Chordoma is a rare malignant neoplasm derived from notochordal tissue that primarily affects the axial skeleton. Almost 40% of patients have non-cranial chordoma metastases. The most common metastatic sites are the lungs, bones, lymph nodes, and subcutaneous tissue. We present a 52-year female with a history of sacral chordoma presenting with abdominal fullness, early satiety, and a palpable abdominal mass. Abdominal magnetic resonance imaging (MRI) revealed an isolated, highly vascularized, and multilobed liver mass in the left lateral segment. The mass was surgically removed using a clean surgical margin. A histological examination and immunohistochemical staining were consistent with a metastatic chordoma. Two years later, follow-up imaging studies showed a 6.5 × 4.0 × 2.0 cm right liver lesion with multiple lungs, chest wall, pleural, and diaphragmatic lesions. Microscopic- and immunohistochemical staining revealed a recurrent metastatic chordoma. Herein, we present a unique case of metastatic recurrent chordoma in the liver with the involvement of other sites. To the best of our knowledge, no other case of recurrent liver metastasis has been reported. Chordomas are rare primary bone tumors that originate from the notochord remnants . They acChordomas can be further subdivided into those not otherwise specified (NOS), chondroid chordoma, and dedifferentiated chordoma. Grossly, classical chordoma shows a gray-white lobulated cut surface, and microscopically, a chordoma shows epithelioid cells with an eosinophilic vacuolated cytoplasm and round nuclei with variable degrees of atypia. Chondroid chordomas show features of chordomas and chondrosarcomas. The degree of atypia associated with the tumor’s aggressiveness remains unclear . DediffeHerein we report a case of recurrent metastatic chordoma on the liver after the complete resection of the primary tumor and recurrent metastatic foci from the liver.A 52-year-old woman presented with a sacral mass that was excised in 1991 and was diagnosed as a chordoma at an outside institution. The surgery was complicated by the loss of bladder function and intermittent catheterization was performed. After 15 years of an asymptomatic course, she presented at our institution in December 2004 with abdominal pain and fullness, with a palpable liver mass under the left rib cage. Abdominal MRI demonstrated an isolated large mass in the left lateral segment of the left lobe of the liver. In contrast images, the mass was highly vascularized and well encapsulated . A diagnosis of a hepatic adenoma was made based on the well-encapsulated nature of the mass and its vascularity. The mass was surgically removed using a clean surgical margin. Microscopically, the lesion revealed an infiltrative growth pattern with a lobular architecture and extracellular myxoid areas. The cells were epithelioid with abundant clear to eosinophilic cytoplasm, prominent nucleoli, and vacuolated cytoplasm . Immunohistochemistry revealed that the tumor cells were positive for S100, vimentin, epithelial membrane antigen (EMA), and epidermal growth factor receptor (EGFR) . The tumor was negative for Melan A and hepatocyte-specific antigen (Hep-Par1). Based on the morphology and immunohistochemical staining, a diagnosis of a metastatic chordoma was made. Owing to her history of metastatic chordoma, the patient was scheduled to return for imaging every 6 months. The patient has been symptom-free for approximately five years. Follow-up MRI revealed a right-sided liver lesion in segment six and an enlarged left lower lobe pulmonary nodule. A complete liver ultrasound was performed, yielding a 1.2 × 1.4 cm lesion near the edge of the posterior inferior aspect of segment six . A furthIn the operating room, the right lobe of the liver was found to be markedly hypertrophic. A 6.5 × 4.0 × 2.0 cm mass was laparoscopically resected from segment six of the liver, in addition to a left lower lobe lung wedge resection ×6, a left chest wall resection ×2, a left anterior and posterior diaphragmatic sulcus resection, a left dome of the diaphragm resection with repair, and a left periaortic mass resection. Postsurgical changes and fibrosis in the pelvis and sacrum from the original neoplasm suggested a recurrence. The histopathological examination results were consistent with recurrent and metastatic chordomas. The incidence of a second metastatic recurrence in the liver is otherwise undescribed in the literature. The patient was scheduled to return for 3 months for a follow-up CT to check her right-sided nodules, in addition to monitoring for new left-sided lesions.The patient returned early for repeat imaging 20 days post-op based on a new-onset fever and the presence of flank pain post resection procedure. This CT scan shows the hemoperitoneum of the right conal fascia. The hemoperitoneum was drained, and the aspirate was sent for pathology which returned negative. At the follow-up, the patient inquired about chemotherapy to prevent the recurrence of a chordoma. She was counseled and chemotherapy options were discussed. As she did not have any active tumors, she was counseled to stick to PET scan surveillance. Follow-up imaging two months later revealed new nodules in her lungs and left supraclavicular lymph nodes; however, it was decided to delay imatinib treatment as all the lesions were <1 cm. Another set of images was obtained two months later with zero progression of the nodules. The patient was lost to follow up after the imaging set.Chordomas are malignant tumors arising from notochordal differentiation. Three types of chordomas have been documented. Of the chordomas, 95% were the NOS (not otherwise specified) type. A Chondroid chordoma has an occurrence rate of 4%, and dedifferentiated chordoma has the lowest occurrence (<1%). The most common sites of chordomas include the clivus, sacrococcygeal bones, or