retrograde ureteroscopic resection are widely accepted treatment options although they may be limited by the size and location of the cyst. The work access sites connected to percutaneous nephrostomy invariably result in renal injury, such as severe perinephritis, retroperitoneal abscess, and subsequent ureteropelvic junction obstruction. This is in contrast to percutaneous resection or ablation. For some cysts, the retrograde method might be less invasive. The danger of severe consequences has decreased because of the flexible ureteroscopic approach. One of the most important aspects of PPC removal is the placement of the renal cyst wall. PPC wall thickness may determine if it is removable or not. Laparoscopic management has been sparsely reported and apprehended in literature because of extensive dissection and technical difficulty PPC is usually asymptomatic, however, if it obstructs the normal urinary tract, it will cause hydronephrosis and comprehensive imaging is needed to better visualize the cyst. This case represents one example of a PPC causing hydronephrosis with a good prognosis even without cyst removal surgery.I, on behalf of all authors, confirm that we have obtained written informed consent for publication from the patient. Subjects voluntarily participate in this study. Subject agrees that Subject has been given the opportunity to ask questions and have them answered to Subject's satisfaction. The subject had noticed that his ultrasound and CT scan findings would be published for scientific purposes. Subject has received a copy of this consent form signed by the researcher.


We report the rare case of a giant low-grade appendiceal mucinous neoplasm (LAMN), presenting as ileocecal intussusception. An 80-year-old woman presented in the emergency department of our institution with progressively worsening diffuse abdominal pain during the last 24 h. A CT scan revealed a giant abdominal mass (98.7 × 127.3 × 107.6 mm) with air-fluid level and imaging characteristics of ileocecal intussusception. An emergency exploratory laparotomy was performed, and a well-circumscribed cystic mass deriving from the appendix was found. A right hemicolectomy was performed, and the histopathological examination confirmed the diagnosis of LAMN. This report aims to raise awareness among surgeons and radiologists, about LAMNs as a differential diagnosis of right iliac fossa masses presenting as acute abdomen. Appendiceal neoplasms represent 0.5% of all gastrointestinal tract tumors, whereas almost 0.9–1.7% of appendectomy specimens may harbor malignancy .Appendiceal mucinous neoplasms (AMNs) are rare tumors, with a benign to malignant potential . AMNs arHerein, we present a rare case of a giant LAMN presenting as ileocecal intussusception.2.An 80-year-old postmenopausal woman presented to the emergency department of our institution with progressively worsening diffuse abdominal pain for the past 24 h. The patient also reported several episodes of vomiting. Her medical history included two cesarean deliveries, arterial hypertension, dyslipidemia, trigeminal neuralgia and a reducible umbilical hernia. There was no report of previous endoscopic or imaging studies of the abdomen. She was neither a smoker nor an alcohol consumer. Her BMI was 25 kg/mThe patient presented hemodynamically stable, with clinically generalized peritonism. Abdominal examination revealed diffuse tenderness, guarding and a positive Blumberg’s sign. Abdominal auscultation confirmed the absence of bowel sounds. Digital rectal examination showed an empty rectum. The following vital signs were recorded: blood pressure: 110/70 mmHg, pulse rate: 98 beats/min, SpO2: 96%, body temperature: 37.1°C.Laboratory examinations  revealedAbdominal ultrasound revealed a dense, fluid-filled mass in the right iliac region, with a significant amount of free intraperitoneal fluid adjacent to the ascending colon. The CT scan demonstrated a large lesion (98.7 × 127.3 × 107.6 mm) with a fluid-gas level that appeared to be attached at the base of the cecum . It exteThe patient was submitted to an emergency exploratory laparotomy. Intraoperatively, a well-circumscribed cystic mass at the region of the ileo–cecal junction was found  and b. THistology results  and b reLAMNs are semi-malignant tumors of the appendix, diagnosed as an incidental finding in up to 1% of appendectomy specimens, at a mean age of 62.03 years, and with females being predominantly affected . The terPreoperative diagnosis of LAMNs remains challenging, because of clinical heterogeneity. An acute appendicitis-like presentation with right lower quadrant pain is common for early stage disease, as opposed to advanced-stage symptoms like chronic abdominal pain, urinary retention, constipation and new-onset umbilical or inguinal hernias because of increased abdominal pressure , 8. IleoPreoperatively contrast-enhanced CT has an accuracy of 89.7% . For theThe tumor mass of the reported case was characterized by the absence of perforation, a strong inflammatory response  and negative tumor markers, despite the advanced presentation, disease stage and association with NLR; 7.17  and PLR; 0.19  .Generally, small LAMNs can be treated with open or laparoscopic appendectomy, but for larger LAMNs, laparotomy may be necessary because of possible cecal involvement . If LAMNIn our case, a giant LAMN presented as an ileocecal intussusception. Because of the intraoperative findings a CME right hemicolectomy was performed.None declared.


