Spleen Spontaneous Rupture SRS is a rare phenomenon in which the spleen ruptures without associated trauma. This pathology is rarely caused by Chronic Lymphocytic Leukemia.We present a case of a 59-year-old male patient with chronic Lymphocytic Leukemia, who was admitted with an acute abdomen whose clinical and paraclinical examinations revealed a spleen spontaneous rupture. The treatment consisted of a splenectomy.Spontaneous spleen rupture was first described by Rokitansky in 1861 and mentioned in many cases since, the common causes of non-traumatic Splenic rupture include myeloproliferative diseases, vasculitis, and infections. However, Chronic Lymphocytic Leukemia (CLL) remains an obscure cause of splenic rupture that requires unique attention. The diagnosis of splenic rupture should be considered in all patients with hematologic malignancies presenting with abrupt onset of abdominal pain, hemodynamic instability, or acute anemia. The choice between conservative treatment and splenectomy depends on different variables: the etiology of the SRS, the hemodynamic stability, the amount of packed red blood cells transfused. Thus, an interventional approach can be advocated for a spontaneous splenic rupture over nonoperative management. Splenic embolization can provide patients with the advantages of both operative splenectomy and conservative management. The mortality rate from SRS is 12.2 %. Neoplastic pathologies were most significantly associated with fatal outcomes.The high mortality rate seems to be mainly related to the delayed diagnosis and/or the severity of the underlying pathology. Given its seriousness, it requires a rapid diagnosis and adapted management. •Spontaneous rupture of the spleen is a rare phenomenon where the spleen ruptures without associated trauma.•chronic lymphocytic leukemia (CLL) remains an obscure cause of splenic rupture that requires unique attention•Clinical presentation is usually in the form of a severe abdominal syndrom with hemodynamic instability.•The choice of treatment: conservative, surgery or embolization depends on several factors.•The high mortality rate is related to delayed diagnosis and the severity of the underlying pathology. It is an unusual complication of malignant hematological disorders. According to the literature only few incidences of spontaneous splenic rupture are caused by chronic lymphocytic leukemia Non-traumatic splenic rupture is due to many causes, including myeloproliferative diseases, vasculitis, and infections . However, chronic lymphocytic leukemia (CLL) remains an obscure cause of splenic rupture that requires special attention We present here the case of a 59-year-old male patient with a chronic lymphocytic leukemia history, who got admitted with subcapsular hematoma of the spleen, the surgical intervention consisted of splenectomy and abdominal drainage.In this article, we represent the clinical manifestations, evaluation, management, and prognosis of this type of diseases. This work has been reported in line with the SCARE criteria 2020 2We present the case of a 59-year-old male patient, followed in the onco-hematology department for chronic lymphoid leukemia stage C with negative deletion 17p. A year before his admission, he had benefited from 6 cures during FCR chemotherapy protocol, he never showed up again till he was consulted to the emergency department for acute onset diffuse abdominal pain.The biological parameters had demonstrated a hyperleukocytosis 13,000 predominantly lymphocytes predominance of 60 %. Microcytic hypochromic anemia at 8.3 g/dl, thrombocytopenia at 98,000 platelets/mm3. Liver and kidney tests came normal and CRP at 23.The abdominal and pelvic CT scan showed Splenomegaly with subcapsular hematoma of the spleen measuring 30 mm in diameter, with an intraperitoneal effusion at the level of the rectouterine pouch, as well as the lumboaortic and coeliomesenteric nodes .Fig. 1CTAfter multidisciplinary discussion involving visceral surgeons, anesthesiologists, and onco-hematologists, the decision was to proceed with an emergency laparotomy due to a pre-rupture hematoma on a pathological spleen under general anesthesia with endotracheal intubation, the surgical exploration revealed the presence of a giant spleen exceeding the umbilicus below and reaching the midline on the right of multiple intraperitoneal lymphadenopathies and an abdominal effusion of moderate abundance.The surgical intervention consisted of splenectomy with abdominal drainage, During the procedure, the patient required a transfusion of 2 units of red blood cells. Postoperatively, the patient had febrile neutropenia complicated by septic shock transferred to intensive care where he received empirical broad-spectrum antibiotic therapy based on 3rd generation cephalosporin in combination with metronidazole and an aminoglycoside then escalation to piperacillin tazobactam following The MASCC clinical score  and died on the sixth postoperative day. The Macroscopic examination revealed a spleen that weighed 3150 g and is the site of several foci of hemorrhagic infarctions and recent hematomas , Fig. 3.3spontaneous spleen rupture (SRS) was first described by Rokitansky in 1861 and mentioned in many cases since Renzulli and all summarized 845 patients with atraumatic splenic rupture from previous reports from 1980 to 2008, the analysis revealed that the hematologic cause accounted for 30.3 % of the cases, followed by infection in 27.3 % of the cases, inflammation in 20.0 % of the cases, and drug-related causes (anticoagulants and thrombolytics) in 9.2 % of the cases In another review by Giagounidis and all analyzing 136 cases of SRS due to hematological diseases, chronic Lymphocytic Leukemia (CLL) was described in 5.8 %, with only 8 cases of SRS The mechanism of occurrence of spontaneous rupture of the spleen is not specific, Hynes and all. Suggested three mechanisms: mechanical effect of leukemic infiltration in the spleen, especially if the capsule is invaded, splenic infarction with subsequent subcapsular hemorrhage and subsequent rupture of the splenic capsule due to blood coagulation abnormalities. Regarding SRS in chronic lymphocytic leukemia, the incidence is not known, the pathogenesis is still poorly understood, and the mechanisms of leukemic infiltration of coagulopathy and infarction are incriminated Clinical presentation is usually in the form of a severe abdominal syndrome, which may be accompanied by nonspecific symptoms simulating acute coronary syndrome or acute pancreatitis which delays the diagnosis and the treatment In most cases, the diagnosis of a splenic rupture or hematoma is made with an improved imaging scan. The results can be obtained quickly which helps diagnose and guide the surgical planning by determining the presence and abondance of intraperitoneal hemorrhage and can sometimes provide information on the etiology of SRS .The choice of the treatment depends on different variables: the etiology of SRS, hemodynamic stability, the amount of red blood cells transfused, the operability of the patient, the amount of hemoperitoneum as well as the lesion grades according to the World Society for Surgery emergency (WSES) Examinations of survival associated with spontaneous splenic rupture reported in the literature, 63 % of splenectomies survived Some investigators use these data to make such aggressive claims that emergency splenectomy is the only possible treatment for these patients Although successful conservative management has been reported, the mortality rate is still high for this strategy Thus, an interventional approach can be advocated for a spontaneous splenic rupture over nonoperative management. Splenic embolization can provide patients with the advantages of both operative splenectomy and conservative management. Decreasing the blood flow may reduce the risk of delayed splenic rupture and preservation of functional splenic tissue which minimizes the risk of post-splenectomy infection The study by Renzulli and all calculated the overall mortality rate from SRS to be 12.2 % and it suggests that the major risk factors for death are: splenomegaly and the age over 40 years The examination of the six major etiologic groups revealed that neoplastic disorders were also significantly associated with a fatal outcome. A traumatic rupture of a normal spleen without etiologic factors (atraumatic-idiopathic splenic rupture). On the other hand, it was associated with a significant decrease in SRS mortality 4The pathological splenic rupture, although rare, must be considered in case of acute abdomen in patients with hemato-oncological pathologies. The high mortality rate seems to be mainly related to delayed diagnosis and/or the severity of the underlying pathology. Given its seriousness, it requires a rapid diagnosis and an adapted management.The author(s) received no financial support for the research, authorship and/or publication of this article.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.1.Name of the registry:2.Unique identifying number or registration ID:3.Hyperlink to your specific registration (must be publicly accessible and will be checked):Dr. Madani Ayoub▪Dr. Madani Ayoub: Have written the article, have consulted the patient, prescribed all of tests and prepared the patient for surgery and participated in the surgery-▪Dr. Hajar Abdelouahab Pr Imane Kamaoui: Interpretation of Radiological data.Dr. Mohamed Yassine Mabrouk: have helped writing the article, data collection.-Dr. Miry Achraf: Interpretation of histological data.▪: management and follow up of th patient▪Pr Jabi Rachid: supervised the writing of manuscript.-Pr Bouziane Mohamed: (oncology surgery professor): have supervised the writing of the paper, and has been the leader surgeon of the case.Pr Siham Hamaz, Pr Khalid Serraj SrNo conflit of interest.