vertebrae .Metastatic chordomas are rare, with only 3% to 30% of cases reporting metastatic lesions ,9. SacroSmall liver metastases may resemble liver cysts. A comparison with the previous images, if cystic lesions are found upon imaging in a patient with a previous chordoma, is strongly recommended . RadioloSurgery is the most effective treatment for chordomas. It is usually difficult to obtain tumor-free margins during the initial surgery because the anatomical location of these tumors limits the ability of the surgeon to remove the entire tumor .Chordomas have shown a poor response to chemotherapy. Various chemotherapy regimens such as anthracyclines, cisplatin, and alkylating agents, have been used in advanced chordomas but have not been effective .Cetuximab and gefitinib can be considered for EGFR-positive chordomas. They both interfere with EGF signal transduction at different points. Their combination has been shown to control the disease in one case in which all other treatment options were exhausted. Their combined treatment prevented the further growth of both the primary and metastatic chordomas and even caused a slight regression over a follow-up period of 9 months. There was also an improvement in tumor-induced back pain. Cetuximab is usually well-tolerated. Acne-like skin reactions can occur and correlate with an improved treatment response. Since the drugs were administered in combination, the efficacy of each drug for chordomas must be evaluated .Anti-PD-L1 therapy in metastatic chordomas is a potential therapeutic target regardless of the level of PD-L1 expression in the primary tumor. A patient with metastatic chordoma to the lungs treated with pembrolizumab showed a 30% reduction in tumor load with a PFS duration of 9.3 months. Subsequently, the tumor further metastasized to the bone. This initial response to pembrolizumab and mutations in the PBRM1 gene warrants further exploration as a potential treatment for chordomas .Molecular studies have revealed the overexpression of PDGFR, KIT, and STAT3 receptors, suggesting their role as molecular targeting agents . A recenKinase inhibitors are a potential treatment for metastatic chordomas due to their heavy expression of several activated receptors. Multiple studies have shown 69–83% of chordomas to be EGFR positive, and EGFR has been shown to be the most active kinase in chordomas. Furthermore, the expression of c-MET was associated with a younger age of diagnosis and a better prognosis. PDGFR-α and EGFR were indicators of a poor prognosis . A largeChordomas are rare tumors with a benign course. Our research documents an unusual case of recurrent metastasis to the liver following the resection of the primary tumor and two years after liver metastasis with clean surgical margins. Although metastasis has been reported in the literature, to the best of our knowledge, our case is the first to report a recurrent liver metastasis.


Ectopic adrenal rest is a rare phenomenon usually discovered incidentally during microscopic evaluation. The most common site reported in the literature is the genitourinary system and pelvis. Ectopic adrenal rest is more common in male than in female children. The documented site for females is mainly along the broad ligament. However, only two cases of ectopic adrenal rest in the fallopian tube have been reported in the literature, showing that they are extremely rare.In this article, we outline a case of adrenal cortical rest that was discovered incidentally during a microscopic examination of the left fallopian tube after a total hysterectomy with a left salpingo-oophorectomy specimen from a 49-year-old female patient who was complaining of severe bleeding related to severe adenomyosis. Ectopic adrenal tissue (EAT) is one of the rare entities discovered by Morgagni in 1740. The usual anatomical location of the adrenal glands is above the kidneys . In the In this article, we report a case of an ectopic adrenal rest in a 49-year-old female patient, which was discovered incidentally during microscopic examination of a subtotal hysterectomy specimen that was excised due to extensive adenomyosis.The case involves a 49-year-old female nulliparous (Parity 0 + Gravida 12) with hypertension and recurrent pulmonary embolism. She presented to the emergency department complaining of menorrhagia. She had a regular cycle, but she noticed that the menses had been heavier than usual for the previous five months. The patient had a history of recurrent abortion (P0+12) with no laboratory evidence of antiphospholipid syndrome. The heavy bleeding was associated with palpitation, headache, and dizziness. She had a history of recurrent pulmonary embolism. The abdominal examination was unremarkable. The initial laboratory test showed hemoglobin of 6.5 g/dl and mean corpuscular hemoglobin of 25.5 picograms.Pelvic magnetic resonance imaging Figure  revealedBased on the clinical and radiological evaluation, subtotal hysterectomy with left salpingo-oophorectomy was performed, and the specimen was sent for histopathological examination. On gross examination, the intact specimen was composed of the uterus, left fallopian tube, and ovary. The cut section of the uterus showed a thin endometrial lining with 0.2 cm thickness. The myometrium was thickened and measured 12.5 cm × 12 cm × 5 cm. Its cut surface showed a whirly tan surface with small cystic and slit-like spaces. The fallopian tube measured 5 cm in its maximum dimension. The left ovary was atrophic.On microscopic evaluation, the hematoxylin and eosin (H&E) stain of the myometrium showed features of adenomyosis with benign atrophic overlying endometrium. Incidentally, during the examination of the fallopian tube, a well-circumscribed nodule was identified and composed of a collection of polygonal cells with distinct cellular borders. The nucleus was central and showed a normal nuclear