VHL tumor suppressor gene. Here, we describe a patient with unknown VHL disease who has complained of hypoesthesia of the right lower limb for about six years. A lumbar MRI was performed and revealed an expansive foraminal lesion at the right L3-L4 level and multiple serpiginous intradural and extramedullary flow voids involving the dorsal aspect of the spinal cord. The patient underwent digital subtraction angiography to exclude a spinal dural arteriovenous fistula, which revealed imaging features suggestive of spinal hemangioblastoma. In the presence of a spinal hemangioblastoma, a brain MRI was performed for further evaluation to rule out the possible diagnosis of VHL disease, and a solitary hemangioblastoma on the right cerebellar hemisphere was found. The patient underwent lumbar spine surgery, confirming the suspicious diagnosis of hemangioblastomas related to VHL disease.Von Hippel-Lindau (VHL) disease is a rare neurocutaneous disorder characterized by multiple benign and malignant tumors involving different organs  due to mutations in the  Von Hippel-Lindau (VHL) disease is a rare neurocutaneous disorder whose prevalence is estimated to be between 1 in 36 000 and 1 in 50 000 individuals. It is characterized by multiple benign and malignant tumors due to mutations in the VHL tumor suppressor gene that can involve many compartments, such as the abdominopelvic, urogenital, and central nervous systems .Hemangioblastoma is one of the manifestations of the disease, and it occurs in the intracranial compartment in most cases, mainly in the posterior fossa (44%-72%) and in the spinal cord 13%-59%) [3%-59% [1A 47-year-old man with no relevant personal medical history presented with hypoesthesia on the lateral side of the right lower limb for about six years, worsening in recent months, without motor deficits or sphincter alterations. The physical examination was unremarkable. Thoracic spine magnetic resonance imaging (MRI) Figure  revealedThe patient underwent digital subtraction angiography Figure , which rDue to the suspicion that it could be a hemangioblastoma. A brain MRI Figure  was perfThe patient underwent lumbar spine surgery through a posterior approach with laminotomy and foraminotomy at right L3-L4 level, confirming an hypervascularized expansive lesion with extradural and intradural components, causing compression of the roots of the cauda equina. Under optical microscopy, complete resection of the lesion was performed, with dural resection. The homeostasis review was carried out with two units of Superclot®, an absorbable hemostatic system to control bleeding. Hermetic dural synthesis under the microscope was performed, as well as replacement and bone fixation of the laminotomy with plates and screws. Intraoperative neurophysiological monitoring with an electrode to root stimulation was performed to ensure the preservation of root function during tumor excision. Histology examination revealed compact stromal cells with numerous associated capillaries Figure , and an Fourteen months later, the patient performed a follow-up lumbar spine MRI Figure , demonstSpinal hemangioblastomas are rare, representing 2.1% of all spinal tumors, and are mostly sporadic and usually present in the fourth decade [The most common location for hemangioblastomas is the thoracic cord, and the majority of these lesions have an intramedullary component, exhibiting eccentric growth and, sometimes, an exophytic component  ,5.Although the literature reports entirely extradural or extra- and intradural hemangioblastomas, they are considered to be a very rare feature, making this diagnosis particularly challenging ,6. To ouIn these challenging cases, a brain MRI must be performed to exclude other potential differential diagnoses. Our case demonstrates the role of the brain MRI in establishing the diagnosis of hemangioblastoma in the context of VHL disease. The presence of flow voids in and around the tumor, the low signal on the diffusion-weighted image (DWI), the strong homogenous enhancement after gadolinium, and the high rate of tumor blood flow in the ASL technique suggested this diagnosis . Owing tMicrosurgical total resection is the treatment of choice for symptomatic spinal hemangioblastomas because a good prognosis may be expected after successful surgery with clinical improvement . The recThe present case represents atypical imaging findings of spinal hemangioblastoma, a hypervascularized tumor that can cause an arteriovenous shunt, mimicking a spinal dural arteriovenous fistula with schwannoma due to the foraminal extension, in a patient with Von Hippel-Lindau disease. In these challenging cases, because many other entities can present similar radiological imaging, investigation with other complementary means of diagnosis should be performed in order to establish the correct diagnosis and define the best therapeutic approach for the patient.


Primary bone lymphoma (PBL) is a rare extranodal presentation within lymphomas and primary bone malignancies. Pathologic fracture (PF) is a common complication of metastatic bone disease but is, rarely, the presentation of a primary bone tumor. We report a case of an 83-year-old man with a history of untreated prostate cancer, presenting with atraumatic fracture of his left femur after months of intermittent pains and weight loss. Radiographic workup revealed a lytic lesion suspicious for PF secondary to metastatic prostate cancer; however, initial core biopsy results were inconclusive for malignancy. A complete blood count with differential and complete metabolic panel was within normal limits. During surgical fixation and nailing of the femur, a reaming biopsy was performed as a repeat measure and revealed diffuse large B-cell lymphoma. Staging with positron emission tomography and computed tomography found no evidence of lymphatic or visceral involvement and chemotherapy was promptly initiated. This case highlights the diagnostic workup challenges for PF secondary to PBL, especially in the setting of concurrent malignancy. Because of the non-specific presentation of a lytic lesion on imaging associated with atraumatic fracture, we highlight PBL as an important diagnostic consideration. The 2020 World Health Organization classification of tumors of bone classifies primary non-Hodgkin lymphoma of the bone as a neoplasm of malignant lymphoid cells in the bone with no lymph node involvement or other extranodal involvement . OriginaApproximately 95% of diagnosed PBL is pathologically characterized as diffuse large B-cell lymphoma (PB-DLBCL) , 6, 7. MHerein, we present an unusual presentation of PB-DLBCL as a PF in a patient with concurrent untreated prostate cancer. This case highlights the importance of considering PBL in the differential diagnosis for patients with a PF.An 83-year-old male Caucasian prison inmate with a past medical history of untreated prostate cancer presented to the prison hospital after spontaneous development of pain and weight-bearing limitation in the left leg. Over the previous 2 months, the patient reported intermittent sharp shooting pains in his left thigh and 10 pounds of unintentional weight loss. He denied experiencing any episodes of fever, chills, or night sweats, and the only other positive symptoms upon a review of systems included occasional constipation and diarrhea. Family and social histories were, otherwise, felt to be non-contributory. The physical exam was significant for tenderness to the left lower extremity at the superior femur with warmth but no bruising. Dorsalis pedis and posterior tibial pulses were palpable bilaterally. On musculoskeletal exam, the patient was able to move his toes and ankle without pain bilaterally but exhibited pain during left knee and hip range of motion tests. The range of motion at the left hip was completely inhibited by pain. He had a full painless range of motion in his right lower extremity. Complete blood count showed a decreased hemoglobin level of 10.5 g/dl with no other abnormalities; basic metabolic panel was notable for hypocalcemia to 7.9 mg/dl with all other electrolytes and creatinine within normal limits. Prostate specific antigen was 5.08 ng/ml. Left lower extremity x-ray showed an irregular 2.5-mm displaced left femur shaft fracture with a “moth-eaten” appearance consistent with PF (vs. hematoma (Computed tomography (CT) scan of the left lower extremity showed a comminuted fracture of the mid-femoral diaphysis with permeative appearance of the cortex at the level of the fracture. A large heterogeneous surrounding soft tissue enhancement was noted, representing a possible soft tissue lesion via Ki-67 indicated a high proliferation rate at approximately 80%–90% in the viable areas. In situ hybridization was negative for Epstein–Barr virus. This established a diagnosis of DLBCL. Because of the presence of a solitary osseous lesion without extraosseous evidence of disease, the tumor was further classified as PB-DLBCL. The diagnostic assistance of MRI was no longer needed, so attention was turned toward treatment and staging.Magnetic resonance imaging (MRI) was planned for the left leg, but the patient first underwent intramedullary nail fixation and reduction, during which a repeat and conclusive intraoperative left femur reaming biopsy was taken Figure 2Initial treatment was delayed because of the necessary post-operative recovery. A positron emission tomography (PET) and CT scan was performed, which revealed hypermetabolic activity of the mid-femoral bone at the site of the bone lesion .2), cyclophosphamide (400 mg/m2), doxorubicin (25 mg/m2), and vincristine (1 mg) all given intravenously on day 1 of each cycle (C) and oral prednisolone (40 mg/m2) on days 1–5. During this time, he noted a steady progression in his ambulatory status ultimately using a walker in his prison unit. Of note, vincristine was dropped after C2 from the regimen due to development of constipation, neuropathy, and palpitations. Pancytopenia was noted after C5 but resolved. Radiation therapy was planned with pending CT simulation, set to begin after completing chemotherapy. However, prior to his appointment for C6, the patient had a fall in his unit resulting in fracture of his right femur. He underwent subsequent right hip hemiarthroplasty which delayed C6. CT Simulation scan of the left hip was still done, which found incomplete response to chemotherapy. Repeat PET-CT was planned to re-stage, but the patient unfortunately developed septic shock secondary to right lower lobe pneumonia and died before any further diagnostic or treatment methods were employed.The patient completed five of six planned cycles of R-miniCHOP chemotherapy, consisting of 3-week intervals of rituximab  periosteal reaction, a lytic or permeative marrow pattern, and the presence of a soft-tissue mass . Althougvia PET. PET-CT scanning is quickly gaining interest as a tool for initial characterization, staging, biopsy selection, evaluation of treatment response, and surveillance of tumor recurrence , which has a better prognosis and is commonly found in PB-DLBCL , 8. A stAlthough surgery plays an important role in bone stabilization and resolution of fracture, PBL treatment typically consists of anti-CD20 (rituximab) and CHOP based therapies, with the possible addition of radiotherapy (RT) if necessary . CompareOne severe limitation in our approach to this case was poor access to MRI, normally an essential aid to diagnosis of any bone lesion suspicious for cancer. Likewise, our initial ultrasound-guided core needle biopsy was unhelpful, the inconclusive results of which may be insufficient amount of diagnostic material. However, we were able to obtain an adequate sample to establish our diagnosis from the reaming biopsies. Furthermore, lack of interim or routine surveill63ance PET-CT  hindered our treatment plan, as the patient was ultimately not showing much response to the r-mini CHOP regimen . HoweverHere, we summarize our experience with managing PB-DLBCL presenting with PF in a patient with a synchronous untreated prostate cancer and provide a comprehensive review of literature on this topic, with emphasis on the diagnostic challenges and use of reaming biopsy during fracture stabilization to make the diagnosis. Although PBL rarely presents with PF and even more rarely is synchronous with another primary tumor, it should be considered as a possible primary bone tumor in the differential diagnosis for PF.The original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding author.Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.Study conception and design: PoB, AY, and KL. Manuscript preparation/drafting: PoB, ZH, AY, BG, RA, DW, PeB, AR, TM, PM, JM, and KL. Expert review: KL. All authors contributed to the article and approved the submitted version.