Diagnosis of venous aneurysm may be difficult and can be misjudged as mass-like lesions, including hernias. Here we present the case of a patient with a great saphenous vein aneurysm misdiagnosed and operated as an inguinal hernia.A 39-year-old Middle Eastern/Persian male presented with left inguinal bulging 15 years ago, which was misdiagnosed and operated on with a diagnosis of inguinal hernia. He was referred to our clinic, in which color Doppler sonography revealed left-sided saphenofemoral junction incompetence with severe flow reversal during the Valsalva maneuver, in favor of a great saphenous vein aneurysm. Ligation of left saphenofemoral junction and stripping of saphenous vein, and stab avulsion phlebotomy of left lower extremity varicose veins, were done. He was discharged the next day after the operation with an uneventful postoperative course or complication on follow-up.Venous aneurysms can be misdiagnosed as other, more common, mass-like lesions, such as inguinal hernias. Therefore, our report emphasizes the consideration of thorough assessment and utilization of color duplex sonography to prevent further misdiagnosis and unnecessary intervention and operations. A venous aneurysm is defined as a localized dilatation of a vein that connects to the main vein structure through a single channel. Venous aneurysms are unusual vascular pathologies that often appear as asymptomatic and incidental findings on imaging studies or physical examinations . Yet, thA 39-year-old Middle Eastern/Persian male with a known case of left lower extremity varicose vein was referred to our vascular surgery service with a chief complaint of left inguinal bulging since 15 years ago. He was asymptomatic and reported no pain or restrictions in movement or other clinical findings aside from the bulging, which resolved under local pressure. The patient had been misdiagnosed and operated on 10 years ago with the impression of a left inguinal hernia; however, no hernia sac was detected at that time.Five years later, the patient developed left lower extremity varicose veins. On physical examination, severe varicose veins of the left lower extremity with a soft, nonpulsatile, reducible inguinal bulging were detected that became more prominent by performing the Valsalva maneuver Fig. .Fig. 1A On the basis of the patient features, color Doppler sonography of left lower extremity venous system was performed, which revealed left-sided saphenofemoral junction incompetence with severe flow reversal during the Valsalva maneuver Fig. .The patient was scheduled for surgery in which ligation of the left saphenofemoral junction and stripping of the saphenous vein, and stab avulsion phlebotomy of the left lower extremity varicose veins, were done. In the course of surgery, a 5 × 2 cm venous aneurysm of the great saphenous vein with about 2 cm distance from the saphenofemoral junction was detected. Fig. . No compVenous aneurysms are dilatations of veins with single communication to the main venous system without any association with pseudoaneurysm or arteriovenous fistula . They arPrevious studies documented the presence of venous aneurysms in different parts of the venous system, such as portal, superior vena cava, intracranial, popliteal, splenic, and great saphenous vein, among which the most common site in the lower extremity is the popliteal vein, followed by the saphenous vein. The distribution of venous aneurysms is the same in both sexes and can occur in any age group. The exact etiology of venous aneurysms is not clearly defined, although trauma, weakness of the venous wall due to a congenital defect in connective tissue structure, degenerative changes, and inflammation can be the basis of aneurysm formation , 5.et al.: type I, which is the most common type (52%), is located in the proximal third of the saphenous vein, not at the saphenofemoral junction but instead just distal to the subterminal valve; type II venous aneurysms are located in the distal third of the thigh in the shaft of the saphenous vein; type III is the coexistence of types I and II in the same extremity; type IV is an aneurysm of the superficial small saphenous vein [There are four types of venous aneurysm proposed by Pascarella ous vein .The differential diagnosis of a mass-like lesion or bulging on the lower extremities with the indication of surgery has a broad spectrum, and misdiagnosis may lead to complications and difficulties for the patient and the surgeon. Nowadays, the noninvasive diagnostic method is color Doppler sonography. Venous disorders can be easily distinguished from other conditions using a color Doppler examination. The diagnosis may also be aided by ultrasonography or venography. Nevertheless, the venographic diagnosis may be hampered by the presence of thrombi within the venous aneurysmal sac. To detect thrombus within the aneurysmal sac and measure the aneurysm's diameter, venous color Doppler ultrasonography is superior to venography .There are several surgical techniques, including ligation, simple excision, excision and vein patching, tangential excision with lateral venography, and complete resection, for the management of venous aneurysms. Endovenous laser, endovenous radiofrequency, and sclerotherapy can be used as an alternative therapy in selected cases , 8.We reported a case of a venous aneurysm that was resected during stripping of the great saphenous vein. A venous aneurysm can be misdiagnosed as other, more common, mass-like lesions, such as inguinal hernias. This case was misdiagnosed with an inguinal hernia and operated once with the impression of an inguinal hernia; thus, our report emphasizes the consideration of thorough assessment and utilization of color duplex sonography to prevent further misdiagnosis and unnecessary intervention and operations.


Dentigerous cysts (DCs) are one of the most common cysts in the oral and maxillofacial region, and they are often discovered by chance in young people. The methods of treatment differ according to the size they reach, but the prognosis and results of the treatment are generally good. DCs are often associated with impacted teeth, especially mandibular third molars and maxillary canines, and they are usually discovered when they reach large sizes or get infected after they have caused great absorption and destruction of the surrounding cortical bone and displacement in adjacent teeth.This case report expresses the importance of conservative treatment of large oral cysts  in the preservation of jaw bones, in a young female child of 12 years, with a DC associated with an impacted second permanent lower molar. This cyst occupied nearly half of the mandible with the danger of causing more harm to the jaw. In brief, marsupialization is a very effective method of treatment for DCs, especially those that reach very large volumes. A dentigerous cyst is an extension of the dental follicle , more thWhen DCs reach large volumes in size, they cause eggshell crackling on clinical examination, palpation, and root resorption, but neurosensory deficits are not common . FrequenDentigerous cysts appear radiographically as a radiolucent lesion, round or ovoid, with well-defined borders around the crowns of unerupted teeth . The difThe principles of treatment of dentigerous cysts are enucleation (excision), marsupialization ,7, and dMarsupialization means the conversion of the cyst into a pouch  by makinConsequently, the jaw bone starts to remodel over a long period of time, causing a reduction in cystic volume to the extent of permitting it to enucleate or to leave it to heal completely ,12.This article was previously presented as an abstract on the Authorea website (10.22541/au.165494343.36193429/v10).A female child, 12 years of age, came to the oral and maxillofacial surgery department in the faculty of dentistry at Damascus University with an enlargement on the left side of the face.The clinical examination is conducted for the patient and appears a tough enlargement on the left side of the face, extending from the body of the mandible to almost the ramus with limitations in mouth opening Figure .The intraoral examination showed the presence of a large tough enlargement in the lower left buccal vestibule, the absence of a lower left permanent second molar with obvious movement in the lower left permanent first molar, and the premolars with the absence of carious lesions on teeth or any inflammatory appearance on the covered gingiva.On aspiration through the cyst wall, a clear yellow fluid is visible.Orthopantomogram (OPG) and cone beam conventional tomography (CBCT) show the presence of a large radiolucent cystic lesion with radiopaque borders involving a large volume of the mandible and extending from the lower molar to include the entire volume of the mandible and extending from the lower left canine to take the entire volume of the body of the mandible and ramus toward the coronoid and condylar processes, with radiopaque borders and an impacted second molar in the cyst Figure .Sections of CBCT confirmed the extreme thinning and extension of the buccal and lingual plates of the mandible from the left side Figures -4.After studying the case, it was decided to do the surgery under general anesthesia, and the selected treatment was marsupialization.Surgical stepsThe patient was prepared for the surgery after conducting all necessary consultations. First, exposure and disinfection of the surgical field clarified the disappearance of the second permanent molar. Then, the elevation of the full mucoperiosteal flap showed the extreme thinning of the buccal mandibular plate Figure . After tA surgical biopsy was done and the result of the pathological examination confirmed the diagnosis (dentigerous cyst). The microscopic view shows the wall of the dentigerous cyst lined by thin epithelial layers of undifferentiated cells. The fibrous cyst wall was relatively inflamed Figure .An OPG after one week of surgery shows rapid formation of radiopacity and decrease in gauze volume during gauze replacement clinically Figure .Follow-up of the case showed rapid reduction in cystic volume and formation of bone in a very short period of observation. Figures This case is characterized by the fact that the cyst reaches a large size that nearly occupies half of the mandible and causes extreme thinning in both buccal and lingual bony plates, and in rare cases, lesions like this are neglected to reach that volume. So marsupialization helped in preserving the safety of the mandible and inferior alveolar neurovascular bundle and saved the child from a more aggressive surgery to treat the lesion. Some practitioners advocate the use of the decompression method, which means making a small window in the cystic wall  and then using a tube (or stent) to enter the cyst through that hole . While oSo, because of the large size of the cyst in this case and because it is containing important structures, such as the inferior alveolar nerve and its vascular bundle and the proximity of the cystic borders to the condyle, the most appropriate treatment was marsupialization. Despite the long period of observation and treatment, the result is considered perfect according to the large extension of the cyst, which may be attributed to the skill of the surgical team, continuous weekly follow-up for the patient, and the high capacity for remodeling in children.In addition, the importance of histological diagnosis, supported by clinical and radiological diagnoses, must be emphasized, as it contributes to determining the correct treatment plan.In this case, two biopsies were sent to different laboratories. The first was sent to an external laboratory but the result of the first biopsy was completely different from the differential diagnosis of the medical team, in that it was claimed to be fibrous dysplasia Figure  because Cysts are common lesions that are frequently found in our practice in oral and maxillofacial surgery. The features in this case  lead us to choose this approach of treatment . Finally, referring to previous literature led to this treatment result.