An abdominal aortic aneurysm (AAA) is a confined dilatation involving the abdominal aorta. The incidence is rare and the etiology is unknown. Cases associated with conditions like Kawasaki, connective tissue, Behcet’s diseases, and vasculitis are considered acquired. Our patient had a clinical criterion of Behcet’s disease. Management involves a surgical approach. Endovascular intervention is not an option here, as the aneurysm is close to the bifurcation evident in computed tomography angiogram scans. Usually, they have good long-term outcomes. In our paper, we aim to describe the clinical presentation, management approach, and the outcome of our patient with an acquired AAA. An abdominal aortic aneurysm (AAA) is defined as a confined regional enlargement in the abdominal part of the aorta with at least 3 cm or 50% above the normal diameter of the vessel . The incDescribing the AAA case in the literature will help us identify the potential etiology behind it. Furthermore, a good outcome depends on early identification and an active management approach. Hence, we aim to report an acquired inflammatory AAA in a 10-year-old boy which was managed effectively by surgical intervention.A 10-year-old boy, with no comorbidities, presented to our hospital complaining of recurrent buccal mucosal ulcers on the right cheek and tip of the tongue. Also, he had blurred vision, intermittent headaches, and photophobia for the last three months before the presentation. There were no other associated symptoms like fever, genital ulcer, rashes, vomiting, and bloody diarrhea. His past family history was insignificant. Upon physical examination, the child looked well, not in pain or dehydrated, with an average body build, and normal color of the skin. General examination was unremarkable apart from an oral ulcer which was evident on oral examination, involving multiple areas of the right buccal mucosa, and the tongue. Fundoscopic examination showed no signs of disc edema. Upon admission, the patient was hemodynamically stable, and had a normal heart rate, normal body temperature, and respiratory rate, along with normal oxygen saturation on room air.However, initial laboratory results showed low hemoglobin (8 g/dL) and thrombocytosis. Inflammatory markers were elevated, C-reactive protein (CRP-226 nmol/L), and erythrocyte sedimentation rate (ESR-121 mm/h). Formulating all giving pieces of information, with the clinical presentations, physical assessment, and laboratory investigations, the differential diagnoses were: Behcet’s disease (BD), Crohn’s disease, connective tissue diseases, and unspecified vasculitis. A computed tomography (CT) brain scan was done initially and showed massive sinus thrombosis extending to internal jugular veins bilaterally. In addition, magnetic resonance imaging (MRI) whole spine was obtained and showed “spinal leptomeningeal enhancement, and nerve root thickening”. After considering the findings in the CT scan with the MRI and the clinical presentation, BD was the top differential diagnosis. Initially, the patient was started on steroid pulse therapy at 10 mg/kg with an infusion of infliximab as per the guideline protocol from the rheumatology side which was improved dramatically in terms of clinical response, and resolution of the patient’s symptoms. He also was kept further on low molecular weight heparin (LMWH), folic acid, oral prednisolone (tapered down later on), azathioprine, colchicine, omeprazole, and calcium Sandoz. Post-treatment, inflammatory markers were markedly reduced (CRP was 26 nmol/L and ESR was 1 mm/h). The patient recovered sufficiently to be discharged home with a multidisciplinary team follow-up.Six months later, he started to develop nausea and vomiting, with severe right lower quadrant abdominal pain over the last 72 hours, and was admitted again to our hospital. As part of the outpatient workup, the patient had a CT scan of the abdomen outside our hospital, with findings consistent with terminal ileitis, and ruled out the differentials of acute appendicitis and mesenteric adenitis. Then, he was referred to us for further investigation and management. In the beginning, he was evaluated by a gastroenterologist and they proceeded with a colitis workup including MRI enterography which demonstrated an incidental finding of AAA Figures -1B whichFour months later, he was re-admitted due to multiple oral ulcers and poor oral intake. Computed tomography angiogram (CTA) showed the same previous findings in the MRI with no changes Figures -3C. RegaAAA is an abnormal bulging of a specific abdominal aortic site beyond its normal diameter. In 1967, the first case of congenital AAA was reported by Howorth [The histopathological findings are usually not correlated with the exact cause of AAA because of its late intervention . This coUsually, AAA is asymptomatic and the most common clinical sign is a pulsatile painless abdominal mass, which can be ruptured sometimes and is mostly discovered in the elderly. Older children usually have no symptoms, and the majority are identified while going for different procedures . In one Imaging modalities which include ultrasonography (US), CTA, and magnetic resonance angiography (MRA) are considered the main methods for diagnosis. In terms of screening for AAA, the US is useful in detecting and measuring AAA diameter. Preoperatively, CTA provides an accurate diagnosis of AAA due to its ability to determine the capability of doing endovascular repair (EVAR) . HoweverThe management of AAA would involve a conservative or surgical approach . The conRegarding the prognosis and mortality outcome, there were no studies that had a clear and focused long-term follow-up for an acquired AAA patient. However, the mortality rate was high 30.8%) due to delayed diagnosis and management according to the literature review done on 26 patients with congenital AAA . The mor% due to Management of vascular conditions like AAA in pediatrics and when to intervene or the type of interventions must be cautiously planned. A relatively small arterial diameter, ongoing growth-up of the child, and availability of suitable vascular conduits need to be considered in the pediatric age group. Therefore, wide and precise knowledge with a careful understanding along with knowing the interventional options is particularly important in managing children with this condition. Early identification of both congenital or acquired AAA and the careful individualized surgical repair carry favorable outcomes, and interventions would be chosen either with or without grafts. More studies are required to further classify those cases, monitor their long-term outcomes, and build up a precise management algorithm is warranted.