to cytoplasmic ratio and fine granular chromatin. The cytoplasm was clear to amphophilic. All of these features were typical of the adrenal cortex’s normal appearance Figure . No atypEAT is considered an incidental histological finding with a 1% incidence among adult populations . EmbryolMicroscopically, EAT is easy to diagnose based on classic histological appearance. Immunohistochemical studies need to differentiate between the adrenal rests from metastatic clear cell renal cell carcinoma, displaced ovarian luteinized theca cells, and heterotopia of ovarian hilus cells. The adrenal cortex is typically immunoreactive for Melan-A (MART1), inhibin, synaptophysin, and calretinin and has a high nuclear positivity 86%) to antisteroidogenic factor-1 (anti-SF-1). While metastatic clear renal cell carcinoma is negative for these markers and positive for CD10, AE1/AE3, and carbonic anhydrase, the ovarian hilus cell heterotopia of the fallopian tube shows similar immunopositivity to adrenal cortical cells with the difference of the additional positivity for chromogranin, which is negative in adrenal cortical cells [6% to antWe reviewed the English literature in PubMed, Google Scholar, and Ovid using the following terms: “ectopic adrenal tissue,” “ectopic adrenal rest,” and “fallopian tube.” We found only two cases reported in fallopian tubes. The first case was reported by Tingi and Ogah in 2018 in a 48-year-old female with heavy abnormal bleeding. The patient underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy. After a microscopic examination of the left fallopian tube, they found the adrenal cortical tissue, which they designated as adrenal ectopic tissue . As in oThe second case was reported by Tzigkalidis et al. in 2021 in a 37-year-old female diagnosed with ectopic pregnancy in the right fallopian tube. They noticed a minute nodule, firm, yellowish in color, and measuring 2 mm, which they labeled microscopically as adrenal ectopic tissue. They found this nodule to be positive by immunohistochemistry for MART1, synaptophysin, calretinin, and inhibin-A. As in our case, the EAT showed immunoreactivity for the calretinin and inhibin-A .To date, the presence of EAT has no known major clinical significance and no related symptoms or functional properties have been previously documented. However, it is crucial to recognize EAT because it may develop the same pathologies that arise from the adrenal gland in its normal location, such as hyperplasia, adrenal insufficiency, and neoplastic transformation.EAT is an uncommon finding, and discovery of the ectopic tissue in the fallopian tube is extremely rare. EAT has no clear clinical significance. Nevertheless, precise evaluation should be performed to rule out other differential diagnoses and primary pathological abnormalities in ectopic tissue.


Fibroepithelial stromal polyps (FEPs) are benign tumors of the integumentary system with mesodermal origin. They are commonly found over the skin. Rarely, they can be found in the nipple. We report a case in a 62-year-old female with a large FEP emerging from the left breast nipple. It started as a “mole” 10 years ago and slowly progressed in size. The patient did not complain of pain but did report occasional bloody discharge. She has no family history of breast cancer. On physical exam, a pedunculated large polyp, with rubbery consistency, emerging from the left nipple was noted. The lesion consisted of hyper-pigmentation with scattered ulcerations and cauliflower-like growth. Surgical excision was performed and histopathologic analysis showed extensive fibrovascular tissue with dense collagen fibers deposition consistent with FEP. Surgical treatment is curative with minimal skin deformity. In order to make the diagnosis and exclude malignancy, histopathology is mandatory. Fibroepithelial stromal polyps (FEPs) or acrochordons are mesoderm-derived benign lesions. They are commonly found in the skin, oral cavity, urinary tract, and urogenital tracts. Rarely (incidence <2%), they can be located in the nipple area . FEPs haA 62-year-old female with a past medical history of hypertension presented with a growing pedunculated exophytic breast lesion, emerging from her left nipple. She stated the breast lesion started as a “mole” 10 years ago, and progressively grew in size. She described it as a nontender, nonpruritic mass, with occasional bloody discharge. She was not on hormone replacement therapy. She has no family history of breast cancer. On review of systems, she denies weight loss, fever, or chills. No other positive or negative pertinent symptoms. On physical examination, a large 3.1 x 2.6 x 2.0 cm short pedunculated polyp lesion, with rubbery consistency, emerging from the left nipple was noted. The polyp had areas of hyperpigmented fleshy-like appearance intercalated with small areas of ulceration and a cauliflower-like appearance (Figure  This report presents a 62-year-old female with a slow-growing large left breast lesion emerging from the nipple, which was ultimately diagnosed as FEP after surgical excision and histopathologic examination. FEPs, also known as acrochordon, are common benign dermal mesenchymal tumors. They are characterized by a polypoid proliferation of the stroma with overlying squamous epithelium that gradually enlarges in size ,4,5. TypFibroepithelial polyps are common skin benign tumors and rarely occur in the female nipple. Clinically, those types of benign tumors, as in this case can present as a slow-growing pedunculated tumor, with a rubbery, nodular, or cauliflower-like appearance. Occasionally, they can ulcerate and cause bloody discharge. Histologically, FEPs are characterized by hyperplastic epidermis surrounding a core of fibrovascular tissue with dense collagen fibers. Intraductal papilloma and squamous cell carcinoma are differential diagnoses. Surgical resection is curative.