Desmoid fibromatosis is a rare benign tumor, but due to its rarity and diverse clinical course, treatment guidelines have not been established. However, since a good prognosis can be expected, an accurate diagnosis and appropriate treatment are required. We describe a rare case of desmoid fibromatosis on young female that presented as huge abdominal mass.A 28-year-old female with left upper abdominal pain 1 month ago was referred.Abdominal computed tomography and magnetic resonance imaging revealed a heterogeneous soft tissue mass approximately 29 × 17 cm in size in the left abdomen with abdominal wall invasion and pathological fracture in costochondral junction of the left 8th to 10th ribs.Surgical resection was performed.33 × 23 × 6 cm sized tumorous mass showed proliferation of bland fibromatosis and myofibroblast with nuclear β-catenin expression on pathological examination. Desmoid fibromatosis arising from intra-abdominal soft tissue with ribs and pericardium invasion was diagnosed.The mainstay of treatment of symptomatic desmoid fibromatosis is surgical resection, and in the case of abdominal tumor, it can be more dangerous when it invades adjacent organ. We report a case that required additionally multidisciplinary approach for surgery and postoperative treatment of huge abdominal desmoid tumor which infiltrate bone and pericardium beyond abdominal cavity. It has an incidence of 0.03% of all neoplasms and <3% of all soft tissue tumors. Despite of its nature as benign tumor, desmoid tumor can develop as a result of progressive and aggressive behavior. Therefore, clinical management of patients with desmoid tumor is difficult for clinician. Clinical suspicion, early detection and curative resection of surgical method is important for treatment of desmoid tumor. Herein, we report a case of desmoid tumor which invaded to ribs and pericardium in young female with huge abdominal mass.Desmoid fibromatosis, also known as desmoid tumor, is a benign, uncommon neoplasm but frequently aggressive tumor of originated from myelofibroblast.6/μL; hemoglobin, 120 g/dL; hematocrit, 39.4%; platelets, 278 × 106/μL; C-reactive protein, 0.3 mg/L; lactate dehydrogenase, 134 IU/L). Contrast-enhanced abdominal computed tomography (CT) was showed 29 × 17 cm sized heterogeneous soft tissue mass in left abdomen. It was accompanied with left anterior abdominal wall invasion with pathological fractures in costochondral junction of left 8th to 10th ribs . Under US, huge mass was found. On admission to our hospital, she had palpable mass on left side abdomen. She had no specific past medical history or obstetric history. On physical examination, a huge, fixed and hard mass with tenderness was palpable throughout the left side whole abdomen. Blood tests showed unremarkable results  was performed. On chest, abdominal CT and PET-CT, there was no pathological findings for remained tumor. Adjuvant radiation therapy was planned. The patient has provided informed consent for publication of the case. The study was approved by the Institutional Review Board of the Chung-Ang University Hospital (No. 2022-0088). It is classified as benign neoplasms due to their histopathological features and nonmetastatic properties, but show invasive growth and local infiltration. Desmoid fibromatosis occur between the age of 15–60 years, and more commonly observed in women than in men (2:1–5:1). Depending on the tumor site, divided into abdominal or extra-abdominal disease, and the abdominal desmoid fibromatosis is the most common type, which is associated with familial adenomatous polyposis or abdominal surgery.Desmoid fibromatosis is uncommon neoplasm comprised of fibroblastic proliferations that arise in the deep soft tissues.Although desmoid fibromatosis has low morbidity rate, can have potential for mortality due to their ability to invade adjacent critical organs and structures. Thus, desmoid fibromatosis shows various clinical course according to the tumor growth pattern, size, location, and organ invasion. In case of abdominal desmoids, surgery can be much more dangerous than nonabdominal desmoids, due to hemorrhagic complications or extensive enterectomy.Because of these features of “benign malignancy,” management is difficult and many issues remain controversial. Traditionally, marginal negative resection was the standard of the treatment, but it has not proven an effect of microscopic margins on recurrence. Efforts for marginal negative can lead to unnecessary morbidity and do not reliably prevent local recurrence. Thus, the mainstay of surgical therapy should be focused on preserving function and structure while keeping the resection margin as clear as possible.In our case, patient had a huge tumor size accompanied by organ invasion including ribs and pericardium. Among intra-abdominal desmoid fibromatosis, vigorous invasion to the adjacent organs is rare, but there are cases reported Table . We foun Church et al have proposed a staging system for intra-abdominal or trans-abdominal desmoid fibromatosis, and Latchford et al suggested therapeutic algorithm, but there are no consensus guidelines yet.,17 Appropriate treatment can be varied from just “wait and see” to radical resection or systemic therapy, so patients with desmoid fibromatosis should be managed with multidisciplinary approach.Depending on the stage of the disease, various therapeutic options such as NSAIDs, antihormonal therapies, cytotoxic agents, targeted agents have been investigated. Although most relapses occur within 3 years, it is possible even years after treatment. Surgery is indicated for the management of recurrences, however further local recurrence is even higher in these patients because surgical trauma itself is the risk factor for recurrence. Therefore, close surveillance is necessary for a sufficient period.One of the difficult aspects of dealing with desmoid fibromatosis would be high recurrence rate. Recurrence rates following surgery of intra-abdominal desmoid fibromatosis have been reported ranged between 57% and 86% are higher than those reported for extra-abdominal tumors.


We describe a case of an uncommon early pancreatic cancer presentation in a patient in his 60s who had haemorrhagic shock from extensive haematochezia and required blood transfusions as well as surveillance in an intensive care unit. A splenic artery pseudoaneurysm that had been effectively embolized by angiography was seen to be actively bleeding into the colon lumen on a computerized tomography (CT) scan along with a necrotic mass of the pancreatic tail. A pancreatic mucinous adenocarcinoma was diagnosed by a transgastric biopsy. A pancreatico-colic fistula was discovered by CT scan after a colic contrast enema. A transabdominal drainage of the necrotic collection and targeted antibiotic treatment had been performed with a satisfying patient outcome. In order to assess a potential secondary surgical resection, systemic chemotherapy was planned. In conclusion, haematochezia with hemodynamic instability originated from a splenic artery pseudoaneurysm fistulising into the colon (arterio-colic fistula) and sepsis originating from a tumoral pancreatic abscess fistulising into the colon . Pancreatic ductal adenocarcinoma (PDAC) is the seventh leading cause of cancer-related mortality  and is aThe majority of tumors (about 70%) develop in the head of the pancreas and frequently present with biliary obstruction, which results in dark urine 49%), jaundice (49%), appetite loss (48%), fatigue (51%), weight loss (nearly 55%), and exocrine pancreatic insufficiency (25%) [9%, jaundThe majority of pancreatic malignancies (90%) are PDACs and their variations, with 60–70% of cases occurring in the head, 15% in the body, and 15% in the tail of the pancreas , 5. WhenTumor local invasion is based on its resectability and falls into three categories: resectable, borderline resectable, and unresectable. The latter are divided into two subcategories: locally advanced (LA) and metastatic.The tumor is considered LA if: the degree of contact or invasion of the superior mesenteric vein, portal vein, superior mesenteric artery, or celiac artery (CA) is equal to or exceeds 180°; the contact or invasion of the common hepatic artery shows contact or invasion of the proper hepatic artery and/or CA; and the aorta is in contact or invaded by the tumor .Therefore, given the close anatomical relationship between the tail of the pancreas and the blood vessels of the spleen, the latter is likely to be involved during the progression of pancreatic tumors of the body or tail of the pancreas  leading We report a case of pancreatic cancer presenting with haematochezia originating from a splenic artery pseudoaneurysm rupture into the colon lumen.2 93%, temperature 36.7°C, pain evaluation 0/10, and Body mass index (BMI) 38 kg/m2. Abdominal examination showed diffuse tenderness with no palpable mass or peritonitis. Patient has a personal history of coronary artery disease with a diagnosis of Non-ST-elevation myocardial infarction (NSTEMI) treated with coronary stents and aspirin, recurrent superficial venous thrombosis, diagnosed 1 year ago, treated with rivaroxaban for 3 months, metabolic syndrome with dyslipidemia, hypertension, type 2 insulin-dependent diabetes mellitus, obesity, and active smoking at 40 pack year. No family history was discovered. Blood tests showed a haemoglobin value of 121 g/L at first check and 78 g/L 12 hours later, justifying the transfusion of 4 units of blood and 1 plasma concentrate. Three phase computerized tomography (CT) scan showed a hypodense air-containing mass measuring 8 cm × 8 cm × 7 cm in the tail of the pancreas suggestive of a necrotic tumor lesion, with infiltration of the colon splenic flexure and fistula  was 15/15, heart rate was 105 beats/minute, blood pressure was 100/71 mmHg, respiratory rate 28 breaths/minute, SO fistula . An irreStaphylococcus epidermidis. After an unsuccessful endoscopic attempt to drain gastric contents due to thickened necrosis, CT-guided percutaneous drainage was performed with the insertion of a 10 F drainage catheter, upsized with a 20 F catheter. Antibiotic therapy was discontinued after 1 month as the collection was completely emptied and the patient's condition stabilized. A neoplastic pancreatic abscess fistulising into the colon (neoplastic pancreatico-colic fistula) was confirmed by CT imaging after positive contrast injection into the catheter (Figures Therapeutic angiography was performed, confirming the formation of a middle third splenic artery pseudoaneurysm without active bleeding on angiography . Emboliz Figures . On hospPatient outcome was subsequently favourable. An interdisciplinary consensus for evaluating future surgical and oncological plans advocates systemic neoadjuvant chemotherapy with nab-paclitaxel and gemcitabine as first-line treatment 3 months after initial hospitalization. Furthermore, evaluation will be done after 3 months to plan for the possibility of multi-organ resection, but hemodynamic instability due to bleeding, accumulation of infection, and LA neoplasms make it a non-first-line treatment. The patient was discharged in stable condition with drainage in place. At the start of chemotherapy treatment, as the disease progressed, the patient developed colic obstruction at the level of the tumor mass, which caused a compressive effect. A colostomy was performed because the colic dilation was 56 mm in the transverse and 95 mm in the cecum. The patient was newly admitted to the emergency department in the context of haemorrhagic shock, after two episodes of hematemesis. The CT scan showed the development of a fistula between the tumor and the stomach, thus presenting a gastro-pancreatic fistula with intraluminal arterial bleeding from the distal third of the transverse colon and from the left colic angle, with a possible source of bleeding from a branch of the left colic artery. Gastric fistula explains hematemesis. After discussion with the family and the patient, the decision to discontinue treatment and care for comfort was made. The patient died 6 months after the initial diagnosis.Massive haematochezia due to splenic artery pseudoaneurysm rupture, fistulised into the colon, is a potentially lethal condition, which requires prompt diagnosis and treatment and a potentially lethal and challenging condition . A massiThe most common evolution of pancreatic necrosis is abscess formation  generatiThe initial approach often requires multimodality imaging to properly depict the extent of the disease. Contrast multiphase abdominal CT can identify both active bleeding due to ruptured pseudoaneurysm and signs of a malignant fistula extending into the colon. It should be noted that positive contrast media should not be used as it will not reveal the passage of vascular contrast medium into the colon through the fistula. The precise localization of the source of bleeding can be achieved by selective angiography of the visceral arteries. Arteriography with selective embolization of the bleeding artery is the best treatment in emergency phase .In case of refractory sepsis, surgical colon resection or derivation may be mandatory.Endoscopic procedures may also take place in the treatment, even if with some limits. In case of a pancreatic collection with complete necrosis of the neck or body of the pancreas, endoscopic retrograde cholangiopancreatography could be difficult as the pancreatic gland integrity is rarely preserved and may lead to superinfection. Endoscopic sphincterotomy and placement of a short transpapillary stent may reduce intraluminal pressure and associated inflammation, promote healing, and repair ductal discontinuities . When thOther approaches may be considered in certain circumstances. If immediate surgical indication is not possible, as in the case shown here, the colic fistula can be closed with endoscopic clips , 22. LarThe timing of surgery is critical to achieve oncological R0 resection. Hemodynamic stability should be restored, and the septic condition should be treated first. Oncologic staging should evaluate the resectability, and treatment options should be discussed in a multidisciplinary team meetings. Neo-adjuvant chemotherapy may increase the rate of R0 resection in cases of borderline or LA PDAC –26.In conclusion, advanced pancreatic cancer with massive haematochezia can occur secondary to neoplastic pancreatico-colic fistula and ruptured splenic artery pseudoaneurysm, and requires prompt diagnosis and multimodal therapy. Treatment options must be weighed according to several factors, and the risk of morbidity and mortality remains high.