Giant fibroepithelial polyp (FP) of the scrotum in infants is a rare disease. We reported the first case of FP in China. The child was only 9 months and 12 days old and was admitted to the hospital due to rapid growth and rupture of the scrotal mass. The patient underwent scrotal exploration under general anesthesia, and the mass was cystic-solid with clear boundaries. The tumor did not invade the sarcolemma of the scrotum and testicular tissue. The intraoperative pathological frozen section tended to be benign, and the scrotum's tumor and subcutaneous pedicle tissue were removed entirely after 0.5 cm from the boundary of the mass. The operation was successful. The mass was confirmed as FP by postoperative pathology. 6 months after the operation, the incision healed well without recurrence. This case report has a detailed diagnosis and treatment process and adequate examination results. It can provide a reference for diagnosing and treating FP in infants and reduce the risk of misdiagnosis and mistreatment. Fibroepithelial polyps (FP), called soft fibromas, are a kind of pedunculated benign tumor. It is common in middle-aged or older adults , especiaHere, we report a case of a 9-month-old boy with a rapidly expanding mass in the scrotum that ruptured and was completely removed by surgery. Postoperative pathology confirmed that the child was a giant FP of the scrotum, and there was no recurrence after 6 months of reexamination.At birth, the child was found by his parents to have a light purple mass in the left scrotum, about 0.5 cm × 0.5 cm in size, protruding from the scrotal skin, with clear boundaries, tough texture, and no tenderness. The patient had no fever and was in good general condition. The family members of the children ignored it and did not go to medical institutions for treatment. The mass enlarged rapidly with age and covered most of the left scrotum. One week ago, the mass ruptured when his family changed the diaper. The scrotal mass of the patient was ruptured from a “mass” to a “cock's comb.” The ulcerated surface resembled new granulation tissue with a small amount of oozing blood. He went to our department for further diagnosis and treatment. Since developing the disease, the child has had good spirits, a good diet, regular urination and defecation, and weight gain. After admission, the relevant examinations were performed. The specialized physical examination showed a mass in the left scrotum with a size range of 6.5 cm × 5.5 cm, which was cauliflower-like growth and scattered with a broad base. There was no obvious blood oozing, redness, swelling, or tenderness, and a clear boundary with normal scrotal skin . PreoperScrotal tumor resection was performed under general anesthesia after excluding surgical contraindications. Intraoperative frozen pathology showed that the tumor tended to be benign . After eFP mainly occurs in postmenopausal women and is considered to be closely related to estrogen levels and the occurrence of polycystic ovaries . PreviouFP usually occurs in the face and neck, axilla, and groin regions. A few are located in the vulva , bladderThere are few studies on the pathophysiology and susceptibility of FP. El Safoury et al. analyzed skin sections from 15 patients with skin vegetations (TS) using enzyme-linked immunosorbent assay and reverse transcriptase polymerase chain reaction and showed that mast cells, tumor necrosis factor-α (TNF-α), and TNF-related apoptosis-inducing ligand (TRAIL) might play an important role in ST patients . Farag eTypical FP is characterized by the formation of pedunculated polypoid nodules protruding from the skin surface. According to the morphological characteristics, it can be divided into three types: (1) exanthematous soft fibromas: multiple small papules, usually occurring on the neck; (2) filamentous soft fibroma: filamentous growth of myxoid processes; (3) pedicle type soft fibroma: more common in the lower part of the trunk and inguinal region, with pedunculated polypoid processes, soft, often more than 1 cm in diameter. Giant FP refers to FP larger than 5 cm in diameter, which is rare in a clinic and mainly occurs in adults . AccordiColor doppler ultrasound and other imaging examinations have some value in diagnosing FP. FP can appear as a hypoechoic mass with clusters of hyperechoic spots on ultrasound. Abundant rod-like blood flow signals can be seen under CDFI . HoweverThe diagnosis of FP requires pathological examination. The typical gross feature of FP is a pedunculated polypoid mass protruding from the skin surface, with a gray-white or gray-yellow cut surface and soft texture . MicroscFP should be differentiated from Dermatofibrosarcoma protuberans (DFSP) and Pleomorphic fibroma of the skin (PFS). Microscopically, DFSP comprised all peripheral nerve components, including Schwann cells and fibroblasts. Most of the nuclei were slender, wavy, and serpentine. The positive expression of S-100 and SOX-10 in immunohistochemistry can confirm the diagnosis of DFSP. In contrast, however, FP was negative for both measures. In PFS, the tumor comprised a small number of spindle cells and abundant collagen fiber bundles, with characteristic multinucleated giant cells and many pleomorphic cells. Immunohistochemically, MSA and CD34 were positive and could be distinguished from FP. In this case, the immunohistochemical S-100, S0X-10, and MSA were negative, which provided a solid basis for the differential diagnosis of FP.Since FP is a benign tumor, local surgical resection is the primary means of clinical treatment. However, paying attention to the complete separation and resection of the tumor and the pedicle tissue during the operation is necessary, which can effectively avoid recurrence and obtain a satisfactory cure rate . HoweverCongenital giant FP of the scrotum in infants is clinically rare, and our center has performed the first case report from China. The lack of obvious clinical symptoms can easily cause guardian neglect, thus delaying treatment. The diagnosis of FP requires a combination of pathological HE staining and immunohistochemical results. The disease is a benign tumor with a good prognosis. However, attention should be paid to monitoring during clinical diagnosis and treatment to reduce the risk of recurrence and malignant transformation.