Primary cardiac lymphoma (PCL) comprises 1–2% of all cardiac tumors and 0.5% of all lymphomas. Due to its rare occurrence and variety of clinical symptoms, this tumor is difficult to diagnose, and biopsy is indispensable for diagnosis and therapy [We report on a 75-year-old male, immunocompetent patient, who presented with a decrease in general condition and palpitation in the cardiology department of the University Hospital Frankfurt. He complained of thoracic pain and dyspnea on exertion for two weeks. The patient had a history of hypercholesterolemia and gout; however, his family history was negative. Acute coronary syndrome was initially ruled out. Echocardiography revealed inhomogeneous wall thickening in the right ventricular free wall with bulging into the right ventricle, which severely impaired right ventricular function. Subsequent cardiac CT Fig. a and MRIBeing a very rare tumor with non-specific symptoms, the diagnosis of PCL is often made too late, which may lead to a poor prognosis for the affected individuals. Patients are required to undergo several imaging procedures to differentiate PCL from other possible entities, such as angiosarcoma. A biopsy is ultimately mandatory to make the correct diagnosis. , 3. TherThis case report highlights the high importance of a fast diagnosis of PCL in the prognosis of this rare tumor. Furthermore, it emphasizes that direct transcutaneous CT-guided puncture of the heart is feasible and should be considered a potentially useful tool in diagnosing percutaneous accessible cardiac tumors to facilitate prompt diagnosis and initiation of therapy.


Brain stone is an umbrella term for benign intracerebral calcifications and may be associated with various diagnoses. The surgical decision should be made on a case-by-case basis. Sometimes, conservative management should be considered, irrespective of the underlying pathology. We present a critical case with a brain stone treated conservatively. A 17-year-old female patient was admitted to our department with a headache. The neurological examination revealed no abnormal findings. Cranial CT and MRI scans showed a contrast-enhanced, highly calcified lesion located deep in the white matter at the level of the left centrum semiovale. Surgery was found unnecessary. The patient presented no neurologic deficits or symptoms during the three-year follow-up period. In this case, the differential diagnosis included arteriovenous malformations (AVMs), cavernomas, calcifying pseudoneoplasms of the neuroaxis (CAPNON), etc. The localization of the lesion, expression of the symptoms, and potential outcomes of a possible surgery should be carefully estimated before making the final decision. In summary, conservative treatment should also be considered for critically located, benign calcified lesions, irrespective of pathology, unless they cause intense neurologic symptoms or deficits. They are also called.  lesions . Brain s lesions ,3. SometA 17-year-old female patient with no medical history was admitted to our outpatient clinics with a headache. She was experiencing a dull headache that had worsened over the past two months and was more intense at night. The patient had no pertinent family history of systemic or neurologic disease and showed no impairment in social functioning. The patient is a non-smoker and does not use illicit or recreational drugs. The neurologic examination was unremarkable. Computed tomography revealed a left frontoparietal calcified and lobulated space-occupying lesion at the level of the centrum semiovale, located deep in the white matter. These findings were consistent with cavernoma, as per radiologists . Contrast-enhanced MRI revealed a highly contrast-enhanced space-occupying lesion, 25x30 mm in diameter, extending from the corona radiata to the lateral ventricle on the left side, including vascular structures. This image was thought to be consistent with a low-grade glioma. The lesion was iso-hyperintense in T1-weighted non-contrast-enhanced sequences ; oligodendrogliomas, AVMs, and calcifying pseudoneoplasms of neuroaxis (CAPNON) were considered in the differential diagnosis.Although there was no consensus on the initial diagnosis, the lesion was considered benign. And so, surgery was deferred. The patient was followed up. Three months later, at the first follow-up, the CT scan showed no increase in the size and diameter of the lesion. Painkiller medications were prescribed, and the patient declared she was headache-free. The patient showed no further headaches in the three-year follow-up period. The patient continues to be followed up at regular intervals.Brain stones are intracranial calcifications associated with a broad range of diagnoses. Patients with brain stones may present with seizures or be discovered incidentally on imaging ,4. They Cavernomas and AVMs are the most common causes of these intracranial calcifications. Large calcified intracranial lesions, or brain stones, are common pathologies. A few reports in the literature used the term ''brain stone'' and explained its association with various intracranial lesions . In our When considering intra-axial brain stones, neoplastic, infectious, congenital, and metabolic causes should also be considered . DiffereBenign intracranial calcifications may be associated with a wide range of diagnoses. Although finding the pathology of the lesion is crucial, a definitive diagnosis may not be necessary for surgical decision-making in such cases. Although there was no consensus on a definitive initial diagnosis, the lesion was considered benign regardless of pathology. Because of the benign-looking lesion, its critical localization, and the patient's intact neuro-exam, surgery was found unnecessary, and the patient was followed up. During the three-year follow-up period, the patient showed no more headaches. Since only a few cases are reported in the literature using the term brain stone, more studies are needed to clarify the term and compare the approaches to these lesions.


Sirenomelia is a rare congenital disorder that was once thought to be a severe case of caudal regression but is now thought to be entirely separate. It is often referred to as the "mermaid syndrome" because it causes the lower limbs to atrophy to varying degrees, giving the impression of a mermaid's tail or fin. The syndrome is often viewed as fatal due to the accompanying visceral deformities.Our case was a live born, delivered at term by caesarean section, to a 30-year-old third gravida having twin pregnancy. Examination of the baby revealed caudal dysgenesis with fusion of lower limbs, non-identifiable external genitalia and anus. The infant survived for 11 hours after birth. We report this case due to their rarity and term live birth.While sirenomelia is uncommon, the absence of distinct lower limbs on ultrasonography in the presence of oligo or anhydramnios may prompt consideration of the diagnosis of sirenomelia. The mermaid syndrome, also known as sirenomelia, is a rare, fatal multi-system congenital syndrome. The most common findings are lower limb fusion into a single extremity, deformities of the sacrum and pelvic girdle, absence of external genitalia, absence of anal patency, and lethal renal abnormalities . These iA 30-year-old female with G3P2L2 was booked for antenatal checkup in a dispensary. She had previous two full-term normal vaginal delivery of both male child. Her first ultrasound was done at 24 weeks which showed twin monochorionic pregnancy. Fetus A was normal, while fetus B had absent kidneys and bladder. The weight of fetus B was 30% less as compared to fetus A. The female was counselled. She wanted to continue her pregnancy as one fetus was normal. An oral glucose tolerance test was done which was normal. The female was referred to our medical college for further management. Ultrasound done in our hospital at 37 weeks showed Fetus A of 2.8 kg in breech presentation with all parameters corresponding to 37 weeks, while Fetus B of 1.7 Kg, with all parameters corresponding to 31 weeks and nil liquor. No comment was made on the spine due to poor visualisation of the fetus due to anhydramnios. This was a case of discordant twin. The patient was taken for caesarean section because of term pregnancy with the first twin in breech presentation. Twin A was a live male baby of 2.5 kg. Twin B was a sirenomelic baby of 1.6 kg with an unknown gender which cried on stimulation  [Sirenomelia can be first diagnosed at 13 weeks by a transvaginal ultrasound . AnhydraThough it has been called "fused lower limbs," this is actually more likely to be a result of the lower limb buds failing to undergo blastogenesis . There aA case study by Di Lorenzo et al. shows sirenomelia in monozygotic twins where one twin was a normal male and the other was sirenomelia. The sirenomelic baby in the study was born at term, weighing 2.7 kg having normal facies, two femurs, two tibias and two fibula. The sirenomelic infant was sedated and allowed to die . AnotherAnimal models (mice) with sirenomelia have been found to contain mutations in the CYP superfamily . SpecifiSirenomelia is a lethal congenital anomaly. Our case is a unique case of sirenomelia as there is a striking contrast between the affected twin and the spared one. This observation underscores the complexity of ethical considerations in cases involving twins with lethal diseases. In our case, the woman knew about the condition but chose to continue the pregnancy as the other twin was normal. This case serves as a poignant reminder of the ethical complexities inherent in managing rare congenital disorders in twin pregnancies, promoting further reflection on best practices in such challenging clinical scenarios.