In this case report we describe a rare case of chondrosarcoma of the Temporomandibular joint in a 70 years old female who presented with a right preauricular swelling, trismus and neuralgic pain. On examination, firm and tender swelling was noted in the right preauricular region. CT Scan revealed 3.48 × 3.0 cm size mass lesion in the region of mandibular condyle and extending into the right temporomandibular joint space. The cytopathological report was suggestive of chondroid malignancy. The tumor was excised and histopathological examination showed large sheets of atypical tumor cells with cartilaginous matrix and diagnosis of a well differentiated Chondrosarcoma was confirmed. Post-surgical resection, patient remains disease free at 15 months follow up. Chondrosarcoma is a malignant cartilage forming tumor . ChondroA 70-year-old female patient presented to our outpatient department with the complaints of a swelling in the right preauricular region and limitation of jaw movements due to pain for 4 years. The patient also complained of right sided headache and facial pain. On examination the swelling was approx. 3 × 3 cm in size, firm, tender and fixed to the underlying structure while the overlying skin appeared normal and mobile. Her mouth opening was significantly reduced up to one finger. There was no facial palsy or any cervical lymphadenopathy. Her routine blood investigations were normal. Fine needle aspiration cytology revealed calcification, chondromyxoid stroma and malignant cells with moderate pleomorphism and well-defined cytoplasm which was suggestive of Cartilaginous malignancy (possibility of chondrosarcoma). Plain radiograph of the skull Fig. a revealeCECT Head, Neck & Thorax Fig. b revealeA preauricular incision was given and the tumor was resected along with a part of the ascending ramus. The mandibular fossa was found to be uninvolved.The excised specimen Fig. f measure. In our patient there was no clinical or radiological evidence of metastasis or local infiltration so a simple excision without neck dissection sufficed. Post-surgical excision there has been no recurrence in 15 months follow up.Chondrosarcoma of the temporomandibular joint is a very rare occurrence , 3 and a


Chronic encapsulated intracerebral hematoma (CEIH) is a rare solid mass characterized by the presence of a fibrotic capsule that can present a variety of signs and symptoms due to the mass effect and hydrocephalus. It may be caused by post-traumatic or spontaneous bleeding as related to an adjacent aneurysm, angiomas or neoplasms. Differential diagnosis must be applied in order for it to mimic neoplasm or a vascular malformation. Several cases of CEIH have been reported but only a few of them have an intraventricular localization. A forensic autopsy of a 50-year-old male who died suddenly while driving is discussed. Gross analysis, histology and toxicology were performed and a CEIH of the right lateral ventricle was found in a case of acute coronary death. Chronic encapsulated intracerebral hematoma (CEIH) is a rare mass characterized by the presence of a fibrotic capsule that histologically resembles the outer capsule of chronic subdural hematoma. It can show a variety of signs and symptoms due to the mass effect and hydrocephalus, depending on the localization. Differently from an intracerebral hemorrhage which shows a sudden onset, CEIH often begins with progressive neurological deficits due to the mass effect . SometimThe most common differential diagnosis has to be made with brain tumors . Perifocal edema is commonly observed by imaging on both CEIH and tumors. CEIH and brain tumors show also a progressive neurological deficit . RadioloA case of CEIH as occasional finding in sudden cardiac death (SCD) is reported. A 50-year-old male, 105 kg in weight and 180 cm height  with a 30 pack-year smoking history and enalapril therapy for hypertension and no neurological symptoms or pathology, died suddenly while driving. A family history of sudden cardiac death (SCD)—father died at 55 years old for myocardial infarction—was also reported by relatives and the primary care physician. After 72 h, a forensic autopsy was performed.Histology. For each organ, histological evaluation was carried out according to the guidelines for autopsy investigation of SCD . After fToxicology. Femoral blood was analyzed for alcohol (ethanol) and volatiles by head-space gas chromatography coupled with a flame ionization detector (GC/HS-FID). Urine and bile along with brain, liver and kidney were also sampled for the toxicological analyses. All post-mortem specimens were screened for the presence of the main different classes of drugs  by immunological or chromatographic methods as appropriate. A systematic toxicological analysis (STA) was performed by the LC–MS/MS system (API 3200 triple quadrupole ABI-SCIEX) in multiple reaction monitoring (MRM) mode.At autopsy, the heart showed concentric hypertrophy consistent with a history of hypertension. Macroscopically, the heart was 420 g in weight, the transverse size (width) was 13.5 cm. The thickness of the mid-cavity free wall of the right ventricle was 0.5 cm, of the left ventricle was 1.6 cm, and of the interventricular septum (excluding trabeculae) was 1.2 cm. Valves appeared with localized thickening. Coronary sub-occlusion due to atherosclerotic plaque was found at the anterior descending branch of the left coronary. Subtle luminal narrowing and wall thickening was also observed at the right coronary artery soon after the emergence. After cross sectioning of the heart, no clear signs of ischemic