To identify the characteristics of inflammatory fibroid polyps (IFP) in the terminal ileum and to investigate the methods, safety, and efficacy of colonoscopic minimally invasive dissection and resection therapies for its treatment. Colonoscopy and colonoscopic ultrasonography were used to diagnose patients with protruding mucosal lesions in the terminal ileum, and the results suggested a high suspicion of IFPs. Colonoscopic minimally invasive dissection and resection were performed for these patients, and IFP was confirmed by postoperative pathological examination and immunohistochemical staining. Twelve cases of IFP from April 2016 to December 2020 in our hospital were examined pathologically and immunohistochemically. The IFPs in the terminal ileum were all successfully excised by colonoscopy. There were no postoperative perforation, bleeding, or recurrence cases during the follow-up. The features of the lesions, as well as the efficacy of colonoscopic minimally invasive dissection and resection, were reviewed. Terminal ileum IFPs have corresponding colonoscopic and endoscopic ultrasonographic features. For IFPs less than 2 cm in size and within 10 cm of the ileocecal valve, removal by colonoscopy was proven less invasive, safe, and effective. The lesion was first reported in 1949 and was called “submucosal granuloma with eosinophilic infiltration”, which was later renamed “inflammatory fibroid polyps” in 19533. IFPs tend to occur in adults aged 20 to 90, with an average age of 60. Its typical size ranges from 0.2 to 4.2 cm, with an average of 1.7 cm. IFPs are usually located in the stomach and small intestine, and the incidence is slightly higher in women. The clinical symptoms are related to the location and size of the lesion. IFPs in the gastrointestinal tract can manifest with weight loss, melena, nausea, and abdominal pain, and patients with large lesions are prone to intussusception and intestinal obstruction. Many patients are diagnosed by incidental symptomatic examination4. Small intestinal IFPs often grow in the terminal ileum. Due to its rarity5, clinicians often misdiagnose IFP, especially in the terminal ileum, which is usually small and without corresponding clinical symptoms.Inflammatory fibroid polyps (IFP), also known as “Vanek tumors”, are rare interstitial lesions of the digestive tractCurrently, there is no relevant literature or reviews on minimally invasive resection of terminal ileum IFP via colonoscopy. From April 2016 to December 2020, 12 cases of terminal ileum IFP  were successfully resected in our Digestive Endoscopy Center. The clinical characteristics of the above cases were retrospectively analyzed and summarized.A total of 12 cases of terminal ileum IFPs were diagnosed and resected successfully, including 4 males and 8 females. Notably, 8 out of 12 patients were female , which is consistent with the incidence of IFPs. The mean age was 59.4 ± 10.87 years. Two patients had recurrent pain in the lower right abdomen, one had positive fecal occult blood, and the other 9 showed no clinical manifestations , CD117 (−), DOG-1 (−), S-100 ( −), desmin (−), and low Ki-67 proliferation index (1–5%). Eleven cases were CD34 (+), while one was CD34   mutations12, which are also seen in KIT-negative gastrointestinal stromal tumors. Therefore, some researchers have proposed that IFPs may be neoplastic rather than a simple inflammatory lesion13. Most researchers believe that IFPs are not metastatic or aggressive, and their onset is insidious. However, some lesions may gradually grow in size and cause complications, including intussusception14, abdominal pain16, bleeding18, and obstruction20, so resection is recommended.To this day, the etiology of IFPs remains unclear, though many studies have suggested that they may be related to chemical, physical, and metabolic inducing factorsAll lesions in this study were small in size and resected early in the course of the disease using colonoscopic resection. The lesions were mainly early-onset and without complications. The insertion rate of the terminal ileum by colonoscopy in our gastrointestinal endoscopy clinic is about 86.5% (4439/5132), and the detection rate of end ileal disease is 8.8% (392/4439). We retrospectively analyzed 12 treated cases of terminal ileum IFPs from 2016 to 2020 in our hospital, accounting for 3.1% (12/392) of all lesions in the terminal ileum. Therefore, the insertion rate of the terminal ileum is emphasized during colonoscopy to improve the detection rate of terminal ileum lesions, which contributes to the early diagnosis and treatment of diseases in the terminal ileum. There was some inconsistency between the clinical characteristics of the present study and previous observations, which could be attributed to the small sample size.During the colonoscopic procedure, there were some technical challenges and corresponding procedural skills: (1) The lesion was close to the ileocecal valve, so the colonoscopy was liable to slip out the ileocecal valve, and it was necessary to repeatedly enter the ileocecal valve during the colonoscopic operation, and the operator must be skilled at controlling the colonoscope. In this situation, the operator should try to make the endoscopy body form a fulcrum outside on the examination bed to stabilize the endoscopy body. To ensure the accuracy of the operation, the operator should handle the endoscopy body alone, and the assistant should not fix the endoscopy body. (2) The intestinal wall of the terminal ileum is thin, the submucosal layer is weak, and the submucosal injection layers are not well displayed, so the possibility of damaging or perforating the muscularis propria or the tumor is high. Thus, carbon dioxide was used during the procedure. The injection points were selected 0.5 cm away from the lesion, allowing the submucosal injection fluid to gradually