Leydig cell hyperplasia (LCH) is a rare cause of hyperandrogenism that has been described only in case reports. The cases presented herein contrast the traditional presentation of LCH with an affected asymptomatic individual. The first case involves a 74-year-old woman presenting with symptomatic hyperandrogenism, whose symptoms resolved after bilateral salpingo-oophorectomy (BSO). The second patient presented with postmenopausal bleeding and an abdominal mass. Following total abdominal hysterectomy (TAH) and BSO, pathology showed ovarian LCH with concomitant endometrial cancer. The diagnosis of LCH is complex and requires careful investigation of many differential diagnoses. Incidentally discovered LCH may shed light on evolution and disease progression. Cases of LCH found in the setting of endometrial pathology may have implications on other states of testosterone excess. Leydig cell hyperplasia (LCH) is a rare cause of hyperandrogenism that has been described only in case reports. Here we present two cases of LCH, with one patient experiencing symptomatic hyperandrogenism and another identified incidentally following surgery for endometrial carcinoma. These cases contrast the more traditional presentation of LCH with an asymptomatic individual. The etiology of this condition is poorly understood and disputed. The unique presentations of these cases may shed insight into the evolution of this condition.A 74-year-old woman was referred by her endocrinologist for symptomatic hyperandrogenism. Her testosterone levels had been persistently elevated for three years, with her total testosterone ranging from 134 to 177 ng/dL. The patient endorsed numerous symptoms that she attributed to the elevated testosterone, including male-pattern alopecia, worsening seborrheic dermatitis, and increased central adiposity with a protuberant, firm abdomen. She also noted mood changes, including increased anger and aggression. The patient had previously been treated with finasteride and spironolactone, with no change in symptoms.Her past medical history was notable for type 2 diabetes mellitus, chronic kidney disease, polycystic ovarian disease, coronary artery disease, peripheral vascular disease, Sjogren's syndrome, Hashimoto's thyroiditis, basal cell carcinoma, and a non-specific disorder of mitochondrial metabolism. She reported menarche at 12 years old with regular menses throughout her life until undergoing menopause at 54 years old. The patient's daughter had a history of thyroid cancer, but otherwise no family history of cancer. Physical examination was notable for moderate alopecia. Her pelvic exam was unremarkable with no palpable masses on the bimanual examination.α-hydroxyprogesterone (56 ng/dL), DHEA-sulfate (21 μg/dL), prolactin (2 ng/mL), sex hormone binding globulin (26 nmol/L), insulin-like growth factor-I (153 ng/mL), aldosterone (12 ng/dL), and a dexamethasone suppression test.The patient's primary care doctor had completed a workup before referral with no clear etiology identified. The patient had dedicated imaging, with separate magnetic resonance imaging (MRI) to evaluate the pituitary gland, ovaries, and adrenal glands, which were all normal. Her labs were also unremarkable, including normal values for 17-The patient was counseled that her symptoms were likely secondary to exogenous testosterone production in the ovaries, given her negative workup for an extra-ovarian source. She was offered a BSO given her bothersome symptoms. Hysteroscopy with dilation and curettage were also recommended, due to nonspecific fluid noted within the endometrial cavity on MRI. She underwent diagnostic laparoscopy with BSO, peritoneal biopsies, and pelvic washings as well as a hysteroscopy with dilatation and curettage. Surgery was uncomplicated, and the patient was discharged home on the same day. Intra-operative findings were notable for grossly normal ovaries and a benign-appearing cystic nodule on the peritoneum overlying the bladder, which was biopsied. Hysteroscopy demonstrated a benign appearing polyp and atrophic changes. On gross pathologic examination, the right ovary measured 2.4 cm × 1.9 cm × 1.7 cm with unremarkable, lobulated cut surfaces. The left ovary measured 2.7 cm × 2.2 cm × 1.4 cm and was grossly unremarkable. On microscopic examination, both ovaries contained large rounded/polyhedral cells with abundant eosinophilic cytoplasm, central nuclei, and prominent nucleoli predominantly in the hilar area consistent with LCH. The cells stained strongly positive for inhibin A by immunohistochemistry Figures . PatientA 55-year-old female was referred for postmenopausal bleeding and an abdominal mass. She was diagnosed with a 16 cm pelvic mass, likely degenerating fibroid, on ultrasound and computerized tomography (CT) after her gynecologist noted a palpable mass on a routine examination. The patient endorsed new constipation, early satiety, and bloating for several months before presenting for care. She also reported vaginal spotting over the last year that had become increasingly heavy over recent months.Her past medical history was notable for class III obesity (BMI 50.7), obstructive sleep apnea, chronic pain syndrome, and polysubstance abuse. She reported menarche at 14 years old and menopause at 50 years old.On physical examination, there was scant blood in the vagina and no palpable masses on the bimanual examination (limited by body habitus). An endometrial biopsy was obtained and demonstrated FIGO grade 1 endometrioid adenocarcinoma. The patient had a repeat CT of the chest, abdomen, and pelvis, which showed an increase in the size of the pelvic mass to 17.8 cm.She underwent a total abdominal hysterectomy, BSO, and prolonged lysis of adhesions. Final pathology reported FIGO grade 2 endometrioid carcinoma with a microcystic, elongated, and fragmented pattern of invasion and involvement of the cervical stroma. The left ovary grossly measured 3.5 cm × 2.4 cm × 1.7 cm with a subserosal cyst that had smooth outer and inner linings. On microscopic examination, the left ovary contained large rounded/polyhedral cells with abundant eosinophilic cytoplasm, central nuclei, and prominent nucleoli in the hilar area, consistent with LCH . An inciLCH has previously been described in case reports as a rare cause of postmenopausal hyperandrogenism. Our first case provides a classic example of the typical LCH presentation with symptoms and laboratory findings consistent with excess androgen production. Some level of hyperandrogenism can be considered a normal physiologic response to a menopause-induced drop in estrogen with a lagging concomitant decline in androgen production . SymptomOne of the most common causes of hyperandrogenism in females is the familiar polycystic ovarian syndrome (PCOS). The first patient in this series had a history of PCOS, with worsening hyperandrogenic symptoms after menopause. While PCOS can be associated with mild testosterone elevations in postmenopausal women, the virilization symptoms reported by the patient were atypical. As demonstrated by this patient, evolving or severe symptoms must be scrutinized further. Though they comprise less than 5% of all ovarian tumors, androgen-secreting neoplasms of the ovary should be considered in patients with virilization , 3. InclEvaluation of symptomatic patients often starts with quantification of androgen levels and testing for hypercortisolism. This is traditionally followed by imaging of the adrenal glands, pituitary gland, and ovaries, with additional tests as warranted based on results . The extThe cases presented herein provide an excellent contrast of the varied presentations and diagnostic challenges presented by this condition. Our second case had abnormal imaging, however, it was ultimately unrelated to her incidental diagnosis of LCH. Though this patient's LCH was not causing disturbing symptomatology, these distinct presentations of LCH could provide insight into different etiologies of this rare condition. While the pathophysiology of LCH is ultimately unknown, several hypotheses have been presented. Leydig cells typically originate in the ovarian hilus, near the nonmedullated nerves, and are seen in almost all postmenopausal women . Hilar cThe second case presented is additionally unique for its association with uterine pathology. While some hormonally active ovarian tumors are known to be associated with endometrial hyperplasia or carcinoma, to the best of our knowledge, our second case is the first description of endometrial carcinoma found in conjunction with LCH , 15. WhiLCH represents a rare cause of hyperandrogenism in females. The diagnosis of this rare condition is complex and requires careful investigation of a long list of differential diagnoses. Furthermore, research is needed to determine the most effective diagnostic algorithm. Treatment is successful with BSO, with resolution of symptoms. However, this rare condition can also be found incidentally in the asymptomatic female or association with other gynecologic pathology. Incidentally discovered LCH may shed light on evolution and disease progression. Cases of LCH found in the setting of endometrial pathology may have implications on other states of testosterone excess.


Hemangioma is a congenital vascular malformation that occurs commonly in the skin and soft tissues of younger individuals but rarely in the bone. The term hemangioma occurring in bone has been referred to as venous bony malformation also. In these rare cases, vertebral bodies occur more often, followed by the craniofacial skeleton and long bones. Most rib tumors are malignant, and hemangiomas of the rib tend to grow expansively and disrupt the cortex. Venous malformations in ribs are not tumors but can be misdiagnosed as aggressive tumors or infectious processes. In fact, hemangioma of bone is a locally aggressive benign vascular malformation associated with a good prognosis. To date, no more than 50 cases of rib hemangiomas have been described in the English literature. This report presents a case of an asymptomatic 27-year-old female patient who found a quail egg-sized lump on the right side of her chest that was the size of a cocoon 2 months prior. Then, the lump grew rapidly to the size of an egg when she presented to the hospital. Because of its worrisome histomorphologic features and aggressive clinical radiologic findings, it was once misdiagnosed as a malignant tumor by most doctors. However, the pathological results after the operation confirmed rib hemangioma. Therefore, this case report aims to share this particular case so that more doctors can better understand the particularity of this disease’s progression. Hemangioma is a benign malformation of blood vessels that can occur throughout the body , 2. The A 27-year-old female patient was admitted to the hospital after finding a lump in the right chest wall for more than 2 months. Approximately 2 months ago, a quail egg-sized lump in the right chest wall attracted the patient’s attention. However, the patient did not feel any discomfort, such as pain or numbness. Therefore, the patient initially disregarded the lump. However, in the following 2 months, the lump grew rapidly, which prompted the patient to present to the hospital. Recently, she had no fever or other discomfort and no local skin redness or swelling. Physical examination showed that an irregular round lump the size of an egg was palpable at the intersection of the right mid-axillary line and the 10th rib. The skin on the surface of the lump had no abnormal changes, the lump was hard in texture, the boundary between the lump and surrounding tissues was blurred, and the lump could not be moved, but its surface felt smooth. There was no pain or sensitivity when touching the lump area. All serological examination results were normal, and there was no evidence of inflammation. Chest CT examination showed a large lump in the right chest wall, approximately 7.3 × 8.0 × 6.8 cm in size. The internal density of the lump was uneven, showing chaotic and complex high-density shadows, with string-like high-density ossification changes. The edge of the lump was smooth, without burrs, and did not invade surrounding tissues. The distal end of the right 10th rib was obviously expansive and damaged. The lump compressed the liver, but the boundary between the lump and the liver was clear (see Bone hemangiomas are benign malformations that have small or large vascular channels , 7. TypiAlthough rib hemangiomas are predominantly incidental findings that are asymptomatic, there have been reports of symptomatic presentations such as chest pain, swelling, shortness of breath, intercostal paresthesia, backaches with pleural effusion, and thoracic outlet syndrome . Previou18FDG uptake (Chest X-ray examination manifested as a strip or round abnormal shadow in a rib of the chest, which may be accompanied by bone destruction of the rib. It presents as a round and irregular lump on chest CT, with uneven-density and high-density shadow lump-like calcification and rib cortical disruption –37. MRI G uptake .As there is a lack of literature available to guide, we remain unable to recommend how to best treat rib hemangiomas. All but one previous case report has been treated with resection to definitively rule out cancer. Although this case of non-operative management of rib hemangioma with a 1-year follow-up did not reveal any malignant degeneration or size increase, rib hemangiomas required surgical intervention sooner or later, as they could not heal spontaneously , 30. MorIn conclusion, the clinical manifestations and imaging examination results of cavernous hemangioma of the rib showed characteristics of malignant bone tumors, such as osteosarcoma and chondrosarcoma. Cavernous hemangioma of the rib should be considered for chest wall tumors conforming to the above characteristics. Surgical resection is still the key measure to manage cavernous hemangioma of the rib. We maintain a cautious attitude toward adopting needle biopsy to clarify the histopathological results of chest wall lumps with the above clinical characteristics before surgery. In addition, we hope that this particular case could be shared to provide materials for clinical teaching or peer reference or further research.The original contributions presented in the study are included in the article. Further inquiries can be directed to the corresponding authors.Ethical review and approval were not required for the study on human participants in accordance with the local legislation and institutional requirements. The patient provided written informed consent to participate in this study. Written informed consent was obtained from the individual for the publication of any potentially identifiable images or data included in this article.SL, LX and FD wrote the manuscript, collected the data, and analyzed the data. X-MW and LY designed this study and checked and provided guidance during the manuscript writing process. All the authors participated in the diagnosis, treatment, and follow-up of the patient and participated in the collection and arrangement of medical records. All authors have agreed to submit the manuscript in its current form and to revise and publish it according to the journal review comments. All authors have agreed to select the current journal for submission and have agreed to be accountable for all aspects of the work.