Fibrosing mediastinitis (FM) is considered a benign disease, but it can be fatal if progression leads to compression of the hilum of the lungs or invasion of the heart. Echocardiographic reports of this disease are very rare.We present a 14-year-old male patient whose non-enhanced chest computed tomography showed unclear soft-tissue dense lesions in the anterior superior mediastinum. Echocardiography showed the heart was extensively wrapped by soft tissue lesions. The histology confirmed FM.When FM affects the heart, echocardiography can help to characterize the disease and aid in the diagnosis. Echocardiography should be considered an important tool to follow the progression of this disease and guide the therapeutic approach.The online version contains supplementary material available at 10.1186/s12947-022-00289-y. Fibrosing mediastinitis (FM) is a rare and benign disease, but progression can potentially be fatal. It is characterized by compression and obstruction from hyperplastic fibrous tissue that encloses the hilar structure of the lungs, and often needs to be differentiated from malignancy . HistopaWe report a case of idiopathic FM with extensive cardiac involvement. We examined the patient using echocardiography and performed follow-up over 4 years. To our knowledge, this is the first description of the application of echocardiography in FM.A 14-year-old male patient presented with chest pain for 2 months and hemoptysis for 3 days. There was no clinically relevant medical history, and no family history of malignancies or autoimmune diseases. The patient showed no signs of infection and the physical examination results were normal. A non-enhanced chest computed tomography (CT) showed a soft tissue mass in the anterior superior mediastinum, but the structure was not clear. Laboratory tests showed the C-reactive protein (CRP) was slightly elevated  and the erythrocyte sedimentation rate (ESR) was within the normal range . There were no meaningful findings in the laboratory indicators of infectious diseases, tumors, or autoimmune diseases , lateral wall of the left atrium and left ventricular (4 × 3 cm), and the top of the left atrium (3 × 2 cm). The lesions were hard/brittle and bled easily upon touch. The same lesions were present in the transverse pericardial sinus and around the pulmonary arteries. The pathological examination revealed the lesions consisted of hyperplastic fibrous tissues with infiltration of lymphocytes and plasma cells, a blurry lesion boundary, and involvement of the surrounding adipose tissue, with a surrounding of small nerves and blood vessels. There was no evidence of granuloma or malignant cells, and the IgG4 staining test was negative. Fungal culture and acid-fast staining tests were also negative. After multidisciplinary discussion, we made a diagnosis of idiopathic FM based on the highly suggestive radiology, definitive pathological results, and the exclusion of other diagnoses.Glucocorticoid is a standard treatment for FM. Thus, we initially prescribed 6 prednisone tablets  per day. We initially reduced the dose by 1 tablet every 3 months; when the dose was 15 mg per day, we reduced the dose by 1 tablet every 6 months. Eventually, we maintained the dose at 1 tablet (5 mg) per day. During the 4 years of follow-up, there was no recurrence of symptoms, the imaging findings revealed significant improvement Fig. , Video 1FM can be idiopathic or secondary, and most cases of secondary FM have infectious or non-infectious etiologies. The infectious etiologies include histoplasmosis, blastomycosis, coccidiomycosis, aspergillosis, and tuberculosis. The noninfectious etiologies include sarcoidosis, silicosis, rheumatoid arthritis, systemic lupus erythematosus, antineutrophil cytoplasmic antibody-associated vasculitis, IgG4-related disease, Riedel’s (fibrous) thyroiditis, and Behçet’s syndrome, tumor, and radiation or drug therapy . We exclThe two main imaging features of FM are local soft tissue masses around the mediastinum and diffuse mediastinal infiltration . DiffuseThe current case is very rare, in that the lesion had extensive invasion of the heart and had imaging features indicative of FM. Five specific features made our case unique. First, there was diffuse proliferation of fibrous tissue that invaded multiple chambers. Second, the lesion mainly invaded the large blood vessels of the heart, with further involvement of the atria and the ventricles. Third, the soft tissue mass was wrapped extensively along the anatomical structures of the heart, resulting in lumen stenosis. Fourth, the lesion protruded into the atrium and formed a soft tissue mass. Fifth, soft tissue masses infiltrated the myocardium. In addition, our CDFI showed a high-speed blood flow signal in the narrow pulmonary arteries and pulmonary veins.The imaging findings of FM may suggest its etiology. Sherrick et al. reviewed the records of 33 patients with FM and found that the most likely etiology of the 27 localized cases was histoplasmosis, and the most likely etiology of the 6 diffuse cases was idiopathic or non-infectious [It is very rare for diffuse lesions to invade the heart, and a differential diagnosis to exclude tumors is necessary. Unfortunately, there are limited studies on the use of echocardiography to distinguish tumor from FM, especially because heart tumors are rare. We suggest two criteria to distinguish these two conditions. First, a malignant tumor mainly infiltrates the heart, but FM mostly encapsulates or wraps the heart. Second, a tumor may invade any part of the heart with no fixed pattern, but FM usually grows along the large blood vessels and then down to the atria and ventricles.Encapsulated growth may also be encountered in Erdheim-Chester disease (ECD), whose imaging features are very similar to those of FM. ECD is a non-Langerhans histiocytosis of unknown origin. Its most common cardiac manifestation is a pseudotumor close to the right atrium that invades the right atrium and atrioventricular groove. Soft tissue masses also form around the aorta, pulmonary arteries, and their branching vessels . BecauseThe diagnosis of FM is the most important element of the current case. Although CT and MRI can provide more comprehensive information, echocardiography has the advantages of being more readily available, less expensive, and radiation-free. Thus echocardiography may help in the diagnosis and management of FM. Although echocardiography does not eliminate the need for other imaging techniques, such as CT and MRI, it may allow clinicians to reduce their use during follow-up. The extensive cardiac involvement in the current case suggests this may be a prerequisite for the application of echocardiography. In addition, ultrasound has high resolution and provides a good visualization of soft tissue. Coupled with CDFI, it can be used to determine the possible nature of lesions. Although there are no echocardiographic criteria for the diagnosis of FM, our study shows that echocardiography can aid in the diagnosis of FM.Echocardiography can help in the characterization of the cardiac involvement of FM. It is helpful in the diagnosis, although histopathologic confirmation is mandatory, as well as for monitoring the progression of FM and the therapeutic responses to systemic therapies.Additional file 1: Video 1. Echocardiography showed the characteristics of FM invading the heart, as well as follow-up records.