extend to the lesion. Then the mucosa was incised to establish the submucosal space. (3) Endoscopy progression is difficult in the terminal ileum, where it is difficult for the colonoscope to access the lesion, or the transparent cap is difficult to function. This difficulty is usually associated with failure to straighten the endoscopy body or excessive insufflation during manipulation. Thus, the transparent cap must be fully applied to progress the colonoscope along the lumen, reduce the injection of CO2, untie the formed loop in time, and ensure that the colonoscope reaches the ileocecal junction without loop formation. Colonoscopic treatment at this site requires a high level of technical experience; therefore, the director of the Department of Gastrointestinal Endoscopy at our hospital operated on the 12 cases. He completed more than 6000 cases of endoscopic mucosal dissection (ESD). Traction devices were not used in any of the 12 cases. However, for relatively less experienced operators, using appropriate traction techniques can help expose the lesion more fully and facilitate the establishment of the submucosal space, thereby reducing the difficulty of the procedure. (4) Colonoscopic suturing of lesions at this site was difficult, but the operator was experienced, and all wounds were successfully sutured with hemoclips or nylon sutures. For less experienced operator, the insufflation should be reduced to ensure better visualization during the procedure, and the transparent cap should be used more frequently to expose the surgical field. Less insufflation can help to avoid overstretching of the intestinal canal, thereby reducing the difficulty of suturing.ESD at the terminal ileum requires a very experienced operator to perform it, and for less experienced operators, resection of less complexed lesions can be attempted based on the above skills.4.The main histopathological feature of IFPs is the lesions’ location below the mucosa. They present as short spindle-spindle cells arranged in bundles or sheets with medium cell density and an abundance of inflammatory cells in the background. Eosinophils in varying amounts are also commonly found in the interstitium21.The white-light colonoscopy and endoscopic ultrasonography findings of terminal ileum IFPs must be differentiated from lipomas. Lipoma is soft at white-light colonoscopy, without a “bubble” appearance, while under ultrasound colonoscopy, a lipoma is manifested as a homogeneous hyperechoic mass of submucosal origin demarcated from the muscularis propria5. All lesions in the present study were immunohistochemically examined after resection4. The immunohistochemical results of all 12 lesions showed that CD34 and vimentin were highly expressed, while CD117 was not expressed. Therefore, the incidence of IFPs at this site was considered significantly higher than that of other spindle cell tumors, such as leiomyomas and stromal tumors.Additionally, IFPs must be histopathologically distinguished from some digestive tract tumors, non-neoplastic diseases, and spindle cell lesions, including inflammatory fibrosarcoma, spindle cell carcinoid, leiomyoma, gastrointestinal stromal tumor, and nerve sheath tumorIFPs have a fixed spherical protuberance of the mucosa with a slightly yellowish color and hard texture in the terminal ileum. Colonoscopic ultrasound showed that IFPs originate from the inner surface of muscularis propria, protrude into the cavity with a clear boundary, and have equal or slightly higher echogenicity than muscle. Colonoscopic minimally invasive dissection and resection is an important method for treating terminal ileum IFPs, with advantages in terms of safety, efficacy, and reduced trauma. Colonoscopy-assisted removal of IFPs less than 2 cm in size and within 10 cm proximal to the ileocecal valve is safe and effective.The ethics committee of our institution has issued ethical approval for this study.Records of colonoscopy cases in the Fourth Hospital of China Medical University between April 2016 and July 2020 that met the following criteria were reviewed: (1) Colonoscopy revealed protruding lesions in the terminal ileum; (2) the lesions were consistent with IFPs based on colonoscopic ultrasonography; (3) mucosal dissection and resection were conducted; and (4) postoperative pathological and immunohistochemical examinations were consistent with IFP. A total of 12 patients met the above conditions, and informed consent was obtained from all of them. Colonoscopic ultrasonography and colonoscopic minimally invasive resection were performed by experienced specialists in all cases.Routine blood tests, coagulation tests, blood gas tests, pulmonary function tests, Doppler echocardiography, and other preoperative examinations were carried out 24 h before surgery. Dimethicone was also orally administered 6 h before surgery after bowel preparation using polyethylene glycol electrolyte solution. Intravenous general anesthesia was used. Cap-assisted colonoscopy was progressed to the ileocecal junction using a distal attachment cap  attached to the distal end of the electronic colonoscope . The length of the endoscopy progression was recorded as the colonoscope continued to advance into the terminal ileum for at least 10 cm to observe the morphology, size, texture, and surface mucosa of the lesion.Using the degassed water congestion method, a miniature ultrasound probe  was used to scan the lesion at 2.0–3.0 cm from the lesion at an ultrasound frequency of 20 MHZ. The lesion’s size, origin, border, echogenicity, and infiltration were recorded.Racemic anisodamine hydrochloride was given intramuscularly before the procedure to reduce intestinal peristalsis. CO2 insufflation was used during colonoscopies, and normal saline containing methylene blue, Hemocoagulase Bothrops Atrox, and hyaluronic acid  was injected using an injection