Müllerian adenosarcoma is a very rare gynecological disease, comprising 5% of uterine sarcomas. Extragenital localizations are even rarer. We report a very interesting case of a 27-year-old woman complaining of pelvic pain, with a subsequent diagnosis of extragenital Müllerian adenosarcoma. This is the first case reported in the literature with a complete and wide imaging description. Even if rare, Müllerian adenosarcoma should be hypothesized in case of young female patients presenting with suspicious pelvic mass. Müllerian adenosarcoma is an uncommon variant of the Müllerian mixed tumor of the uterus.A 27-year-old woman refers to our hospital complaining of dyspepsia and abdominal distension for a few months. Her past medical history is silent. Her blood exams show an elevated CA 125 of 1,309 UI/ml. Upon physical examination, she presents considerable abdominal distension and obtuseness to the lower abdominal quadrants as for the presence of ascites. A transabdominal ultrasound, performed using a Philips C5–2 MHz USB curved array transducer  confirms the ascites in all peritoneal recesses  and reveMüllerian adenosarcoma is a very rare tumor and comprises only 5% of uterine sarcomas, occurring in patients from 14 to 89 years old (median 58).In most adenosarcomas with a low-grade stromal component, the stromal element expresses estrogen receptor, progesterone receptor, CD10, WT1, smooth muscle actin, low MIB1 proliferation index and is negative for P53.Müllerian adenosarcoma should be included in the differential diagnosis of young patients presenting with complex masses in. Although Müllerian adenosarcoma is a very rare disease, more studies are needed to improve the knowledge of this condition to provide its diagnostic, clinical and therapeutic management.


Langerhans cell histiocytosis (LCH) is a rare idiopathic disease that uncommonly affect the spine in adults.In this report, we presented a rare adult case of symptomatic spinal LCH with asymptomatic systemic involvement. She was a 46-year-old previously healthy lady who presented with subacute thoracic sensory level, urine retention, constipation, and pyramidal paraplegia. Her magnetic resonance imaging (MRI) of the spine revealed T6 compression fracture with an epidural mass compressing the cord.Sellar MRI showed pituitary gland enlargement with hyperintense signal in the posterior lobe. Positron emission tomography (PET)/computed tomography (CT) scan showed an increased uptake in the right parotid gland uptake and renal cortex, indicating systemic involvement.Surgical excision, decompression, and screw fixation were performed, and the patient improved. The prognosis is usually good in patients with solitary spinal LCH. •LCH is a rare idiopathic disease that uncommonly affect the spine in adults.•The prognosis is usually good in patients with solitary spinal LCH.•Spontaneous resolution is seen in a considerable proportion of patients within a year.•In focal neurological deficits, surgery may be done prior or concomitant to other treatments. The estSymptoms of LCH are highly variable based on the organs and systems involved. It can be unifocal or multifocal affecting a single organ or systems, commonly the bone, referred to as ‘eosinophilic granuloma’ . It can In adults, the vast majority of cases with LCH (69 %) affect multiple systems, whereas only one third (31 %) of the cases involve a single system . Lung isSpine LCH, despite being common in children, is very rare in adults, and only few adult cases with isolated spinal LCH were reported in the literature ,6. In th22.1.This was a 46-year-old lady with unremarkable past medical history who presented to the primary health care (PHC) on July 25th, 2022, with back pain that had been treated conservatively. Four days later, she developed with numbness of lower extremities and exacerbation of the back pain. Within one week, she returned back to the clinic as her condition was progressed to paraparesis, urine retention, and constipation. Her neurological examination revealed thoracolumbar tenderness, sensory level at T7, symmetrical lower limbs weakness of pyramidal distribution, along with brisk deep tendon jerks and Babinski sign. Her Eastern Cooperative Oncology Group (ECOG) Performance status score was 2. This case report followed the SCARE guidelines for its realization [2.2Her routine chemistry panel revealed a white blood count (WBC) of 10.6 thousand cells/μL, haemoglobin (Hb) level of 13.4 g/dL, a platelet count of 251 thousand cells/μL, an absolute neutrophil count (ANC) of 7.2 thousand cells/μL, an absolute lymphocyte count of 2.6 thousand cells/μL, a creatinine level of 84 mg/dL, a total bilirubin of 8.8 g/L, total proteins of 80 g/L, an albumin level of 3.9 g/L, a sodium level of 139 mEq/L, and an ionized calcium of 2.13 mg/L. The hormonal and endocrinal panel was unremarkable.2.3Magnetic resonance imaging (MRI) of the spine revealed a pathological compression fracture of T6 vertebra along with a prevertebral, paravertebral, and retro-vertebral intraspinal mass compressing the spinal cord . The T6 Brain MRI was also performed with special focus on sellar region and showed a centralized and thickened pituitary stalk measuring 4 mm in transverse dimensions. The pituitary gland was bulky with a high T1 signal intensity in the posterior lobe . Mild biOverall, these results found that sellar MRI showed pituitary gland enlargement with hyperintense signal in the posterior lobe. Positron emission tomography (PET)/computed tomography (CT) scan showed an increased uptake in the right parotid gland uptake and renal cortex, indicating asymptomatic pituitary and renal involvement. However the surgical excision, decompression, and screw fixation were performed then.2.4The presence of T6 pathological compression fracture with epidural mass compressing the spinal cord and causing considerable neurological deficits was an indication for surgical intervention. Therefore, the patient underwent T4 to T8 pedicle screws fixation, T6 decompressive laminectomy and biopsy of epidural mass lesion. Following anesthesia, the surgery was performed with the patient laid in prone position of Allen table. Regular prepping and draping were performed, and the surgical level was confirmed with X ray. A skin incision was then made, and the thoracic fascia was opened. Subperiosteal dissection was performed till the corresponding vertebrae were exposed. A free hand technique was used, and pedicle screws were inserted to T4 (4 × 40 Bil), T5 (4 × 40 Bil), T7 , and T8 (4 × 40 Bil). An X ray was used to confirm the appropriate location of the screws inserted. T6 laminectomy was then done, and the cord was decompressed. A biopsy was also taken from the ventral epidural soft tissue mass compressing the cord. Ten-millimeter rods were inserted bilaterally, and bone granules with autologous bones chips were applied over the constructs laterally. The homeostasis was maintained, a drain was inserted, the facia was closed with vicryl 2, and the subcutaneous closure was performed using vicryl 0/2. The skin was closed with staplers, and a dressing was applied.2.5The specimen sent for pathological examination consisted of two soft and gritty fragments, measuring 0.4 to 0.6 mm, that were entirely submitted in one cassette following decalcification.The histopathological study of the specimens showed cellular proliferation of oval mononuclear cells characterized by abundant pale cytoplasm, irregular and elongated nuclei with prominent nuclear grooves and folds, fine chromatin and indistinct nucleoli. The cells appeared to infiltrate the bone. Occasional multinucleated giant cells were identified. The background showed mixed inflammatory cell infiltrate composed of neutrophils, eosinophils, plasma cells and lymphocytes. There was no evidence of brisk mitotic activity or necrosis. By immunohistochemical sains, the cells are positive for CD1a, langerin, S100 and CD68. The features were consistent with LCH.2.6The patient was diagnosed as a case of LCH with symptomatic spinal and asymptomatic pituitary involvement. Following surgery, positron emission tomography (PET)/ computed tomography (CT) scan of the spine was performed and showed an increased uptake in the T6 vertebra . There was also noted an increase in the right parotid gland uptake, representing LCH involvement of the gland. An increased uptake was also noted in the renal cortex, indicating resolving acute kidney injury (AKI). Therefore, the patient was recommended to receive few cycles of low intensity systemic chemotherapy such as vinblastine,6-mercaptupurine, or low dose methotrexate.3Vertebral LCH is not uncommonly reported in children ,8,9. In Langerhans cell histiocytosis of the spine presents with a wide range of clinical symptoms spectrum ranging from asymptomatic to severe neurological deficits due to myelopathy and cord compression . AsymptoFew cases of spinal LCH in adults were reported in the literature, affecting either the cervical, the thoracic, or the lumbar regions. Similar to our case, thoracic LCH was reported in several adult cases in the literature, with different presentations and treatment strategies. For instance, Lim and Hwan  reportedKouhen et al.  have alsKim et al.  in 2017,Cervical LCH was also reported in adults. Sang-Deok Kim et al.  reportedFewer cases of LCH in adults were reported to affect the lumbar spine. For instance, Feng et al.  in 2012,Hassan et al.  also repAs discussed, different treatment strategies exist in the literature for spinal LCH. To date, there are no guidelines or consensus recommendations for the optimal treatment strategy for spinal LCH . TreatmeThe prognosis of spinal LCH is also variable based on the clinical presentation, number and sites of lesions, systemic involvement, and patient's characteristics . In patiWritten informed consent was obtained from the patient for the 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.Baghdad hospital exempts ethical approval and supply the informed written consent for this case report.There were no sponsors for this case report.N/A.All authors equally contributed to the analysis and writing of the manuscript.Md Moshiur Rahman.The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.