myocardial lesions or scarring of myocardial areas were found.At examination the brain displayed no subdural or subarachnoid hemorrhage. An increased volume of the brain (1390 g in weight) with symmetrical hemispheres was reported. Coronal cuts of the organ were performed according to Ludwig’s dissection of brain. Lateral ventricles were dilated but there was not any transtentorial or subfalcine herniation. Lateral ventricles were found to contain blood clots. As an occasional finding, within the right lateral ventricle, next to right choroidal plexus, there was a round-shaped mass of 2 cm in diameter overlying the floor of the ventricle, near the choroid plexus. The mass was characterized by a hard, thick wall with a fibrous capsule not attached to the surrounding tissue as it was removed easily from the ependymal surface B. The maThe microscopic appearance of the anterior descending branch of the left coronary atherosclerosis showed a significant narrowing due to proliferating fibrous tissue, calcium, and fat deposition. A diffuse periadventitial lymphocytes–monocytes infiltration was also observed. Stenosis of more than 80% of the lumen was assessed. In this case of acute coronary death the heart showed also hyperosinophilic anucleated myocytes, patches of wavy fibers and contraction band necrosis consistent with early signs of myocardial ischemia.The microscopic appearance of the brain showed congestion of the choroidal plexus at both lateral ventricles A. TissueToxicological analysis showed no evidence of drugs or alcohol in bodily fluids and samples of internal organs.CEIH is a rare entity, and the exact origin is still very challenging for the pathologist ,3. It isIn the literature, several different hypotheses for the CEIH etiology have been proposed among which the formation of a hematoma in the ventricle is correlated to the degree of hemorrhage, the partial obstruction to the outflow of blood and the amount of adjacent damaged tissue. It has been suggested that chronic hematomas are due to initial bleeding of vascular malformations, such as venous angioma o cavernous angioma . VasculaIt is worth of mentioning that most bleedings related to intraventricular vascular malformations can reach the cerebrospinal fluid, developing an intraventricular hemorrhage instead of encapsulation . In our Clinically, neurological deficits such as symptoms of increased intracranial pressure can appear within weeks or months, with seizures and motor deficits. Before the modern imaging techniques of CT and MRI, skull radiography and ventriculography demonstrated only a vague hyperdensity in the brain parenchyma or ventricular system. CT may show a circumscribed intracranial lesion with mass effect. MRI will demonstrate a well-circumscribed, variably enhancing mass with T2 signal heterogeneity and perilesional edema. Given the high rates of vascular malformations in this case, a vascular imaging technique is recommended, such as MR angiography . SurgeryChronic encapsulated intracerebral hematoma is an uncommon condition that should be reported to contribute to the clarification of etiopathogenesis and the definition of related risk factors. In a case of sudden death, it is important to report findings such as this to understand its role in the mechanism of death.


Neither radiotherapy nor chemotherapy was performed after surgery, and no recurrences were observed for 3 months. Our report suggests that orbital schwannomas should be differentiated from other types of orbital tumors with sufficient evidence and that complete surgical resection remains the first choice to cure this disease.Orbital schwannomas are rare in children, especially those with intracranial extension. Herein, our report refers to a 12-year-old boy who had a cranial-orbital mass with a dumbbell-like appearance. The total neoplasms was successfully removed  Intracranial schwannomas are common and account for 8–10% of all primary intracranial tumors . In thisOur case refers to a 12-year-old boy with painless exophthalmos in the left eye. He had no relevant clinical symptoms, a previous medical history of binocular vision, and a family history of neurofibromatosis. Physical examination showed that binocular vision was normal, the eyeballs could move freely in all directions, and the eyes were sensitive to light reflection. In addition, there were no facial paresthesias or abnormal facial expression.A computed tomography (CT) scan revealed a homogeneous and non-calcified mass in the extraconal compartment of the left posterior eyeball that was similar in density to the brain parenchyma. The inner bone wall of the left orbit was thinner than before, with compressive displacement of the adjacent bone and widening of the supraorbital fissure . Magnetivia a left frontotemporal craniotomy. The solid tumor was found to be located above the eyeball and the optic nerve, which occupied the middle and posterior parts of the orbital region and extended to the anterior part of the cavernous sinus through the enlarged supraorbital fissure. The result of the intraoperative pathological diagnosis was a spindle cell tumor. Postoperatively, the patient's painless exophthalmos had completely disappeared, with no evidence of postoperative sequelae or visual impairment during a follow-up.A gross total excision was performed The mass had a biphasic pattern of Antoni A  and Antoni B (haphazardly spindle and multipolar cells are arranged loosely as a reticular structure) under a microscope. Immunohistochemical analysis showed that the neural marker (S100 protein) was firmly and positively