needle  to the submucosa under the lesion. The distal mucosa of the lesion was incised using a mucosa-cutting knife (AMH-EK-O-2.4X2300(4)-N, Anrui Medical, China), creating the submucosal space, gradually separating the tumor and the muscularis propria, and expanding the incision to both sides of the lesion until the tumor was fully exposed. During the operation, pre-hemostasis and hemostatic treatment were performed on submucosal vessels using thermal forceps . A mucosa-cutting knife was used to maximize the separation of the tumor and the muscularis propria, and a snare  was then applied to remove the lesion whenever it was difficult to dissect the tumor completely. The ERBE VIO 200D generator  was selected for the surgery. After surgery, hemostatic clamps  were routinely applied to close the wound. For difficulties in suturing the wound surface or suspected muscular layer wounds, a ligation device , nylon suture , and hemostatic clip were used for purse-string suturing , and Ki-67. The diagnosis was based on the second edition of “Biopsy Interpretation of the Gastrointestinal Tract Mucosa” by Elizabeth A. Montgomery.The patients were prescribed postoperative fasting, absolute bed rest, acid suppression, fluid replacement, and hemorrhage prevention treatment. A routine blood test was performed on the second day after the operation. Soft food without residue was permitted after 48–72 h, with a gradual transition to a regular diet. Fasting time was extended for patients who had intraoperative injuries or large wound surfaces. Colonoscopy was repeated 1 month, 3 months, and 1 year after surgery to assess wound healing and check for residual hemostatic clips or recurrence.All methods were carried out in accordance with the Declaration of Helsinki and the regulations of our hospital.All experimental protocols were approved by the IRB of the Fourth Affiliated Hospital of China Medical University.Informed consent was obtained from all patients who agreed to publish this article.Supplementary Legends.Supplementary Video 1.


Spontaneous and isolated dissection of the left gastric artery is a rare occurrence, with only a handful of cases reported in the medical literature. Clinical presentation may mimic more common intra-abdominal pathologies; however, it is imperative to identify this condition promptly due to its potential serious consequences. This underscores the importance of maintaining a high level of clinical suspicion and including this pathology in the differential diagnosis of patients presenting with acute abdominal symptoms. Hence, this case report aims to increase awareness among clinicians about the importance of identifying and treating this rare condition promptly. A 69-year-old female experienced severe epigastric pain while attending a yoga class, prompting her admission to the emergency department 24 h later due to the persistence of her symptoms. Following imaging work-up utilizing computed tomography angiography (CTA), she was diagnosed with a dissection of the left gastric artery. Notably, there was no associated aneurysm or any evidence of ischemia in the esophageal or gastric wall. Conservative management, including low-dose aspirin and blood pressure control, was implemented. After 6 months of follow-up, CTA demonstrated expansion of the true lumen and the absence of secondary aneurysm formation, leading to discontinuation of aspirin. The management of spontaneous dissection of visceral arteries is primarily determined by the presence of complications and organ ischemia. In the case of uncomplicated visceral artery dissections, first-line treatment comprises surveillance and antiaggregation. Nevertheless, the optimal duration of antiplatelet therapy and the necessity for long-term follow-up remain unclear. Endovascular or surgical interventions should be reserved for patients exhibiting deteriorating symptoms or complications, and the decision to pursue these interventions should be made on a case-by-case basis. Spontaneous, isolated acute dissection of a splanchnic artery can present with diffuse or localized acute abdominal pain, as well as other less specific symptoms, mimicking other more common intra-abdominal pathologies. Isolated acute dissection of an intra-abdominal splanchnic artery is a very uncommon cause of acute abdominal pain. Yoshimi and al. reported an incidence rate of one per 1,214 abdominal CT scans, and rate of one per 462 in emergency abdominal CT scans  and had persisted for the past 24 h. On admission, she reported experiencing epigastric pain and nausea but had not vomited. Her medical history was notable for arterial hypertension and a laparoscopic cholecystectomy that had occurred one year prior. Physical examination revealed upper abdominal tenderness and abdominal guarding upon deep palpation. While vital parameters were within the normal range, blood pressure was elevated at 186/114 mmHg with a normal heart rate of 72 bpm. Laboratory tests indicated slightly elevated CRP levels at 7.4 mg/L  with normal total leucocyte count. Blood pressure was treated with a short-acting calcium antagonist per os. An abdominal ultrasound scan showed no abnormalities, but due to persistent abdominal pain, a computed tomography angiography (CTA) was ordered to rule out mesenteric ischemia. Imaging revealed patency of the celiac trunk and both mesenteric arteries, along with an aberrant left hepatic artery (LHA) originating from the distal part of the LGA. The arterial wall of the LGA from its origin up to the aberrant LHA was thickened, with partial thrombosis of the lumen, indicating isolated arterial dissection. No associated aneurysm or signs of organ malperfusion were observed .Based on these findings, conservative management was proposed, consisting of blood pressure control and close monitoring. The patient had no postprandial pain and was advised