Duodenal adenocarcinoma (DA) is a rare tumor. We present the case of an 84-year-old lady who presented with episodic emesis with progressive dysphagia to solids and liquids. She also noted a significant weight loss of 31kg over four months. She was reported to have multiple brain masses three months before this admission. A computed tomography (CT) scan showed a heterogeneous mass (8cm) in the left retroperitoneum, inseparable from the duodenum. Additional peritoneal nodules and enlarged retroperitoneal lymph nodes were suspicious for metastases. Esophagogastroduodenoscopy revealed extrinsic compression of the stomach by the tumor. A large friable distal duodenal mass (fourth part) partially obstructed the lumen, which was biopsied. Pathology results demonstrated high-grade dysplasia but did not confirm malignancy. The patient's carcinoembryonic antigen (CEA) was elevated, but cancer antigens (CA)125 and CA19-9 were normal. A percutaneous biopsy of the mass revealed enteric-type adenocarcinoma. Immunohistochemistry showed that the tumor was positive for caudal-type homeobox (CDX)2, negative for special AT-rich sequence-binding protein (SATB)2, and patchy positive for cytokeratin (CK)7 and CK20 staining. The collective evidence suggested a duodenal primary. The patient opted for hospice and died in three days. We lack pathological evidence, but the patient's brain masses were suspicious of metastases. This would be one of the few reported cases of DA with possible brain metastases.  Small intestinal cancers are rare. The most frequently involved segment is the duodenum. Duodenal adenocarcinoma (DA) accounts for 0.5% of all gastrointestinal (GI) tumors . The incAn 84-year-old lady presented with lethargy and emesis for two days. She was experiencing dysphagia for two weeks, progressing from solids to liquids. It worsened to postprandial emesis with every meal. She also noticed an unintentional weight loss of 31 kg over four months. She denied any current or prior use of cigarettes and alcohol. Her family history was significant for breast cancer in her mother.Three months before this admission, she had a stroke. Magnetic resonance imaging (MRI) brain revealed acute frontal and midbrain infarcts. Incidentally, several enhancing masses were seen throughout the supratentorial and infratentorial brain. A mass in the left parafalcine parietal lobe at the vertex measured 2 x 1.5 x 1.8 cm . These include 1) declining CRC incidence and rising SBA incidence, 2) worse prognosis of SBA patients than CRC patients with a five-year survival of 35% and 51.5 %, respectively, and 3) differences concerning the percentage of genetic driver mutations such as tumor protein (TP) 53 (58.4% in SBA vs. 75% in CRC), adenomatous polyposis coli (APC) (26.8% vs. 75.9%) and cyclin-dependent kinase Inhibitor (CDKN) 2A (14.5% vs. 2.6%), leading to potential therapeutic implications -14. The The association of CA 19-9 and CEA is not well established in DA. However, recent data suggest that an elevation of both markers is associated with worse overall survival and higher prognostic impact of CA 19-9 than CEA . In thisThe treatment options for DA comprise surgical, endoscopic, chemoradiation, and immunotherapy approaches. The lymph node metastases occur when DA extends deeper than the submucosal layer. There is no evidence showing the prognostic effect of lymph node dissection. Peripheral lymph node dissection may be considered if it can be safely resected . PancreaThis is a rare case of DA with possible brain metastasis. The primary was identified using histopathological, radiological, and endoscopic techniques. The IHC panel was used to differentiate it from CRC. DA is difficult to diagnose early, and the treatment options are still in developmental stages due to the tumor's rarity.


Dermoid cysts are benign developmental anomalies that can occur anywhere along the neuroaxis or embryonic lines of fusion. While intracranial dermoid cysts at the midline frequently have an associated nasal or subcutaneous sinus tract, it is quite rare to encounter an intracranial dermoid cyst off the midline with a lateral sinus tract. Standard practice for the treatment of dermoid cysts is surgical resection to minimize the risks of meningitis, abscess, mass effect, neurologic deficit, and/or death. A 3-year-old male with a history of DiGeorge syndrome presented with right orbital cellulitis and a right-sided dermal pit. Computed Tomography (CT) imaging demonstrated a dermal sinus tract with an associated lytic bone lesion within the right sphenoid wing and posterolateral orbital wall with intracranial extension. The patient was taken to the operating room in conjunction with plastic surgery for resection of the dermal sinus tract and intraosseous dermoid. This case presents a rare occurrence of a non-midline, frontotemporal dermal sinus tract associated with a dermoid cyst with intracranial extension presenting with pre- and post-septal orbital cellulitis. Important considerations include preservation of the frontal branch of the facial nerve, preservation of orbital structure and volume, complete surgical resection to prevent infectious complications including meningitis, and a multidisciplinary surgical approach with plastic surgery, ophthalmology, and/or otolaryngology.  Dermoid cysts are benign developmental anomalies originating embryologically from the entrapment of ectodermal elements consisting of a stratified squamous epithelium lining containing keratin and hair . IntracrHistoryThe patient presented as a 3-year-old male with a past medical history including DiGeorge syndrome, single cardiac ventricle anatomy following aortic reconstruction, Glenn, and Damus-Kaye-Stansel procedures, heart block with pacemaker, T-cell lymphopenia, and gastrostomy tube dependence, in addition to developmental delay and intellectual disability. He presented to the emergency department with a fever and 24 hours of progressively worsening swelling and erythema of the right eye in addition to mild proptosis concerning for orbital cellulitis. Of note, pupils were equal in size and reactive to light and the patient was without obvious visual field deficit. Interestingly, the parents reported a history of a skin dimple over the right pterion that has been present since birth that intermittently extruded hair and purulent material Figure .The patient was febrile on presentation (38.3°C) with associated leukocytosis (14.65 thousand). Other pertinent laboratory data included C-reactive protein (CRP) of 5.0 mg/dL , erythrocyte sedimentation rate (ESR) of 18 mm/h , and neutrophil count 10.66 thousand . Blood cultures obtained in the emergency department demonstrated no growth, though antibiotics were started prior to the acquisition of blood cultures. The patient was also started on intravenous steroids in addition to intravenous vancomycin and ceftriaxone for broad-spectrum antibiotic coverage for presumed orbital cellulitis. Preoperative evaluationGiven his history of cardiac pacemaker placement, computed tomography (CT) imaging of the head and orbits with and without contrast was obtained as he was not a candidate for magnetic resonance imaging (MRI). The obtained images demonstrated an intraorbital, extraconal rim-enhancing fluid collection along the superolateral wall of the right orbit measuring approximately 1.4 x 1.4 x 0.5cm immediately adjacent to a lytic bone lesion within the right sphenoid wing/posterolateral orbital wall with intracranial epidural extension anterior to the right temporal pole. This was associated with a circuitous cutaneous tract extending through the right temporal soft tissue Figure .Considering the complex presentation, a multidisciplinary team was assembled to review the case and develop a surgical plan including neurosurgery, plastic surgery, ophthalmology, infectious disease, otolaryngology, and critical care medicine. Given the concern for an infected intracranial/intraorbital dermoid cyst and orbital abscess, the decision was made to proceed with a right craniotomy for resection of the dermal sinus tract and sphenoid mass. Operative treatmentThe patient was taken to the operating room in conjunction with pediatric plastic and reconstructive surgery. The frontal branch of the facial nerve was percutaneously mapped based on surface landmarks and found its course in close proximity to the deeper portion of the tract. Considering this, preoperative discussion with parents included the risk of transient neuropraxia versus permanent frontal branch palsy. An incision was made circumferentially around the sinus tract and blunt dissection with limited cautery was used to dissect along the tract while minimizing the risk of injury to the facial nerve. Dissection was carried through the subcutaneous tissue and temporalis muscle down to the periosteum. At this point, a coronal incision was made Figure  and the Postoperative coursePorphyromonas endodontalis. Infectious disease initiated a six-week course of intravenous ceftriaxone, daptomycin, and oral metronidazole. He recovered appropriately and was discharged home on postoperative day seven in stable condition. The patient was monitored in the intensive care unit. Post-operative CT imaging demonstrated resection of the mass and anatomic cranial and orbital reconstruction. The patient did have transient neuropraxia of the right frontalis muscle following the operation. Intraoperative cultures revealed gram-positive cocci on gram stain though no growth was observed. Subsequent broad-range PCR demonstrated positivity for Pathologic analysisPathologic analysis of the tract and mass revealed “portion of skin and underlying tissue with a squamous epithelium lined tract” and “ruptured and inflamed structure with focal presence of squamous epithelium and keratinous debris with associated foreign body type giant cell reaction” Figure . Dermoid cysts are benign inclusion cysts that arise from ectodermal tissue and may be associated with a dermal sinus tract . Dermal The presentation of dermoid cysts varies from the discovery of a benign cutaneous pit with extrusion of keratinous debris to local or systemic infection. These masses can also rupture and, depending on the location, lead to chemical meningitis secondary to the inflammatory nature of fat and keratin. Patients may also exhibit cerebrospinal fluid leakage from the sinus tract, and there have been reports of cranial nerve palsy caused by sequelae of dermoid cysts and associated sinus tracts . As the The presence of dermoid cysts can be confirmed with skull radiographs, CT, and MRI. Dermoid cysts are typically osteolytic with well-defined, sclerotic margins on skull radiographs. CT imaging demonstrates hypodense lesions since the cysts typically contain fat. On MRI, dermoid cysts are hypointense on T1-weighted imaging and T2-weighted imaging and can exhibit marked diffusion restriction. These lesions typically do not contrast enhance. Dermoid cysts may also be associated with developmental anomalies such as Klippel-Feil anomaly, encephaloceles, coccygeal sinus, and venous sinus anomalies . Given tThe treatment of choice for dermoid cysts is surgical resection . A multiAnother important consideration during the resection of frontotemporal dermoid cysts is the preservation of the frontal branch of the facial nerve. Before making an incision, the frontal branch can be mapped using cutaneous landmarks or using compound muscle action potentials . FurtherStaphylococcus aureus [Actinomyces, Peptoniphilus, Propionibacterium species [Escherichia coli [Porphyromonas species, in addition to Finegoldia, Campylobacter, and Bacteroides [Porphyromonas in our patient was of a different species, however. Infectious complications include posterior fossa abscess, ventriculitis, meningitis, osteomyelitis, orbital cellulitis, and epidural abscess [Pathogens associated with dermal sinus tract infections have been documented in prior case reports. These include s aureus , Actinom species , and Eschia coli . There hteroides . The gen abscess  as seen In this report, we document a rare occurrence of a non-midline, frontotemporal dermal sinus tract associated with a dermoid cyst with intracranial extension presenting with pre- and post-septal orbital cellulitis. These lesions commonly go unnoticed until severe symptoms arise as was the case in this patient. Prompt recognition and surgical resection of these masses are the sole means of treatment, thereby minimizing the risk of potentially life-threatening intracranial and orbital infections.