Uterine leiomyomas commonly reduce naturally after menopause. We report a rare case of metastasizing leiomyoma that grew after surgical menopause. A 68-year-old woman suffered from pelvic and lung masses without clinical symptoms. Nineteen years ago, she underwent a total hysterectomy and bilateral adnexectomy for multiple uterine myomas and bilateral endometriotic cysts. She has since been regularly prescribed conjugated estrogens. Surgery was scheduled in order to rule out malignancy; abdominal masses resection and thoracoscopic left partial pulmonary resection  were performed. The histological diagnosis was leiomyoma in both abdominal and lung masses, and there was no evidence of gene mutations, which suggested that leiomyosarcoma was indicated. This case may indicate that hormone replacement was augmented via derived nutrient vessels after a surgical ovarian absence. Uterine leiomyomas are the most common benign tumors in gynecology. Most uterine leiomyomas are commonly detected through changes in the expression of estrogen receptors (ER) and progesterone receptors (PR), and these female hormones are involved in proliferation. Uterine leiomyomas gradually reduce naturally after menopause. Metastasizing leiomyoma (ML) has been known as a rare phenomenon of uterine leiomyoma extending outside the uterus, and it often requires a differential diagnosis from the metastasis of malignant tumors.Here, we report a very rare case of ML that grew in the pelvic cavity and the lungs during long-term hormone replacement therapy (HRT) after surgical menopause following a hysterectomy.A 68-year-old, multiparous woman was referred to our hospital for treatment of multiple abdominal masses with no clinical symptoms. This patient had normal stature (154 cm), with a body weight of 56 kg and had a history of micro-cerebral infarction. At 49 years old, she underwent a total hysterectomy and bilateral adnexectomy for multiple uterine myomas and bilateral endometriotic cysts associated with menorrhagia. Then she was regularly prescribed conjugated estrogens (0.625 mg once every three days) administered in the gynecologic clinic for surgical menopause. She underwent trans-vaginal ultrasonography regularly during the oral administration. At the age of 64, a tumor with a major diameter of 4 cm was found at the right vaginal stump, while there were no clinical symptoms such as abdominal pain, ileus or respiratory discomfort. This mass gradually enlarged and a new mass with a 10 cm diameter was also present, so she was considered to be treated with surgery. The patient had been taking conjugated estrogen preparations until one month before being referred to our hospital.T2-weighted sagittal magnetic resonance imaging (MRI) demonstrated 5 cm and 10 cm diameter irregular solid tumors present over the vaginal cuff extending to the right pelvic cavity and under the umbilicus of the abdominal cavity  indicated multiple solid masses of 5-15 mm diameter of clear demarche in the bilateral lung suspected to be ML  images showed a slight accumulation of standardized uptake value (SUV) max (1.61-1.88) present in the pelvic masses while no accumulation was present in the lung masses. None of the serum tumor markers regarding gynecological malignancies had increased.Surgery for the abdominal masses was scheduled in order to rule out malignancy with abdominal mass and vaginal cuff resection being performed. No lesions other than these two masses were found in the abdominal cavity Figure .The results of the postoperative histopathological examination diagnosed leiomyomas Figure -4B. Two Our custom panel next-generation sequencing (NGS) analysis using the Gynecologic Cancer Panel Version 2  and an IFollowing long-term HRT after the patient underwent a surgically resected uterus and bilateral adnexectomy for multiple uterine myoma leading to surgical menopause, massive ML developed not only in the abdominal cavity but also the bilateral lung.In related literature, extrauterine leiomyomas regardless of surgical interventions were so-called parasitic myomas (PM) or ML. However, extrauterine lesions other than intravenous leiomyomatosis are classified as ML according to the World Health Organization (WHO) classification fifth edition.However, PM was included in the International Federation of Gynecology and Obstetrics (FIGO) classification of uterine myomas and is considered to be uterine myomas that exist apart from the uterus and are governed by a blood flow separate from the uterine artery. Regarding the mechanism of development, fragments generated when myomas were cut into small pieces during surgery possibly remained in the patient and grew slowly. Many patients have symptoms such as abdominal pain, pelvic pain, coital pain, abdominal distension, frequent urination, and constipation, while it has been reported that about 20% are asymptomatic [ML is defined as the condition in which women have extrauterine metastases of leiomyoma with a history of uterine leiomyoma. The most frequent surgical procedure is a total hysterectomy for uterine myoma, which is often found before menopause. The most common site of metastasis is the lung, and about half of these patients experienced multiple lesions. The major symptoms of lung metastasis include cough, dyspnea, shortness of breath, and chest pain, and about 40% of them are discovered during follow-up examinations .There are several theories about the cause of ML: 1) hormone-sensitive smooth muscle bundles originated in the lung grew by exposure to sex hormones and 2) the metastasis of very low-grade uterine leiomyosarcoma ,5. ML maIn this case, the histological findings of the tumors resected in the pelvis were similar to the resected lung tumors. The proliferative index was consistent with uterine leiomyoma. The decision of surgical resections in pelvic or lung masses is important for distinguishing ML from uterine leiomyosarcoma. The tumors were suggestive of metastases in the lungs, clarifying the pathological diagnosis by surgical resection rather than hormone therapy. The result of positive for ER and PR supports the hypothesis of residual lesions after surgery gradually increasing due to long-term HRT. An important point in this case is the continuous administration of HRT for a long duration of approximately 20 years and the advanced age of the patient.Table Nine out of the 11 cases had a clear history of uterine myoma -11,13-17In our patient, the tumor was discovered before respiratory symptoms or abdominal distention because the patient received regular follow-ups from the previous doctor who was administering HRT. In our gene panel tests, which were focused on rare malignant tumors and rapidly progressing malignant tumors, we also examined the evaluation of clonality between pelvic and lung tumors, while the efficacy could not be achieved.In recent years, the search for genetic abnormalities in benign ML has advanced, and a comparison with genetic abnormalities in leiomyosarcoma has also been conducted . The mosOther methods to clinically differentiate between leiomyosarcoma and ML, such as changes in microRNA (miRNA) expression, have been noted. It has been reported that its expression pattern changes in various malignant tumors, and it is expected as a biomarker for the diagnosis and treatment of a wide range of diseases . It has In conclusion, this patient's case demonstrates that the progression of PM or ML via derived nutrient vessels should be considered when the patient has undergone HRT following hysterectomy and bilateral adnexectomy for uterine myoma. ML should be ruled out by pelvic examination or imaging if the patient has abdominal or respiratory masses of unknown origin.


Cystic lesions observed in parotid glands are relatively rare and comprise 2–5% of all parotid primaries. A salivary duct cyst (SDC) is a true cyst representing 10% of all salivary gland cysts. The risk of malignant transformation of SDC’s epithelium is extremely rare. In the literature, only three cases of carcinoma ex SDC of the parotid gland are described. This report presents the first in the literature case of myoepithelial carcinoma (MECA) arising from a parotid SDC. A 75-year-old male patient was referred to the Department of Cranio-Maxillofacial Surgery of the Jagiellonian University in Cracow, Poland due to a cystic tumor arising from the right parotid gland. Superficial parotidectomy with facial nerve preservation was performed. Histological examination confirmed a rare case of MECA emerging from the SDC. The immunohistochemical profile of MECA ex SDC was presented. During 6 months of the follow-up, local recurrence or distant metastasis was not observed. The World Health Organization (WHO) 2022 Classification of Head and Neck Tumours distinguishes tumors and tumor-like lesions, which include a variety of cysts . Cystic The current report describes the first in the literature case of myoepithelial carcinoma (MECA) arising from the parotid SDC.In September 2021, a 75-year-old male patient was admitted to the Department of Cranio-Maxillofacial Surgery of the Jagiellonian University in Cracow, Poland, with a ten-year history of a slowly growing, painless cystic tumor of the left parotid gland. Progression in the last period was not observed. The patient suffered from arterial hypertension, and mitral and tricuspid valve regurgitation. In 2018, the patient had a stroke of the right hemisphere of the brain, with left-sided paresis and balance disorders. Physical examination revealed a soft, movable, and fluctuating cystic tumor of the parotid gland with unchanged skin above it. In fine needle aspiration cytology (FNAC), 15 mL of brown fluid content was obtained. Any signs of peripheral facial nerve paresis were not observed. Computed tomography (CT) showed a unilateral, solitary, well-defined, thin-walled, multilocular cystic lesion with dimensions of 49 mm (ax.) × 29 mm (cor.) × 37 mm (sag.) located in the superficial lobe of the parotid gland. In the distal part of the cystic lesion, a contrast-enhancing solid tumor 9 mm in diameter was revealed A. ImaginThe patient was qualified for surgical treatment. A modified Blair incision was used to perform superficial parotidectomy with facial nerve preservation. During the surgery, intraoperative neuromonitoring was used to identify branches of the facial nerve (Inomed C2 Nerve Monitor). As an antibiotic prophylaxis, 1 g of Cefazolin was administrated. The superficial lobe of the parotid gland with the thin-walled cyst was excised. The tumor was located in the lumen of the cyst, which was situated in the superficial lobe, directly on the lateral aspect of the temporal, zygomatic, and buccal branches of the facial nerve B. The inThe histopathological examination of the surgical specimen revealed a salivary gland with lobules separated by adipose tissue and preserved histological structure. Adjacent to the salivary gland a multilocular cyst with a diameter of 40 mm was found. The cyst was lined with mainly single-layered cuboid glandular cells, in some places with double-layered, and focal formation of small papillary outgrowths up to 0.1 mm in size.One of the cyst’s cavities included a tumor with an 8-mm diameter growing from the cyst’s wall. The tumor was composed of mixed small cells, partially epithelioid, partially spindle without marked cytological atypia, with a tendency to form trabecular structures C. The tuThe entire histological picture supported the diagnosis of a low-grade myoepithelial carcinoma ex-salivary duct cyst. A cluster of cancer cells was found in the cyst wall except for the well-limited remains of the tumor tissue thrust into the lumen of the cyst. Minimally infiltrative growth through the cystic wall without surrounding tissue involvement was observed. Perineural and angiolymphatic invasion was not present. MECA ex SDC was resected with adequate surgical margins . Moreover, in the surgical specimen of the superficial lobe of the parotid gland, ten small lymph nodes with features of reactive changes without metastases were also found.After tumor board consultation, due to the radical resection and early stage of low-grade cancer pT1N0, the patient was qualified for systematic oncological follow-up in accordance with the NCCN guidelines. A follow-up ultrasound examination 3 months after the procedure was performed without signs of locoregional recurrence. During 6 months of the follow-up, locoregional recurrence was not observed and the facial nerve function was preserved. Unfortunately, in February 2022, the patient died due to acute pancreatitis with peritonitis.Eighty percent of SDC occurs in the parotid gland and comprises about 1–2% of parotid lesions and 10% of all salivary gland cysts . SDC is MECAs, also known as malignant myoepitheliomas, are rare entities and are composed of neoplastic myoepithelial cells with infiltrative growth. Myoepithelial cells are observed around the acinus and intercalated ducts of the saliva . MECAs aThe pathological definition of myoepithelioma remains under discussion due to the morphological diversity of the neoplastic myoepithelial cells . DiffereAccording to NCCN guidelines from 2022, surgery is the MECAs treatment of choice . ClinicaSavera et al.’s study revealed that 29% of MECAs patients die from the disease after an average of 32 months. Only high-grade MECAs are strongly correlated with poor prognosis ,21. On tIn conclusion, carcinoma ex SDC of the parotid gland is an extremely rare entity. To our knowledge, this is the first described case of the MECA arising from the parotid SDC. Surgery is the standard of care for both SDC and MECA. MECA emerging from SDC can be successfully treated with superficial parotidectomy with facial nerve preservation without recurrence. MECA, depending on the grade and stage, might require additional neck dissection as well as adjuvant treatment.