stained, which was specific for the diagnosis. The nuclei of those cells showed positive immunoreactivity for SOX10 and less than 1% of the nuclei showed positive immunoreactivity for KI67 .via the superior orbital fissure.Schwannoma arises from Schwann cells and are known to originate from the sensory branches of the ophthalmic division of the trigeminal nerve in an orbit . SchwannSchwannomas are always benign and well-encapsulated nerve sheath tumors, with an insidious and slow growth pattern . There ivia clinical symptoms and imaging evaluation is tricky because of the variable presentation and location (Microscopically, most orbital schwannomas exhibit a histopathologic feature with a biphasic morphology, including Antoni A and Antoni B patterns . Due to location . Accordilocation . CT is olocation .We conducted keyword searches in the PubMed database over the past decade, and the sets of keywords used were associated with orbital schwannoma. According to previous related rare case reports, we summarized the common location of orbital schwannomas, which was an intraocular, orbital, retro-orbital, infraorbital, trigeminal, vagal, or trochlear mass. In part, we also found a low incidence of orbital schwannomas in children, and we believed that the rarity could be explained by the slow-growing nature of this type of tumor type in some patients presenting in early adulthood . Though In the orbital extraocular area, optic nerve glioma is the most common tumor in children , whose sUsually, the first choice for orbital schwannoma is gross total resection , 17, 18.Our case refers to a 12-year-old boy with intracranial-orbital schwannoma. Preoperative diagnosis is difficult due to its different degrees of heterogeneity. However, early assessment and prompt management are necessary to prevent the development of severe complications. We suggest that orbital schwannomas should be sufficiently differentiated from other orbital tumors in children, despite their exceedingly rare occurrence. Our case enriches the information about this disease, enhances our understanding of this tumor, and underscores the importance of complete surgical resection.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.Written informed consent was obtained from the minor's legal guardian, for the publication of any potentially identifiable images or data included in this article.YS, QX, AC, and SC performed the material preparation, image collection, and analysis. YS wrote the first draft of the manuscript. QX supervised the initial draft and critically revised the manuscript. SC and AC analyzed the case report and critically revised the manuscript. MQ corrected the grammar in the manuscript. All authors contributed to the case report conception and have read and approved the final manuscript.


Neuroendocrine tumor (NET) commonly occurs in the gastrointestinal tract, however primary NET of the liver is rare, especially during pregnancy. We present a 34-year-old pregnant woman gravida 3 para 2 at 16 weeks period of gestation with primary liver NET discovered incidentally during the antenatal check-up. She has no risk factors for hepatocellular carcinoma. Her serum alpha-fetoprotein was elevated. A plain magnetic resonance imaging (MRI) of the liver delineating a large well-defined exophytic liver mass at segment V/VI measuring 7.1 × 7.4 × 7.8 cm. Given inconclusive MRI findings coupled with low-risk factors of HCC, we had decided to follow up her liver mass with imaging 6 weekly. She then underwent a right hepatectomy with a caesarean delivery at 32 weeks of gestation in the same setting. The histopathological formal report revealed a neuroendocrine tumor, grade 2 with a Ki-67 index of 3% with negative lymphovascular and perineural invasion, but positive for porta hepatis lymph nodes metastasis. A follow up after 1 year shows both patient and her infant are healthy. Antenatal discovery of liver masses poses a diagnostic and management dilemma to clinicians. A multidisciplinary approach and collective decision making are crucial to determine the best approach tailored to the maternal and fetal benefit. In cases of inconclusive non-contrast MRI in pregnancy with low-risk factors and lack of clinical evidence of HCC, follow-up with imaging modalities aiming to intervene at the third trimester can offer safer, and promising outcomes. Neuroendocrine tumor (NET) arises mainly from the gastrointestinal tract (50%-70%) and lung 20%-30%) with a predilection to metastasize to the liver ,2. Prima0%-30% wiA 34-year-old lady gravida 3 para 2 at 16 weeks of gestation was noted to have worsening creatinine on her renal profile during a routine antenatal follow-up. She was a known case of type 2 diabetes mellitus, hypertension, and Beta thalassemia trait. She works as a school teacher and has neither risk factors for HCC nor a family history of malignancy. During the physical examination, she was not icteric, and without signs of chronic liver disease. There was a gravid uterus which was consistent with a period of gestation but no other masses were palpable. The rest of her general examination was unremarkable. The renal profile revealed a serum creatinine of 105 from 96 umol/L  with a normal serum urea level. The liver function test was normal; however, the tumor marker of serum alpha-fetoprotein was elevated with a reading of 65.5 ng/mL .Abdominal ultrasonography was subsequently performed to look at her urinary tract anatomy, however incidentally a large liver lesion was found instead. The liver lesion was noted to be heterogeneous and hyperechoic, measuring 