Spheno‐orbital meningiomas (SOMs) are complex tumors that grow and extend into nearby structures. SOM tumor growth is often associated with hyperostosis caused by tumor cell infiltration and bone alteration. We describe the case of a 64‐year‐old man with SOM that extended into the sphenoid sinus without a direct connection between the intracranial and extracranial lesions. This report emphasizes the importance of identifying the growth patterns of SOMs and assessing the paranasal sinuses adjacent to the hyperostotic orbit walls from preoperative images. Magnetic resonance imaging (MRI) revealed an en plaque tumor along the greater wing of the left sphenoid bone and periorbita, extending into the temporal muscle. The lesion in the orbit compressed the orbital components and adjacent hyperostotic bones. Moreover, the MRI revealed a lesion in the sphenoid sinus separated from the intracranial and intraorbital lesions by thickened bone Figure . ComputeA temporoparietal galeal flap was prepared for reconstruction under general anesthesia following a left coronal skin incision. Subsequently, a left frontotemporal craniotomy was performed. The intact orbital rim was removed, and the left anterior skull base and middle cranial base were epidurally exposed. After cutting the meningo‐orbital band, the bone around the optic canal and superior orbital fissure (SOF) was drilled away. The hyperostotic bone was removed to the maximum. The sphenoid sinus lesion was excised post the removal of the hyperostotic planum sphenoidale. No direct connection to the intracranial or intraorbital lesions was found. The intraorbital lesion was resected from the orbital compartment and the intracranial and temporal muscle lesions were thoroughly removed. After tumor resection, the dural defect was repaired using the femoral fascia lata. The lateral and superior walls of the orbit were reconstructed using titanium mesh fixed to the orbital rim Figure . The bonThe patient's visual acuity deteriorated immediately after surgery but gradually improved. The patient was discharged without any neurological deficits or CSF leakage 1 week post the operation. No tumor recurrence was observed even 6 months after surgery.Histopathological examination of the lesions in the sphenoid sinus and intracranial, intraorbital, and temporal muscles revealed a meningothelial meningioma with a MIB‐1 labeling index of 1%.3SOMs are a unique subgroup of meningiomas that exhibit a sheet‐like appearance and are associated with hyperostosis.The management of SOMs presents a challenge for clinicians because of the tumor's potential involvement of critical structures, such as the nerves in the SOF or intraorbital muscles, as well as the high recurrence rates associated with incomplete resection.Proper management of skull base defects and dural closure after aggressive surgery for SOMs is crucial to prevent postoperative complications such as CSF leakage, which can be life‐threatening. However, achieving a watertight dural closure with a fascial patch graft can be challenging. Although free fat grafts may seem to be a convenient option, they have a low tolerance for infection. In this case, a vascularized temporoparietal galeal flap was used to cover the skull base defect because it is a well‐vascularized tissue that provides homogeneous thickness, making it a suitable option for skull base reconstruction. This technique is essential to eliminate dead space, ensure the sealing of the skull base, and prevent postoperative CSF leakage. The galeal flap adheres to the dural surface within several days, making indwelling lumbar spinal CSF drainage unnecessary.Orbital wall reconstruction after SOM surgery is crucial for restoring normal orbital volume and preventing ocular complications.The present case report has some limitations. Firstly, the case report represents a single case, limiting the findings' generalizability. Additionally, the lack of long‐term follow‐up data is a limitation, as it prevents us from providing more comprehensive information on the patient's outcomes and tumor recurrence. Lastly, a longer follow‐up period would have provided more robust information on long‐term outcomes and tumor recurrence rates.In conclusion, preoperative assessment of the paranasal sinuses adjacent to hyperostotic orbital walls in SOMs may contribute to accurate diagnosis and appropriate treatment planning by identifying signs of extracranial tumor invasion. Additionally, tailored surgical management, including reconstruction techniques may achieve optimal outcomes for patients with SOMs.Toshihito Maki: Data curation; formal analysis; writing – original draft; writing – review and editing. Eiji Ito: Data curation; formal analysis; project administration; writing – original draft; writing – review and editing. Kiyoshi Saito: Conceptualization; data curation; supervision; writing – original draft; writing – review and editing. Ryuta Saito: Supervision; writing – original draft; writing – review and editing.This research received no specific grants from any funding agency in the public, commercial, or not‐for‐profit sectors.The authors received no financial support for this research and have no conflicts of interest to report regarding the materials and methods or findings of this paper.This study was performed in accordance with the ethical standards of the Japan Neurosurgical Society.Written informed consent was obtained from the patient for the publication of this case report and the accompanying data and images.


Teratomas are the most common benign ovarian neoplasms in young women. Typical computed tomography imaging findings include fat, fat fluid level, tooth or calcification, rokitansky nodule, floating balls sign, and tufts of hair. They can have unusual imaging features leading to diagnostic dilemmas. Studies have shown the presence of intratumoral fat to be specific to ovarian cystic teratoma. However, there are reports in the literature of mature cystic teratoma that do not contain fat in the lumen of the cyst which can hinder an accurate diagnosis. They can be associated with various complications like torsion, rupture, malignant transformation, infection, and autoimmune hemolytic anemias. Presented here is a case of mature cystic teratoma without visible intracystic fat which underwent torsion. Mature cystic teratoma (MCT) is a common neoplasm of the ovary in young women with a wide variety of presentations ranging from pure cystic mass to complex solid cystic mass. The detection of intratumoral fat is the key diagnostic imaging feature. Frequently associated complications include torsion and malignant transformation A 57-year-old previously healthy woman was admitted with complaints of lower abdominal pain since the previous day. She had a single episode of vomiting. There was no history of fever, bleeding per vaginum, loose stools, or food intake from outside. She was a known case of hypertension and type 2 diabetes mellitus on treatment. She had attained menopause 7 years back. On abdominal examination tenderness was elicited in the left iliac fossa.The patient was evaluated using ultrasonography (USG) and contrast-enhanced CT. On ultrasound evaluation, the left adnexa of the patient showed a well-defined cystic lesion measuring 8.5*6.9*6.5 cm with multiple fine internal echoes and chunky calcifications along its dependent part measuring 17*8 mm. Mild free fluid was noted in the left iliac fossa. No definite internal or peripheral vascularity could be elicited.The patient was then assessed by contrast-enhanced CT study. CT images revealed a well-defined large abdominopelvic cystic lesion measuring 9* 7* 7.7 cm noted towards the left side of an atrophic uterus seemingly arising from the right ovary . The rigThe patient underwent a total abdominal hysterectomy, bilateral salpingo-oophorectomy, and omental biopsy where a right ovarian cystic mass of size 8*8*7 cm was obtained which was twisted 2 times around the pedicle. The left ovary and uterus were found to be normal. Histopathological examination showed a mature ovarian cystic teratoma of 9*9*7 cm in size with evidence of torsion .Fig. 5HiMature cystic teratomas (MCT) are germ cell tumors believed to arise from the primordial germ cell. Around 20%-25% of ovarian neoplasms are contributed by ovarian germ cell tumors . BilaterOvarian mature teratomas have a wide variety of imaging features. A good knowledge of radiological presentations of teratomas can help arrive at an accurate diagnosis. Though ultrasonography