Intra-abdominal desmoplastic small round cell tumors (IADSRCT) are rare and aggressive neoplasia that are resistant to chemotherapy. Anlotinib is an oral multi-target tyrosine kinase inhibitor that also has anti-angiogenic and anti-proliferative properties. In this article, we report on a case showing effective and durable responses to chemotherapy combined with anlotinib in a young man with IADSRCT.A 27-year-old man was admitted to our hospital complaining of a palpable periumbilical mass that had been present for longer than 4 months. The diagnosis of IADSRCT was confirmed by biopsy and immunohistochemistry. An extensive unresectable metastasis was found on the initial diagnosis. The patient received six cycles of chemotherapy combined with anlotinib, and maintenance therapy with anlotinib was recommended. Hematochezia, proteinuria and hypertension were observed, however, long-term maintenance therapy was well tolerated. A partial response was observed after two cycles of combined therapy and the patient was still alive with stable disease at the time of reporting.Chemotherapy combined with anlotinib plus anlotinib maintenance showed promising efficacy and manageable toxicity in the treatment of advanced IADSRCT. In 1991, Gerald et al., reported a case series in 19 patients with intra-abdominal desmoplastic small round cell tumor (IADSRCT) that were characterized as rare soft-tissue sarcomas (STS) with intra-abdominal locations. The tumors showed nesting patterns of growth with focal rhabdoid features and highly aggressive behaviors . BertuzzNCT01878448), anlotinib is a landmark success in the treatment of refractory metastatic STS and was approved by the Chinese National Medical Products Administration for patients with STS with progressive disease after one line of anthracycline chemotherapy [Anlotinib is an oral, multi-target, tyrosine kinase inhibitor (TKI) that targets VEGFR-2 and -3, FGFR1-4, PDGFR-α and -β, c-Kit and Ret . Based ootherapy . Also, tBased on these findings, the novel combination provides an alternative treatment for this patient population. However, the combination of chemotherapy and anlotinib in IADSRCT remains unclear. In this article, we report a case study of IADSRCT that was treated with combination therapy and include a systematic review of the literature concerning this rare disease.In May 2019, a 27-year-old man visited our hospital complaining of a palpable periumbilical mass that had been presented for longer than 4 months. Enhanced computer tomography (CT) of the abdomen showed multiple masses in the pelvic and abdominal cavities that involved the intestine, liver, and ureter. Multiple lymph node metastases were also identified with moderate ascites  was started in July 2019. The patient also received anlotinib at an initial dosage of 12 mg, once daily, 2-week on/1-week off. After two active cycles, abdominal CT indicated a partial response (PR) (according to the RECIST version 1.1) as multiple masses were largely reduced  . The young man refused surgical resection and received anlotinib as maintenance therapy. During maintenance therapy, the most common grade 3 TRAE (according to the CTCAE version 5) were proteinuria and hypertension, especially on the first 14-days of the continuous dose schedule. Notably, when anlotinib was discontinued for 7 days, the symptoms regressed. Based on this response, a 1-week on/1-week off regimen was adopted . The dose reduction and active management of toxicities resulted in the maintenance therapy being well-tolerated. A follow-up abdominal CT in August 2020 indicated continuous PR (according to the RECIST version 1.1). Treatment with anlotinib was continued without grade 3 or 4 TRAE. However, the latest abdominal CT in June 2021 suggested progressive disease (PD) (according to the RECIST version 1.1). As the patient refused to restart chemotherapy, immunotherapy combined with anlotinib was recommended.IADSRCT is a rare neoplasm in young adult patients that most commonly occurs in males. The typical presentation is an intra-abdominal primary mass associated with visceral and parietal seeding of the peritoneum and the disease progresses rapidly . The diaMultidisciplinary treatment is the cornerstone of IADSRCT management. Standard chemotherapy consists of vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide and dactinomycin. Complete surgical resection and radiation therapy can improve the survival of patients with IADSRCT . Also, tWe reviewed the literature and summarized the cases of DSRCT with detailed medical information. The characteristic of these studies was summarized in Table By inhibiting tumor growth and angiogenesis, anlotinib has shown encouraging efficacy in metastatic STS. Existing evidence indicates that chemotherapy combined with anlotinib plus anlotinib maintenance therapy can induce objective responses in advanced/metastatic STS , 8. HoweThe patient in our study had extensive metastasis at initial diagnosis and so the intensive combination regimen was recommended as a neoadjuvant treatment aiming to obtain complete resection after maximum tumor regression. When managed with the combined therapy, multiple masses in the patient were significantly reduced and a continuous PR was achieved. Also, the lesions continuously regressed during the maintenance treatment. Overall, the duration of response (DOR) lasted for nearly 2 years.For combined regimens, safety and treatment tolerance are critical. In our study, the most common AEs were hematochezia, proteinuria and hypertension which were in line with previous studies –8. SeverOverall, our study confirmed the value of chemotherapy combined with anlotinib plus anlotinib maintenance in a patient with advanced IADSRCT. The management of AEs is critical to successful outcomes. There is a lack of prospective clinical trials for this rare disease and the findings reported in this study require further validation in large patient cohorts.


Pachydermodactyly (PDD), meaning “thick skin finger” in Greek, is a rare, noninflammatory, benign, superficial fibromatosis. We report the case of PDD in a 15-year-old boy who visited our clinic because of asymptomatic swelling of the proximal interphalangeal (PIP) joints on the third finger of both left and right hands. Physical examination revealed thickening of the skin in the radial and ulnar aspects of the PIP joints of his third finger of both hands without functional limitation or neurological symptoms. He had a habit of biting his swelling fingers, and he belonged to a basketball club at his junior high school. He had no medical history. Plain radiographs and magnetic resonance imaging of both hands showed only soft tissue thickening outside of the radial and ulnar collateral ligament of the bilateral third PIP joint. The lesions were suggestive of PDD. Surgical resection was performed via a midaxial incision and a Z-plasty to confirm the diagnosis and improve the aesthetic appearance of his hands. Histopathological examination of the lesions was compatible with PDD. After surgery on the left hand, the patient underwent the same surgery on the right hand. No recurrence or complications were observed at the one-year follow-up after surgical intervention. Thus, surgery for PDD via a midaxial incision may be a good treatment option for patients who wish to rectify the appearance of their digital deformity. Pachydermodactyly (PDD), which refers to “thick skin finger” in Greek, is a rare, noninflammatory, benign, superficial fibromatosis first reported in 1973 by Bazex ,3. HereiA 15-year-old boy was referred to our plastic reconstructive surgery department due to asymptomatic swelling of the PIP joints on the third finger of both hands following several years of progressive development. He began biting the swelling lesions, especially those on the left hand. He was part of a basketball club at his junior high school. He had no medical history of obsessive-compulsive disorders or psychiatric conditions and no family history of connective tissue disease. Physical examination revealed thickening of the skin in the radial and ulnar aspects of the PIP joints of his third finger on both hands without functional limitation or neurological symptoms , metatarsophalangeal joint (10%), or palm (2.5%) 7.5%, met,6. HowevA Z-plasty was performed to avoid contracture. However, as a midaxial incision is originally a method of avoiding contracture, further assessment for the combined use of Z-plasty with this incision is necessary.We experienced a case that achieved a good result for digit deformity of PDD after undergoing surgery via a midaxial incision. This incision is imperceptible scarring without any complications. Thus, a midaxial incision may be a good approach for patients who wish to rectify the appearance of their digital deformity.


A 13-year-old castrated male Toy Poodle presented with an acute vestibular disorder. Magnetic resonance imaging and computed tomography revealed a large oval space-occupying mass with skull destruction located from the subcutaneous tissue to the posterior fossa region. Histopathologically, the mass was a bundled growth of spindle-shaped mesenchymal tumor cells between the myofibrillar and collagen bundles. The cells were moderately irregular in size and had eosinophilic stained cytoplasm. The cells were highly atypical and had rare mitotic figures. Neoplastic cells were immunoreactive for S100, GFAP, Olig-2, SOX10 and immunonegative for NF, E-cadherin, and Claudin-1. Collective findings were presumptive with a diagnosis of malignant peripheral nerve sheath tumor. Peripheral nerve sheath tumors (PNSTs) mainly occur in the sheaths of peripheral nerves, among which malignant PNSTs (MPNSTs) have a poor prognosis. In MPNSTs, distant metastasis is uncommon, while local invasiveness is high. In addition to the peripheral nerves, PNSTs in dogs are derived from the central nervous system (CNS) and various other sites , includiA 13-year-old, 5.0-kg castrated male Toy Poodle developed acute vestibular disorder, including horizontal nystagmus and stagger (day 1). Complete blood count results were normal at the referring hospital, whereas serum biochemical examinations showed a slight increase in alkaline phosphatase  and blood urea nitrogen  levels. Six days later, the patient was referred to our hospital because of worsening clinical signs (day 7). On admission, physical examination revealed a body temperature of 39.0°C, a pulse rate of 100 beats/min, panting, and a subcutaneous mass in the left occipital region. A neurological examination revealed ataxia with hypermetria, right-sided head tilt, left-sided hemiparesis, and induction of vertical nystagmus in the dorsal position. Except for disorder to the vestibular area, no other obvious cranial nerve deficits were noted. Mental status was alert. Based on these findings, paradoxical vestibular disorder was suggested, and the left cerebellar lesion was suspected. Differential diseases considered included cerebellar infarction, brain tumor, and meningoencephalitis. Head radiographs showed a mass with increased internal permeability and bone obscuration in the occipital area ; howeverPalliative therapy was administered at the owner's request. From day 7, prednisolone  and isosorbide  were started to reduce cerebral edema and intracranial pressure. On day 11, the patient exhibited intermittent tachypnea, with no obvious improvement in clinical signs. Mental status was depressed mildly, however, palpebral reflex or gag reflex were normal. Prednisolone was then changed to 0.5 mg/kg twice a day along the owner's wishes, but he was unable to stand on day 18. Upon arrival at the hospital, decreased consciousness, decerebellate rigidity, and tachypnea were observed, suggesting severe intracranial hypertension. After treatment for decreasing intracranial pressure , his conscious level recovered, his respiratory state stabilized, and he was discharged. On day 19, his condition deteriorated, and the patient died.In the present case, the mass lesion extended intracranially and extracranially. Whether the tumor invaded from the outside in or the inside out had not been definitively determined, since postmortem examination was not available. At the onset of neurological symptoms, a large extracranial mass was already present, with destruction of the skull. Based on the time lapse from onset to diagnosis, it was considered highly likely that the tumor originated in the extracranial subcutaneous region and invaded intracranially with skull destruction.Meningioma is the most common primary intracranial tumor in dogs, followed by glioma and histiocytic sarcoma . HoweverIn the immunochemical staining of timestamp performed in this study, almost all tumor cells were positive for SOX10 and Olig-2. A few of them were also positive for S100 and GFAP. On the other hand, they were negative for NF, E-cadherin, and Claudin-1. Among these staining results, positive results for Olig-2 and GFAP supported glioma as an important differential . HoweverMost MPNSTs in the cranial nerve show hyperintensity on T2WI and FLAIR, hypo- or iso-intensity on T1WI, and a homogeneous enhancement effect of contrast media on MRI . HoweverDepending on the site of origin, MPNST treatment includes local therapy, such as surgical resection , 13, 27,Neoplastic diseases affecting the CNS commonly develop in middle and advanced ages and have a subacute or chronic progressive course. Since our patient developed acute paradoxical vestibular disorder at an advanced age, we considered vascular or inflammatory diseases rather than neoplastic diseases as the major differential diagnosis at the time of onset. Brain edema associated with intracranial invasion of the tumor might have caused the acute onset of vestibular disorder. Subsequently, progressively worsening clinical signs after onset were an important finding that suggested a neoplastic disease. Therefore, it is crucial to always consider the possibility of neoplastic diseases and evaluate the subsequent disease course, even when acute vestibular disorder occurs in older animals.A limitation of this case report is that autopsy could not be performed because the owner's consent was not obtained. From this reason, the exact origin of the tumor and the route of intracranial invasion could not be determined. Furthermore, the small sample size of tumor did not allow for sufficient immunostaining to make a more accurate diagnosis. Hence, in order to make an accurate diagnosis, it is important to collect samples from a number of locations and to be prepared to perform various immunochemical staining as needed.