6.4 × 6.6 × 7.4 cm and located at segment VI . A plainFollowing a discussion between the obstetric, pediatric, hepatobiliary and radiology multidisciplinary meetings, a collective decision was made to perform an elective caesarean delivery at 32 weeks of gestation as well as a right hepatectomy in the same setting considering the inconclusive MRI and feto-maternal wellbeing. MRI liver volumetry was also performed which showed residual liver of 40%. Before the operation, the risks and complications were explained in detail to both patient and her husband. She delivered a baby boy via a standard caesarean section weighing 1700 grams with an Apgar score of 7. The baby was admitted into the neonatal intensive care unit for close monitoring and weight gain. She on the other hand underwent standard right hepatectomy, after an intraoperative ultrasound revealing no other liver lesions. It had compressed the portal vein intraoperatively. The tumor measured 6 × 6 cm, was firm in consistency and had no evidence of liver cirrhosis . Her posThe histopathological formal report revealed a neuroendocrine tumor (NET), grade 2 with a Ki-67 index of 3%. It was negative for lymphovascular and perineural invasion, but positive for porta hepatis lymph nodes metastasis (1/1). Surgical resection margins and vascular stump were free from tumor involvement. Due to the rarity of a hepatic endocrine neoplasm as a primary instead of a metastatic deposit, a follow-up contrasted computed tomography (CT) scan of thorax, abdomen and pelvis was performed and did not show any recurrence or residual tumor and there were no other findings to suggest a primary neuroendocrine tumor elsewhere. She was well without any postoperative complications upon clinic review a week after she was discharged. A positron emission tomography (PET)-CT Gallium-68 DOTATATE scan was performed and revealed neither abnormal increase in Gallium-68 DOTATATE uptake at the resected liver site nor in any other organs to suggest a primary neuroendocrine tumor site. At her recent 1 year follow up postoperatively, both the patient and her son are healthy and well.Dealing with a liver mass during pregnancy, it poses not only a diagnostic dilemma but a management challenge to both obstetricians, and surgeons alike. Although most liver masses in pregnancy have been reported to be benign, the diagnosis of exclusion for malignant cancers especially HCC is vital. Previously published articles have reported an increased incidence of tumor rupture and hemorrhage of approximately 10% of HCC found during pregnancy In pregnant patients with liver masses, the choice of imaging is limited as contrast-enhanced CT is rarely used to avoid exposing the fetus to ionizing radiation during gestation. Ultrasound by default is the most used modality due to no radiation risks in evaluating liver masses in pregnancy. It has a sensitivity of 90% in identifying liver tumors and can distinguish between cystic or solid lesions, single or multiple lesions but is unable to reliably differentiate between solid liver tumors Dealing with a pregnant mother is challenging as it involves both maternal and fetal wellbeing. The decision should not be made by a single practitioner but a collective decision in a multidisciplinary team of surgeons, oncologists, obstetricians, and radiologists to determine the appropriate timing for surgical intervention of the primary problem and at the same time evaluating the potential viability of the fetus throughout the pregnancy. In view of inconclusive MRI findings coupled with low-risk factors of HCC, we had decided to follow up her liver mass with imaging 6 weekly from the initial ultrasound scrutinizing the size of the tumor which remained static without invasion to surrounding structures and monitoring her liver function tests closely. However, considering the elevated serum tumor marker and inconclusive MRI with a LI-RADS 3 which translates to a possibility of 39% being malignant, a collective multidisciplinary decision was made to surgically intervene at 32 weeks of gestation. It is an option to wait and perform a delayed operation in the third trimester when the baby has higher survival outcomes. This is because at 28th gestational week is the critical point of fetal maturation due to fetal lungs achieving sufficient maturity as well as other organs. Performing a delivery before the 28th week of gestation results in less probable survivability of the baby. In addition, delivery before 32 weeks of gestation should be avoided because of fetus immaturity.Though her final histopathology report revealed a grade 2 NET which is rare in liver as a primary site instead of a secondary deposit, her treatment was nevertheless approached in a similar fashion of NET treatment algorithm whereby ultimately a resection of the primary tumor was necessary. In fact, a quick search through PubMed reveals less than 10 reports of NETs occurring in pregnant women ie cervix and pancreas showing how rare these tumors are in pregnant patients Antenatal discovery of liver masses poses a diagnostic and management dilemma to clinicians. A multidisciplinary approach and collective decision making are crucial to determine the best approach tailored to the maternal and fetal benefit. In cases of inconclusive non-contrast MRI in pregnancy with low-risk factors and lack of clinical evidence of HCC, follow-up with imaging modalities aiming to intervene at the third trimester can offer safer, and promising outcomes.A written informed consent was obtained from the patient for the publication of this case report.