is often the first used imaging modality for the assessment of pelvic genital organs, the diagnosis of MCT may be difficult by the sole use of USG as they can have nonspecific appearance. However, a thorough search for characteristic signs such as rokitansky nodule, diffuse or regional high amplitude echoes, tip of the iceberg sign, dot–dash sign, fat fluid level or fluid-fluid level, floating balls sign and comet tail appearance should be undertaken The case presented here proposed a diagnostic dilemma since no evidence of intracystic fat could be demonstrated. Yamashita et al reported 12 cases of MCT without fat in the cystic component CT is better than MRI to visualize small fatty areas (mean diameter about 1 mm) which can be explained by a better signal-to-noise ratio in CT, thinner slices in CT, and magnetic field inhomogeneities in the boundary of the calcified structures Torsion and malignant degeneration constitute the 2 major complications of MCTs. Other complications which occur less frequently are tumor rupture and ovarian vein thrombophlebitis. Findings suggestive of torsion include deviation of the uterus to the twisted side, engorged blood vessels on the twisted side, engorged blood vessels on the twisted side, a mass with high sign intensity rim on T1- weighted MR images, a low signal intensity torsion knot, and thick, straight blood vessels that drape around the mass and cause complete absence of enhancement There are many challenges faced by gynecologists in deciding upon the best surgical management for ovarian MCTs with the laparoscopic approach being considered as the gold standard for the management. The standard operation is oophorectomy except in younger women with a single small cyst Written informed patient consent for publication has been obtained


The incidence of congenital neuroblastoma has increased in the recent years. The purpose of this study was to describe the clinical and biochemical characteristics of cases of congenital neuroblastoma diagnosed in our center.N-MYC oncogen was not amplified in any of the cases studied. Histopathological analysis was favorable in the three cases. The tumor was resected in two patients. The three received chemotherapy.We report three cases of congenital neuroblastoma diagnosed in our hospital. In two, diagnosis was made prenatally, whereas the other case was detected in the immediate neonatal period. In the three cases, neuroblastoma was located in the abdominal region and exhibited elevated concentrations of catecholamines or their metabolites in single voided urine samples. Two tumors were classified as stage M, and one as stage L2. The The measurement of catecholamines and their metabolites is essential in the diagnosis of neuroblastoma. When 24 h urine cannot be collected, single voided urine can be used to calculate the index based on creatinine concentrations. Neuroblastoma (NBL) is the most frequent extracranial solid tumor in childhood, with an incidence of 10 cases per million subjects younger than 15 years. NBL accounts for 7% of all pediatric tumors, and it causes 15% of cancer-related deaths in children .Congenital NBL is defined as neuroblastoma detected during the prenatal period or within the immediate 28 post-birth days, accounting for 5% of all NBLs , 4. HencWe report three cases of congenital NBL diagnosed in our center between 2019 and 2020. An adrenal mass was identified on ultrasound in the fetus of a 29 year-old woman  at 37 weeks of pregnancy. Magnetic resonance imaging (MRI) confirmed the presence of a tumor measuring 2.8 × 2.8 × 3.6 cm in the right adrenal region suggestive of NBL. Delivery was eutocic at 40 + 6 weeks of pregnancy and a boy was born with Apgar 9/10 and 3,360 g of weight. Postnatal ultrasound confirmed the presence of a mass measuring 5 × 3.3 × 3.6 cm.® 1100  system using commercially available Recipe® kits . Elevated levels were observed for norepinephrine (NA)/creatinine; normetanephrine (NMN)/creatinine; homovanillic acid (HVA)/creatinine; and vanillylmandelic acid (VMA)/creatinine ratios (131I-meta-iodobenzylguanidine (MIBG) confirmed the presence of pathological chromaffin cells within the mass, with multifocal bone and liver involvement.Single voided urine samples were collected for 3 days to determine concentrations of catecholamines and their metabolites by high-resolution liquid chromatography (HPLC) on an Agilente ratios . At one e ratios . ScintigN-MYC oncogene amplification and chromosome 11q deletion. The tumor was categorized as a medium-risk stage M NBL ). Subsequently, the patient received chemotherapy (etoposide/carboplatin).Treatment was initiated with alpha- and beta-blockers due to the hypertensive crisis suffered by the patient, to perform the surgical mass resection. Resection was performed by laparotomy at two months of life. Histopathological analysis revealed a poorly-differentiated NBL with a low mitosis-karyorrhexis index (MKI). Genetic analysis ruled out both 131I-MIBG revealed the presence of pathological chromaffin cells at the level of the right adrenal gland, without other sites being involved.A newborn with a prenatal diagnosis of Noonan syndrome, with Apgar 8/10 and a weight of 3,050 g. A right-sided adrenal mass was identified on ultrasound, consistent with congenital NBL. The analysis of single voided urine revealed elevated levels of catecholamine metabolites, especially for the NMN/creatinine ratio . MRI conN-MYC amplification and chromosome 11q deletion. The tumor was classified as low-risk stage L2 (INRGSS). The patient received chemotherapy (etoposide/carboplatin).Histopathological analysis revealed a poorly-differentiated NBL with a low MKI. Genetic analysis showed the absence of both A left-sided adrenal mass and agenesis of the corpus callosum were identified on ultrasound in the fetus of a 35 year-old woman  at 34 weeks of pregnancy. Fetal MRI showed a tumor measuring 2.9 × 2.3 × 3.2 cm. Delivery was eutocic at 39 weeks of pregnancy and a boy was born with Apgar 9/10 and 3,130 g of weight. Postnatal ultrasound revealed a mass at the level of the left hypochondrium measuring 5.3 × 3.5 × 4 cm consistent with NBL.N-MYC oncogene amplification and chromosome 11q deletion. It was classified as medium-risk Stage M neuroblastoma (INRGSS).Determination of catecholamines and their metabolites showed slightly elevated NMN/creatinine, HVA/creatinine, and VMA/creatinine ratios in serial single voided urine samples . At 3 daAt 1 month after birth, chemotherapy was initiated (etoposide/carboplatin), which reduced the size of the left adrenal mass, with a favorable response of liver, subcutaneous, and muscle lesions. At 4 months of life, tumor resection was performed.Diagnosis of NBL is established based on the determination of catecholamines and their metabolites in urine, histophatological analysis and imaging studies . TraditiA limitation to the analysis of catecholamine metabolites in urine in pediatric patients is the difficulty in obtaining 24 h urine samples. When 24 h urine cannot be collected, single voided urine can be used to calculate the index based on creatinine clearance. It should be taken into account that creatinine clearance is influenced by muscle mass and increases with height, which makes it necessary that an appropriate range of reference is established for each age , 11.N-MYC gene amplification or chromosomal aberrations.The International Neuroblastoma Risk Group developed the INRGSS to classify this type of tumors based on imaging studies previous to surgery. This group identified other prognostic factors associated with disease severity, namely, age at diagnosis (>18 months), histology, level of differentiation and cytogenetic and molecular characteristics . None ofNBL embraces clinically heterogeneous tumors with different behaviors. Correct diagnosis will determine the therapeutic approach, which may range from an expectant attitude to intensive chemotherapy and surgery , 2. Earl–Congenital NBL is a rare entity that should be considered in the presence of an adrenal solid mass on prenatal ultrasound.–Upon prenatal suspicion of NBL, performing a biochemical analysis in the immediate postnatal period facilitates early diagnosis. Catecholamine metabolite determination in urine is one of the most reliable diagnostic methods.–When 24 h urine cannot be collected, catecholamine metabolites can be determined through the analysis of a single voided urine sample by correcting metabolite values with creatinine and using reference values adjusted for age.