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 animal study because ethical review and approval are not required for retrospective case reports. Written informed consent was obtained from the patient's owner for publication of this report. Written informed consent was obtained from the owners for the participation of their animals in this study.KH, KS, YS, KN, MN, and YN assisted with the diagnosis of this case and participated in clinical case management. KH and YN participated in the review and editing of the manuscript. All authors contributed to the article and approved the submitted version.Author KN was employed by FUJIFUILM VET Systems Co., Ltd. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Evaluation of anatomical variations is important in pancreatoduodenectomy (PD) because an arterial anomaly is a risk factor for morbidity. Herein, we report a rare case of PD for lower bile duct cancer in which celiac axis stenosis and a replaced common hepatic artery (rCHA) coexisted.An 84-year-old woman presented with epigastric pain. She was diagnosed with a lower bile duct cancer and underwent PD. Preoperative computed tomography showed celiac axis stenosis, and the deformed celiac artery had a “hooked appearance,” suggesting compression by the median arcuate ligament (MAL). The rCHA branched from the superior mesenteric artery. The gastroduodenal artery (GDA) diverged from the rCHA, forming a developed arterial arcade of the pancreatic head. There was an oncological safety margin between the rCHA and common bile duct; however, a part of the collateral artery was close to the common bile duct. Therefore, we planned to preserve the rCHA and resect the GDA to form collateral circulation. The MAL was divided and we evaluated the blood flow of the left upper abdominal organs using indocyanine green fluorescence imaging with a near-infrared camera system. We considered that perfusion of organs was preserved, and PD was performed without vessel reconstruction. No ischemic complication occurred in the postoperative course.The coexistence of these arterial anomalies made the procedure of PD more complicated.Precise preoperative diagnosis of arterial anomalies is necessary to avoid postoperative complications that may be induced by intraoperative arterial injury and organ ischemia. •Median arcuate ligament and a replaced common hepatic artery are rare anomalies.•A case report of Pancreatoduodenectomy in which these arterial anomalies coexisted•Arterial injury or insufficient blood flow may induce ischemic complications.•Precise preoperative diagnosis of arterial anomalies is necessary. She had a medical history of hyperlipidemia. Physical examination revealed no fever or jaundice. Laboratory findings indicated a slightly elevated white blood cell count and liver dysfunction. Magnetic resonance cholangiopancreatography showed obstruction of the lower bile duct and dilation of the upper bile duct , which improved with antibiotic administration. Contrast-enhanced CT performed at 1 postoperative week showed that the contrast effect of the stomach, spleen, and remnant pancreas was preserved and no ischemic change was detected . The pat3PD is an important surgical treatment for tumors located in the head of pancreas, lower bile duct, and duodenum, although morbidity and mortality remain high. Insufficient understanding of anomalies in the celiac artery and SMA systems can lead to intra- and postoperative complications. This is a very rare case in which celiac axis stenosis and a rCHA were recognized on preoperative CT angiography, where a careful intraoperative assessment was required because the coexistence of these anomalies made the procedure of PD more complicated.It is reported that preoperative CT before PD showed stenosis in 11% of cases During PD in a patient with a replaced hepatic artery which originates from the SMA, the conservative approach is most frequently used unless direct invasion of cancer occurs, because of the risk of thromboembolism and postoperative bleeding caused by a pancreatic fistula Through an extensive search of PubMed, we identified only one case report concerning PD in a patient with celiac axis stenosis and a rCHA. PD with preservation of the rCHA and collateral circulation of the pancreatic head was performed for intraductal papillary mucinous neoplasia in the pancreatic head 4A precise preoperative diagnosis of arterial anomalies is required to avoid postoperative complications that may be induced by intraoperative arterial injury and organ ischemia.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.This study was approved by the ethics committee of Shinko Hospital (no. 2126).Written informed consent was obtained from the patient for publication of this case report and accompanying images.TK and KF contributed to study conception and design. All authors contributed to data acquisition and analysis. YT and EM were involved in patient treatment. TK and KF were the major contributors to manuscript writing. All authors read and approved the final manuscript.Not applicable.Takashi Komatsubara, corresponding author of this article.Not commissioned, externally peer-reviewed.None.


Phyllodes tumors, previously known as cystosarcoma phyllodes, are fibroepithelial tumors that consist of epithelial and cellular stromal components. These tumors have a predilection to recur after wide local excision and to attain massive sizes. They account for less than 0.5% of cases of all breast neoplasms and are primarily found in the third to fourth decade of life, and rarely in adolescence. However, less than 25 cases are reported to date wherein this tumor is found in adolescent females, with this case being one of those. This is a report of a rare case of a 12-year-old premenarchal female with bilateral phyllodes tumor, highlighting its peculiarities, diagnostic features, and surgical management in view of the patient’s age and quality of life. Breast is a modified sweat gland that lies in the pectoral region. Out of the several congenital, inflammatory, and neoplastic lesions, phyllodes tumor or cystosarcoma phyllodes accounts for 0.3-0.5% of tumors , with thClinically, it presents as a palpable, rapidly enlarging mass ranging from 4 cm to 20 cm . The skiDefinitive management in such cases is surgical, either in the form of simple mastectomy or wide local excision along with at least 1 cm margin  and aimiA 12-year-old female came with chief complaints of bilateral enlargement of the breast since one year and pain since 15 days. As narrated by the patient herself and her mother, she was apparently alright one year back when she noticed a lump in both her breasts while bathing. The lump in the right breast started in the upper outer quadrant and the left breast lump was first felt in the retro-areolar region. Its size rapidly progressed, such that it was approximately of size 2×3 cm at the start, which progressed to the current size of 10×10 cm. The lump was painless initially but became painful with associated heaviness in the chest region since eight months. The pain was localized in both the breasts and was non-radiating. There was no history of fever, discharge from the nipple, retraction of the nipple, ulceration over the breast, or significant weight loss. She did not take any treatment previously and her history is not significant. There is no history of breast malignancy in the family. Her sleep, bowel bladder habits, and appetite are normal. However, the pain became worse in 15 days to an extent that it hampered her daily activities like bathing and changing clothes, following which she approached our hospital.Her general and systemic examination was normal. Local examination revealed bilateral enlargement of the breast with a lump of size 8×10 cm approximately. There was no puckering, dimpling, ulceration, or fungation of the breast. The skin over the breast appeared to be tense without any engorged veins. The left nipple was at a lower level than the right one and the nipple-areola complex on the left side was completely distorted and destructed. There was no discharge from the nipple, and the arms, thorax, axilla, and supraclavicular fossa appeared to be normal. Local temperature was raised with marked tenderness bilaterally. It was bosselated and was firm in consistency. The lump was not fixed to the chest wall and no lymph nodes were palpable. A clinical photograph of the local examination is shown in Figures The USG revealed a large, heterogeneous, hyperechoic lesion of size 10×6 cm on the right side and 8×7 cm on the left side, without any spiculations or calcifications. The lesion involved all four quadrants and was suspected to be either a giant juvenile fibroadenoma or a benign phyllodes tumor. FNAC was conducted from both sides, which was suggestive of a benign phyllodes tumor. A definitive diagnosis was obtained by biopsy and histopathology confirmed benign phyllodes tumor on both sides, as seen in Figures Ideally phyllodes tumor is dealt with by wide local excision but in our case the lumps in the bilateral breast were extremely huge, making it impossible to conduct a breast-sparing surgery. Also, chances of recurrence in the later phase would have been increased in case of breast-sparing surgery. Hence, bilateral mastectomies were performed. An intraoperative photograph of the tumor is seen in Figures Phyllodes tumor, although accounting for less than 1% of breast neoplasms, should be always kept in mind as a differential diagnosis whenever a patient comes with a rapidly growing mass in the breast. Its median age is around 45-50 years but still is a rare possibility in adolescents as well . Until 2Clinical suspicion, case-based individualized approach, and multimodal investigations lead us to diagnosis, as seen in a 19-year-old girl, in the case report by Prihantono et al., complaining of rapid enlargement of a unilateral breast lump to size 20×16 cm just in three months' span . In someThe presentation in phyllodes tumors can be varied; hence, clinical, radiological, FNAC, or tissue biopsy evaluation followed by subsequent excision is a must for definitive management. As developing pediatric breast tissue is highly sensitive to ionizing radiation, ultrasonography is better than mammography in this age group . CT scanOn USG, it is difficult to differentiate between fibroadenoma and phyllodes as there are several overlapping features; however, phyllodes show more cystic areas than fibroadenoma on an USG ,6,8. OneIt is essential to delineate the differences between phyllodes and fibroadenoma as their plan of management is different. To categorize the lesion as juvenile fibroadenoma, Stanford has issued guidelines as follows: circumscribed and rarely multiple, biphasic stromal and epithelial process lacking a leaf-like growth pattern in a uniformly hypercellular stroma, a lack of atypical features, and a stroma-like periductal increase in cellularity, stromal overgrowth, and cytologic atypia, as well as a mitotic rate <3/high-power field, frequent epithelial and myoepithelial hyperplasia, and an age of between 10 and 20 years [Core needle biopsy is the final investigation to get the definitive diagnosis . DependiDepending on the histopathology reports, as shown above, complications are foreseen and the plan of management is outlined. Benign and borderline variants pose a lesser risk than the malignant ones. If left untreated, they undergo pressure necrosis and ulceration  or can lThe definitive line of management of phyllodes, be it benign, borderline, or malignant, remains surgical wide local excision with a 1 cm margin or simple mastectomy, as the lymphatic spread is uncommon to axillary lymph nodes. However, a study of 19 reported phyllodes cases by Leveque et al. suggests that wide local excision should be performed more often and simple mastectomy should be reserved for recurrent cases . RadiothIn cases of large lumps of benign phyllodes, mastectomy is indicated, as seen in our case. Phyllodes tumors tend to recur and studies have reported that such patients have an increased risk of breast carcinoma in the future if not managed appropriately; hence, simple mastectomy forms a preferred choice here. The patient in our case underwent simple mastectomy bilaterally, wherein the nipple-areola complex on the right side was preserved. She will be planned for breast reconstruction surgery as soon as she attains her menarche. Prosthetic reconstruction also can be done immediately, provided the patient has already achieved her menarche . In the With less than 25 cases reported to date, breast neoplasms in the adolescent age group are extremely rare. Our case report is of a 12-year-old premenarchal girl, who came with a bilateral phyllodes tumor. Such a unique and uncommon presentation requires vigilant diagnosis and prompt treatment. Definitive management in these cases is surgical, hence the patient underwent a simple mastectomy and will undergo breast reconstruction once she attains her menarche, for a better quality of life. However, the massive size of the tumor and the bilateral representation in a premenarchal, unmarried, adolescent female made the decision of surgical treatment even more challenging. Case-based diagnostic approach and reconstructive measures taken in this case may provide useful insight for the clinicians and help them formulate more appropriate management.