to eat normally as tolerated. Single antiplatelet therapy with aspirin at a dosage of 100 mg daily was initiated, and the patient was followed in an outpatient setting. Symptoms gradually improved, and the patient became asymptomatic after 7 days. A follow-up CTA at 6 months showed expansion of the true lumen of the LGA, and no secondary aneurysm formation or imaging signs in favor of fibromuscular dysplasia in other vascular beds were observed . Based o3.Isolated splanchnic artery dissections represent an uncommon cause of acute abdominal pain. Most of them involve the celiac trunk or the superior mesenteric artery (SMA), and in the absence of organ malperfusion, conservative treatment with either antiplatelet agents or therapeutic anticoagulation is associated with favorable outcomes and is a safe first-line treatment , 4.The few cases of isolated dissections of smaller splanchnic arteries, without involvement of the celiac or superior mesenteric artery reported in the literature are summarized in Kwon et al.  reportedTago et al.  reportedSantos et al.  reportedIn three further reports, the dissection was associated with an aneurysm and embolization was performed in two cases, for persistent symptoms. Ichiba et al.  describeLim et al.  reportedD'Souza et al.  reportedIn our case, the patient reported a sudden pain during a yoga course while doing a “bridge stretch” (reclined backbend), with gradual pain decrease over the next days. The decision to conservatively treat and closely follow-up this patient was based on the absence of an associated aneurysm, thromboembolic or ischemic complications, or coexisting aortic pathology and on the relatively mild clinical presentation with symptoms that showed improvement after the acute episode.The etiology of isolated LGA dissection remains unclear. We did not find any other imaging signs of fibromuscular dysplasia in the CTA, which would justify long term antiplatelet therapy . The mosOverall, anticoagulation or antiplatelet agent and follow-up imaging can be proposed as first line treatment for uncomplicated dissections. The choice between anticoagulation or antiplatelet agents should be based on an individual basis by assessing hemorrhagic risks. With moderate to high bleeding risk, an antiplatelet agent should be preferred over anticoagulation. Endovascular treatment, when feasible, is indicated in case of persistent symptoms, progression of dissection, eminent luminal thrombosis or increasing aneurysmal dilatation, with open surgery with as the last resort in cases of complications or failure of endovascular treatment , 12 Fig.4.The patient therefore benefited from a simple, non-invasive treatment and outpatient follow-up, with rapid pain relief, which she particularly appreciated.Spontaneous isolated LGA dissection is a rare variant of isolated visceral artery dissections and without ischemic or local arterial complications can safely be managed conservatively with antiplatelet therapy or anticoagulation, blood pressure control and clinical surveillance. However, there is no consensus on the optimal frequency and duration of follow-up imaging and medical therapy. Initial surveillance is recommended to monitor for disease progression, which can lead to late complications. In cases where patients experience instability or worsening symptoms despite aggressive medical management, endovascular intervention should be considered. Open surgery is reserved for those patients with peritonitis or failed endovascular treatment.


Composite haemangioendothelioma is a rare vascular neoplasm with indolent to intermediate malignant potential. Diagnosis of this disease relays on histopathological identification of at least two different morphologically distinctive vascular components in proper clinical settings. Exceedingly rare cases of this neoplasm can exhibit areas resembling high-grade angiosarcoma, which does not change the biological behaviour. Such lesions tend to occur in the setting of chronic lymphoedema and thus, can mimic Stewart-Treves syndrome, which has a much worse clinical outcome and prognosis.We present a case of 49 years old male suffering from chronic lymphoedema of the left lower extremity who had developed a composite haemangioendothelioma with high grade angiosarcoma-like areas mimicking the Stewart-Treves syndrome. Given the multifocality of the disease, the only potentially curable surgical treatment would be hemipelvectomy, which was refused by the patient. The patient has been followed-up, with no signs of local progression of the remaining disease, nor a distant spread outside the involved extremity for two years.Composite haemangioendothelioma represents a rare malignant vascular tumour, with significantly more favourable biological behaviour than angiosarcoma, even in cases where angiosarcoma-like areas are present. For that reason, composite haemangioendothelioma can be easily misdiagnosed as true angiosarcoma. The rarity of this disease unfortunately hampers the development of clinical practice guidelines and the implementation of treatment recommendations. Most of the patients with localized tumour are treated by wide surgical resection, without neo- or adjuvant radiotherapy or chemotherapy. However, in the case of this diagnosis, the watch-and-wait approach is better than mutilating procedure, highlighting the necessity of establishing of the correct diagnosis. Composite haemangioendothelioma (CHE) is a rare vascular neoplasm, currently described as a borderline tumour or a tumour with intermediate malignant potential. This description reflects large uncertainty in its clinical behaviour and patient prognosis. Local aggressiveness and recurrence are well documented, but metastatic behaviour has been rarely reported –3. The tDiagnosis of CHE relays on histopathological findings. Minimal diagnostic criteria for CHE