Whereas renal cell carcinoma (RCC) is the most common renal tumor in adults, pediatric RCC is a rare malignancy. The previous literature focusing on cross-sectional imaging of RCC concerns mainly computed tomography in adults, whereas in children, a different distribution of subtypes is seen, as well as a preference for magnetic resonance imaging (MRI). Therefore, the aim of this study was to identify MRI characteristics of pediatric and young-adult RCC through a case series and literature review focusing on translocation-type RCC (MiT-RCC) and the pediatric and young-adult population. In our review as well as in our case series T2-weighted hypo-intensity seems to be a potential discriminative characteristic. Moreover, an irregular growth pattern and limited diffusion restriction were often described. Nevertheless, we conclude the discrimination of RCC subtypes, and especially the differentiation of RCC from other pediatric renal tumors, remains difficult.3 (29–2191 cm3). Five tumors had a hypo-intense appearance on T2-weighted imaging, whereas 4/6 were iso-intense on T1-weighted imaging. Four/six tumors showed well-defined margins. The median apparent diffusion coefficient (ADC) values ranged from 0.70 to 1.20 × 10−3 mm2/s. In thirteen identified articles focusing on MRI characteristics of MiT-RCC, the majority of the patients also showed T2-weighted hypo-intensity. T1-weighted hyper-intensity, irregular growth pattern and limited diffusion–restriction were also often described. Discrimination of RCC subtypes and differentiation from other pediatric renal tumors based on MRI remains difficult. Nevertheless, T2-weighted hypo-intensity of the tumor seems a potential distinctive characteristic.Pediatric renal cell carcinoma (RCC) is a rare malignancy. Magnetic resonance imaging (MRI) is the preferred imaging modality for assessment of these tumors. The previous literature has suggested that cross-sectional-imaging findings differ between RCC and other pediatric renal tumors and between RCC subtypes. However, studies focusing on MRI characteristics are limited. Therefore, this study aims to identify MRI characteristics of pediatric and young-adult RCC, through a single-center case series and literature review. Six identified diagnostic MRI scans were retrospectively assessed, and an extensive literature review was conducted. The included patients had a median age of 12 years (63–193 months). Among other subtypes, 2/6 (33%) were translocation-type RCC (MiT-RCC) and 2/6 (33%) were clear-cell RCC. Median tumor volume was 393 cm Pediatric renal cell carcinoma (RCC) is a rare renal malignancy ,2. AlthoMagnetic resonance imaging (MRI) is currently the preferred modality for the assessment of pediatric renal tumors within the SIOP-RTSG given its lack of ionizing radiation and excellent soft-tissue contrast. Furthermore, MRI is subject to continuous technical developments, such as the possibility of calculating the apparent diffusion coefficient (ADC) value using diffusion-weighted imaging (DWI) ,11,12. MContrary to the rarity of RCC in children, this tumor type is the most common renal tumor in adolescents and adults ,19,20,21Until today, studies focusing on the MRI characteristics of pediatric RCC are limited in number, although identification of potential specific MRI characteristics of WTs and non-WTs is important for future validation studies ,25,35. TInstitutional Review Board approval was obtained. For this retrospective study, obtaining further formal consent was waived. All diagnostic MRI scans included were clinically indicated and were performed as the standard of care. Between 2014 and 2019, we identified 6 children with RCC that underwent an MRI scan at diagnosis.The standard of care for localized pediatric RCC is upfront total nephrectomy ,36. OnlyAbdominal MRI for pediatric renal tumors in this study was performed using a 1.5T MRI system . Two patients were scanned in external hospitals at diagnosis before referral to our center . Scan protocols slightly varied but at least consisted of coronal and axial T2-weighted imaging, axial T1-weighted turbo spin-echo and axial DWI with automatically generated ADC maps. Five patients underwent pre- and post-contrast T1-weighted imaging, whereas for one patient, contrast-enhanced MRI was not available .Children were awake, sedated or under general anesthesia depending on their ability to cooperate, according to the standard-of-care procedures. Gadobutrol  was administered intravenously at a dose of 0.1 mL/kg body weight. Hyoscine butylbromide  was administered intravenously at a dose of 0.4 mg/kg body weight to reduce peristaltic artifacts, with a maximum of 10 mg in children ≥6 years and a maximum of 5 mg in children <6 years. All children were screened for contraindications for MRI and those concerning intravenous agents. For the two patients scanned at local hospitals, specifications of gadobutrol and hyoscine butylbromide were not available.The anonymized MRI datasets were transferred to DICOM software Osirix v. 5.5.2 . Two pediatric radiologists , who were blinded to the histopathological subtype and clinical characteristics but were aware of the pediatric RCC diagnosis, reviewed the diagnostic MRI scans. All diagnostic scans were assessed using a case report form based on previous studies identifying potential specific imaging characteristics of different pediatric renal tumors . The pedOur national coordinating SIOP-RTSG histopathologist  reviewed the available macroscopy and microscopy from the surgically resected tumors and biopsies of all patients following the most recent WHO classification system ,37.Following protocol, the dorsal and ventral side and hilar region of the resected specimen were marked with varying color dyes following the instructions of the involved surgeons. The specimens were sliced free-handed in successive 10 to 20 mm transverse macroscopic slices in a cranial to caudal sequence or through longitudinal incision to bivalve the specimen.Due to the small number of patients, inter-observer agreement between the two pediatric radiologists was difficult to assess because Cohen’s kappa is affected by the prevalence of the finding under observation. Only six patients were included in this study, potentially resulting in low values or even an impossible calculation of kappa when focusing on separate characteristics ,39. TherA literature review was performed following PRISMA guidelines to reflect on the case series and elaborate on the current knowledge about the MRI appearance of RCC by focusing on the predominant histological subtypes in the pediatric and young-adult population. For this purpose, PubMed, Embase/Medline and Cochrane libraries were searched in November 2021, using the main search terms ‘renal cell carcinoma’ and ‘magnetic resonance imaging’ . The stuArticles were included when they (1) included MRI characteristics of patients with proven RCC; (2) were prospective or retrospective cohort studies, randomized controlled trials or case reports; (3) were written in the English language; and (4) were available in the full-text form. Subsequently, articles focusing on children (<19 years), potentially also including adolescents or young adults (≤35 years) and articles focusing on MiT-RCC were separated to serve as the focus of this literature review. Given the rarity of studies focusing on the MRI characteristics of MiT-RCC, articles focusing on adults were also included for this purpose. With this approach, we guaranteed identification of all relevant articles while subdividing their relevance for our study based on their full-text content. After removal of duplicates, 7012 articles were screened based on title and abstract, leaving 363 articles for full-text screening, resulting in the inclusion of 95 articles. Of these, 13 articles focused on pediatric, adolescent and young-adult RCC, and 13 articles focused on MiT-RCC, with an overlap of 6 articles . In NoveThe six identified patients in our center had a median age of 12 years (range 63–193 months) . Four paThe average post-operative specimen weight was 898.6 g (range 210–2100 g), whereas the maximum post-operative tumor diameter ranged from 2.4 to 12.9 cm (median 6.8 cm). The post-operative weight of the specimen was missing for one patient, of which the largest tumor diameter was 9.5 cm .Five patients were tested for MiT-RCC, resulting in 2/5 MiT-RCC cases . In 4/5 The median observed agreement between the two observers was 83% (range 33.3%–100%). The few imaging characteristics with low observed agreement were discussed between the two radiologists, and mismatching concepts were resolved . Further3, with varying locations. The shape of the tumors was predominantly lobulated (4/6), and margins were well-defined in a majority of the patients (4/6). Capsule rupture was seen in only 2/6 cases, which was defined as an interruption of the hypo-intense capsule of the tumor. None of the cases presented with a tumor thrombus. Concerning hemorrhage and necrosis, these components were present in 3/6 and 1/6 cases, respectively. Cysts were present in 2/6 cases, whereas fatty tissue and subcapsular fluid were not observed (Tumor volume ranged from 29 to 2191 cmobserved .The tumors presented mainly homogeneously (4/6), with a predominant hypo-intense appearance on T2-weighted imaging and iso-intense appearance on T1-weighted imaging. Almost all cases showed a homogeneous enhancement pattern, varying from mild to strong enhancement . There w−3 mm2/s. The MiT-RCC cases and the case diagnosed as ccRCC but with inconclusive TFE results showed the lowest ADC values, ranging from 0.70 to 0.98 × 10−3 mm2/s  . Therefo−3 mm2/s .We identified thirteen studies focusing on MRI findings of pediatric RCC, with a total of 25 patients , Table 3The location of all reported pediatric RCC tumors in the identified articles varied from central to peripheral . On T1-wRegional lymph node involvement and/or metastases to lymph nodes were reported in five studies  19,24,4,43,51. IConcerning MRI characteristics of RCC subtypes other than MiT-RCC in children, Zou et al. reported a case of a 17-year-old male with von Hippel–Lindau disease with bilateral renal cysts and ccRCC  46]. Th. Th46]. Finally, RMC has been described as a very rare and malignant renal tumor, especially in children and young adults, and is often seen in RCC patients with sickle-cell traits ,51,53. NThirteen studies focusing on MRI characteristics of MiT-RCC were identified, including the six identified studies focusing on pediatric patients with MiT-RCC  13,19,2,57,58,59DWI characteristics were reported in 5 studies for a total of 23 patients ,54,55,57In our case series, the 14-year-old female patient in particular showed a typical presentation of MiT-RCC based on these findings in the previous literature. The tumor showed an ill-defined tumor with capsule invasion and an infiltrative growth pattern, appearing hypo-intense on T2-weighted imaging with a relatively high median ADC value . The preThe RCC subtypes most frequently occurring in children and adolescents besides MiT-RCC are ccRCC, pRCC and chrRCC  3,18]. ,18. 3,18A retrospective study of Wang et al. focused on the MRI characteristics of 57 adult RCC patients, in which ccRCC and pRCC showed hemorrhage in 20–25% of the cases compared to no evidence of hemorrhage for chrRCC . MoreoveOliva et al. described the MRI-features of 21 pRCCs and 28 ccRCCs, concluding that pRCC typically presents with T2 hypo-intensity, whereas ccRCC typically shows T2 hyper-intensity . This fiThere seems to be a lack of specific imaging characteristics for discrimination of pediatric RCC and its subtypes based on MRI characteristics alone ,9,10. NeThe heterogeneous diagnostic appearance of our patients was in line with findings in the identified literature and with previous studies stating that RCC is often indistinguishable from WTs based on MRI characteristics alone ,71,72,73Whereas MiT-RCC is the most frequent histological subtype in children, we reported only two out of six patients with a proven TFE translocation. The MRI characteristics of these two patients were quite different from one another. MiT-RCC, similar to ccRCC, is often described as a relatively aggressive tumor in terms of growth pattern and tumor extension as well as prognosis ,79,80,81Whereas in adult RCC, the main focus is often the discrimination of histological subtypes, in pediatric RCC, discrimination from the much more frequently occurring WTs in the early diagnostic stages is of great importance ,7,9. WTsOverall, there seems to remain a lack of pathognomonic MRI characteristics for the discrimination of pediatric RCC from other renal malignancies in children, as well as for the differentiation of histological subtypes ,9,10. NeOver the past decades, differences between adult and pediatric RCC have increasingly been appreciated. Concerning imaging studies, the direct comparison of the pediatric and adult population has become even more complicated by the preference of CT in the adult population, whereas MRI has developed as the preferred imaging modality within the SIOP-RTSG ,8. NeverOur study has a few limitations, mainly based on its retrospective nature and small study population. The limited number of patients did not allow any statistical analysis or strong conclusions. Furthermore, scan parameters were inconsistent due to not as yet centralized care. Nevertheless, these cases served mainly as an illustration accompanying the literature review in this developing field of research. In this way, this descriptive study contributes to the increasing knowledge of pediatric RCC and its diagnostic presentation on MRI. Concerning the reported imaging characteristics by two independent observers, there was excellent inter-observer agreement ,40. The For a few years, MRI has been the preferred imaging modality for imaging pediatric renal tumors within the SIOP-RTSG protocol. This case series represents one of the largest retrospective reports so far, including an extensive review focusing on MRI characteristics of RCC in the pediatric and young-adult population. The reported cases showed a varying presentation of different pediatric RCC subtypes on MRI, in line with the published literature. Nevertheless, based on this study, T2-weighted hypo-intensity of the tumor has been shown to be a potential distinctive characteristic for the discrimination of RCC from other renal tumors that are prevalent at this age, especially WTs. Future studies should focus on larger study populations through international collaboration, also exploring innovative techniques such as DWI as a non-invasive biomarker.