Ossification of stylohyoid chain corresponds to the ossification of stylohyoid ligament that can vary from thin short to thick long ossification and can be associated with other calcifications. We report in this case a bilateral non painful complete ossification of the stylohyoid complex extended to the thyrohyoidien ligament. Ossification and elongation of the stylohyoid chain can cause neck and facial symptoms, known as Eagle's syndrome. Nonetheless, a bilateral ossified and elongated stylohyoid chain associated with ossification of the thyro‐hyoid ligament may be not painful. A 53‐year‐old man consulted the dental medicine department for a dental check‐up. A routine conventional dental radiographic examination was performed and showed bilateral ossification of the stylohyoid complex Figure , then coHe reported the history of severe cervical trauma caused by a fall down stairs 30 years ago.CT scans of the neck showed an elongated and heavily ossified styloid process, the stylohyoidien ligament extending to the lesser cornua of the hyoid bone with three pseudarthroses, associated with a bilateral ossification of the thyrohyoid ligaments Figure .The stylohyoid complex (SHC) consists of the styloid process, the stylohoid ligament, and the lesser cornu of the hyoid bone.All the authors declare that there is no conflicts of interest.Eya moussaoui performed data collection and interpretation and drafting the manuscript. Sahar kadri involved in collection and interpretation of data. Ouaha lamia revising the manuscript for important intellectual content. Nabiha douki performed conception and design and revising the manuscript.Data from the patient included in this case report were treated anonymously, and a statement of informed consent was signed to allow the use of her medical and dental records and photos.Written patient consent has been signed and collected in accordance with the journal's patient consent policy.


Presacral myelolipomas form a rare disease and are often found incidentally in imaging diagnostics.In this study, we report the case of a 71-year-old caucasian female with an incidental finding of a retroperitoneal tumor on magnetic resonance imaging scan. This report aimed at presenting the clinical course of this patient with emphasis on analysis of pathological, clinical, and epidemiological features in a meta-analysis of reported cases.Presacral myelolipomas are rare and its etiology remains unclear. Surgical resection is indicated in symptomatic lesions and lesions > 4 cm. More clinical and pathological research on this rare entity is warranted. Myelolipomas are benign tumors that are composed of mature adipose tissue and elements of extramedullary hematopoiesis with trilinear hematopoietic cells  with unkThey were first described by Gierke in 1909  and nameAbout 15% of myelolipomas are found in extra-adrenal locations , althougTo date, presacral myelolipomas are described in fewer than 60 cases in English literature published on PubMed and form a rarity. The first patient was described by Blaisdell in 1933, concerning a case of extramedullary hematopoiesis found in a retroperitoneal tumor in an elderly woman .This study aimed to systemically review and meta-analyze clinical, radiological, and epidemiological features of the presacral myelolipoma and present new case of a 71-year-old female with an incidentally detected tumor diagnosed by CT-guided biopsy.All studies published until 30 September 2022 on the topic “presacral myelolipoma” was included in the current analysis with no restriction on age or language.Systematic searches were performed using the term “presacral myelolipoma” on PubMed.Articles were considered by reviewing title, abstract, and the full text if in doubt.Inclusion criteria was a confirmed diagnosis with presacral myelolipoma. Exclusion criteria were reported cases of extra-adrenal myelolipomas that were not located in the presacral region and all research articles on the topic myelolipoma without case presentation.The PRISMA guidelines were followed.The following information was extracted from each study: first author, year of publication, title, number of patients with presacral myelolipoma, gender, age, tumor size in diameter, reported symptoms, imaging technology used for diagnosis, and treatment.All articles were analyzed, and a database was formed. In Table U test was performed for continuous non-normally distributed variables. Categorical variables were compared using the chi-square test.Descriptive data are presented as medians and interquartile range for non-normally distributed data, as appropriate. Categorical data are displayed as frequencies and percentages. Continuous data were tested for their normal distribution by Shapiro–Wilk analysis. Mann–Whitney p < 0.05 was considered to indicate significance. All analyses were performed using SPSS Statistics Software version 28.0  on macOS 12 Monterey.A 71-year-old caucasian female visited our outpatient clinic with an incidentally detected retroperitoneal tumor. An exophytic presacral mass of size 2.6 × 6 × 1.9 cm  of 11.900 was found. Further analysis revealed a normal distribution of white blood cells without abnormal cell proliferation.A CT-guided biopsy confirmed the diagnosis of a presacral myelolipoma consisting of mature adipose cells with trilineage hematopoietic element. No ectopic adrenal tissues were observed Fig. .Fig. 2HiIn consent with the patient, a decision towards further follow-up by MRI imaging of the presacral myelolipoma was given, as the patient was asymptomatic when discharged from the clinic.At 6 months follow-up, there was no evidence of tumor enlargement by MRI imaging and the patient remained asymptomatic. Further radiological reevaluations are scheduled at 6–12-month intervals.et al. described five cases, whereas Grignon et al. and Andriandi et al. described three and two cases, respectively.The PubMed database search identified 64 published articles. Through citation search, one more article was identified. Forty-seven publications with 54 reported cases were considered eligible for inclusion. Of the included articles, no longitudinal cohort studies were identified. A small number of included studies described more than one case. Lee The earliest case included in the current analysis was from 1933, while the latest case was from 2021.Figure The clinical characteristics of the 54 cases searched in literature are depicted in Table The mean age was 66.4 years (IQR 59.7–74.0 years), ranging from 1.5 to 85 years.Three cases were found during an autopsy. Women form the majority of reported cases, with a female predominance of 3:1.n = 12), followed by abdominal discomfort (n = 6) and urological complaints (n = 6). Other symptoms presented were neurological pain in the lower extremities, lower back pain, and bowel obstruction. About half of all cases were incidentally detected (48%).The mean size of the presacral tumor was 7.2 cm. More than half of the patients presented with symptoms . The most frequent symptom was abdominal pain , followed by CT only (30%). Other cases were detected by clinical and ultrasound examinations and dynamic x-ray. The diagnosis was confirmed by a fine-needle biopsy in 28 patients (52%). Thirty patients (56%) received a resection.p = n.s.). No metastasis or recurrence was reported after an R0 resection.There was no significant difference in the size of lesions between symptomatic and asymptomatic patients  or by a fine-needle biopsy (28 patients) including our patient.According to American Association of Clinical Endocrinology (AACE)/American Association of Endocrine Surgeons (AAES) guideline from 2009 for adrenal incidentaloma, small and asymptomatic and hormonally inactive lesions < 4 cm are recommended for radiological reevaluation at 3–6 months and then annually for 1–2 years .The patients may develop a variety of symptoms such as small bowel obstruction , urinaryet al. in 1987, which caused urinary retention and azotemia associated with compression of urinary bladder neck, and the tumor was later completely resected [Myelolipomas larger than 6 cm are prone to complications such as spontaneous rupture or rupture due to trivial trauma and hemorrhage with the probability of an acute abdomen . Indeed,resected .et al. retrospectively assessed a series of 12 patients with 13 myelolipomas in 1997 who received a conservative treatment [Due to the rarity of the tumor, there are few comprehensive studies on the detailed clinical features. Han reatment  and follreatment .In our analysis, no malignant transformation or metastasis was, except for a rare infiltration secondary by other tumors (chronic lymphocytic leukemia), so far reported , 22.The etiology of myelolipoma of the adrenal glands as well as of the presacral counterpart remains unknown. Several hypotheses have been discussed, including metaplasia of reticuloendothelial cells in blood capillaries as a response to an event such as necrosis, infection, or inflammation , 33. HowPresacral myelolipoma is a rare disease with unknown etiology. In this study, we analyzed clinical, radiological features of previously reported 54 cases and reported the case of a 71-year-old woman with an incidentally detected presacral myelolipoma. For asymptomatic tumors, an observation with a regular imaging follow-up can be recommended after histological diagnosis by biopsy. Resection should be considered for bigger lesions > 4 cm and/or for symptomatic patients. Contrast-enhanced MRI and fine-needle biopsy are indicated to determine the definitive diagnosis.