include the presence of at least two morphologically different vascular components of the tumour . VariablApproximately half of the cases of CHE contain areas resembling angiosarcoma, which is usually well-differentiated , 2, 16. Chronic lymphoedema is well-known risk factor for the development of several vascular neoplasms including HG angiosarcoma . The occWe present a case of 49 years old Caucasian male suffering from congenital lymphoedema of the left lower extremity who developed composite haemangioendothelioma during his adulthood. Because of the lymphoedema, the patient was first operated on at the age of 6 years, which had only a minor effect on overall condition of the patient. Later on in his life, he experienced multiple erysipelas and at the age of 47 a vascular tumour was detected in the setting of the lymphoedema as its complication. In another institution, a microscopically non-radical (R1) resection was performed and a diagnosis of angiosarcoma was concluded based on the histopathological evaluation of the tumour. The patient was not indicated for any adjuvant therapy, or re-resection. He was followed up and the first control PET/CT scan showed neither local relapse nor distant spread. Local progression was detected after one year of follow up and the patient was re-operated. Second R1 resection was performed and subsequent histopathological examination revealed an angiosarcoma again. This diagnosis was supported by an early second local relapse after a month. After that, the patient was reported to our sarcoma centre for further treatment.During the clinical investigation, two purple skin affections in the popliteal area at the edges of the dermo-epidermal mesh skin graft were found Fig. . The lefTo evaluate the uncertain undergoing process, the next multidisciplinary meeting led to an indication for excision of the recurrent focus and bioptic evaluation of the nodules of the calf and gluteal area. The operation was performed in February 2022 in form of an excision of two skin affections of the popliteal area (diameter 43 and 78 mm) and another biopsy of the tumour in the calf and gluteal area (diameter 20 mm and 10 mm). Histopathological examinations showed a new finding  and significantly higher proliferation 40–50%) based on the Ki-67 proliferation marker. Such a finding was consistent with the original diagnosis of HG angiosarcoma .Recently it has been discovered that CHE can harbour a fusion gene e tumour . We perfYAP1::MAML2. However this finding does not exclude the diagnosis of CHE as for such gene fusion usually does not occur among adult patients outside acral localisations [MYC amplification using fluorescent in situ hybridisation (FISH) as there is an evidence in literature that high-level MYC gene amplifications (at 8q24.21) occur in majority of post-irradiation and chronic lymphoedema-associated angiosarcomas [MYC amplification was not found in this case, which further supports the diagnosis of CHE favouring it over angiosarcoma.In conclusion no gene fusion was identified, including isations . Moreovesarcomas . HoweverEven though CHE usually shows indolent behaviour, the multifocality of the process in the setting of chronic lymphoedema allowed radical surgical treatment, which would have to be hemipelvectomy in this case. Such a procedure was rejected by the patient. Therefore, the current course of action is the follow up with periodical PET/MR scans. In case of local progression of any nodule, the patient would be indicated for the extirpation of the lesion. However, no growth of the remaining lesions was detected in the follow-up so far and there has been no metastatic spread of the disease since the first detection of the tumour two years ago.Stewart-Treves syndrome first described by Drs. Steward and Treves, represents a development of (lymph)angiosarcoma in the terrain of chronic lymphoedema, usually after mastectomy . NeverthCHE is an extremely rare histological entity, with a potentially fatal impact on patient. It has an intermediate malignant potential and therefore it can lead to local recurrences, loco-regional or rarely to distant spread . PredomiMYC amplification, which occurs in majority of post-irradiation and chronic lymphoedema-associated angiosarcomas [In this case report, the final diagnosis of CHE with HG angiosarcoma-like areas was established based on histopathological analysis of three different vascular lesions resembling PILA, retiform haemangioendothelioma and HG angiosarcoma. The epitheloid haemangioendothelioma, PILA and retiform haemangioendothelioma are described as the most common histopathological components of CHE in the literature , 4. Clinsarcomas , supportThe most important feature distinguishing between CHE with HG angiosarcoma-like areas and the Stewart-Treves syndrome is the biological behaviour of the tumour . During Currently, we are lacking the standards of care for patients with this disease. Most of the published cases describe angiosarcomas in general or angiosarcomas as a part of Stewart-Treves syndrome. We were unable to find any suggested treatment regimens for CHE with HG angiosarcoma-like areas developed in the setting of chronic lymphoedema. Most patients with localized disease, in previously published articles, were treated by surgery alone. The disease itself shows potentially favourable biological behaviour compared to conventional angiosarcoma . HoweverThe high rate of local recurrence is an argument for multimodal therapy. The systemic treatment used in angiosarcomas includes regimes based on anthracyclines , 29. TaxIn conclusion, composite haemangioendothelioma represents a rare malignant vascular tumour, with significantly more favourable biological behaviour than angiosarcoma. If a radical treatment would require mutilating procedure and the disease is stable, we propose a watch-and-wait approach.