Angiomyolipoma (AML) is the most prevalent renal mesenchymal neoplasm that almost always involves kidneys.We present a rare condition of retroperitoneal extra-renal angiomyolipoma which relapsed after 4 years that caused acute abdominal pain.The liver is the most common site of extrarenal angiomyolipoma. Abdominal pain is the most common complaint of extra-renal angiomyolipoma. Computed tomography angiography is the best way for angiomyolipoma diagnosis and surgery and post-surgery chemotherapy is the way for its treatment.extrarenal angiomyolipoma is an extremely rare condition that patients should be followed up for over 5 years by appropriate radiologic imaging. 1).•Angiomyolipoma almost always involves kidneys.•We present a retroperitoneal extra-renal angiomyolipoma case.2)•extra-renal angiomyolipoma is characterized as a benign tumor.•We present a recurrent retroperitoneal extra-renal angiomyolipoma.3)surgery is one of the best ways to both diagnosis and treatment of retroperitoneal extra-renal angiomyolipoma. AlthougIn this case report, we present a rare condition of retroperitoneal ERAML which relapsed after 4 years that caused acute abdominal pain and bleeding in a 43-year-old woman.2A 43-year-old female, a known case of retroperitoneal AML, was referred to the hospital with a complaint of abdominal pain for 5 days. The pain was initially in the periumbilical area and then accumulated in the right lower quadrant and referred to the left lower quadrant and back. It was reduced by bending forward and eating and had increased on the last day. She did not have any symptoms of nausea, vomiting, diarrhea, chest pain, and dyspnea. She did not mention any specific drug, family, or social history. Her past medical history was prominent for retroperitoneal AML next to the right renal arteries, 4 years ago, which was surgically removed. Radiotherapy was offered to him but she was refused this treatment.Upon arrival, she was not ill or toxic. Her vital signs were blood pressure:110/90 mmHg, pulse rate:85/min, temperature:36.7°, respiratory rate: 17/min, and O2 saturation:95%. In the abdominal examination, she had mild generalized tenderness without rebound, guarding, organomegaly, or distention. Her laboratory tests are shown in According to the current situation, previous history, and radiological findings of the patient, a diagnostic laparotomy was performed which showed 500 ccs of bloody fluid in the peritoneal and pelvic cavity. Medial rotation of right colon done. Retroperitoneal fossa explored and an extended mass was found which was lying on the back of the abdomen. The mass was isolated and separated from the aorta and inferior vena cava. The upper edge of the tumor was limited to the pancreas and duodenum with an appropriate plane between them. The tumor was removed completely  and was 3AMLs are benign tumors that have the triad of abnormal blood vessels, muscle fibers, and mature adipose tissue . They beMinija EJ. considered 56 cases of ERAML . They found 18 cases of liver AML (34.61%), 16 cases of retroperitoneum AML (30.76%), 7 cases of uterus AML (13.46%), 2 cases of vagina AML (3.84%), and 2 cases of head involvement (3.84%). Each Hard plate, abdominal wall, penis, fallopian tube, nasal cavity, spermatic cord, and colon AML was reported as a unique case [Venyo AK. considered 30 cases of retroperitoneal ERAML in 2016 . According to the site of involvement, there were different clinical symptoms such as abdominal pain (most compliant (14 cases) – similar to our case), bleeding, masses, back pain, weight loss, abdominal distension, weight gain, vomiting, constipation, hematuria, and urinary frequency. Also, there were three cases of accidental findings .Laboratory investigations  are basic tests undertaken in the assessment of this patient but because of nonspecific findings, they are not helpful for ERAML diagnosis .CTA is the best radiologic method for ERAML diagnosis . RetropeSurgery is the common procedure for ERAML treatment . If the 4Although ERAML is characterized as a benign tumor, it may involve liver or lymph nodes as metastatic features. Therefore, it may be good for patients to be followed up over 5 years by appropriate radiological imaging to minimize the risk of recurrence involvement .There is no financial support statement.Nothing to declare.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Narjes Mohammadzadeh: Chief surgeon, concept and design, final revision.Soroush Kohansal: writing the paper.Amir ghasemlouei: Surgeon, final revision.Name of the registry: N/A.Unique Identifying number or registration ID: N/A.Hyperlink to your specific registration (must be publicly accessible and will be checked): N/A.Amir Ghasemlouei.All authors declare that there is no conflict of interest regarding the publication of this paper.


Appendagitis is an inflammation of the epiploic fringes, generally unrecognized by the clinician. It is responsible for abdominal pain and may mimic other causes of acute abdomen. It can be primary or secondary. In this article, we describe the first case of primary epiploic appendagitis in a renal transplant patient who consulted for left inguinoscrotal pain, which was diagnosed as primary epiploic appendagitis. Epiploic appendagitis is an ischemic infarction of an epiploic appendage caused by torsion. It is considered a rare pathology for acute abdomen, occurring predominantly among males aged between 20 and 50 . ObesityThe patient is a 23-year-old male, who followed up for end-stage chronic renal disease secondary to a segmental and focal hyalinosis, and started hemodialysis in 2017 by a left radio-radial arteriovenous fistula. He has no other medical or surgical history. He received a kidney transplant on April 22, 2022, from a living, related donor. The surgical procedure consisted of implantation of the graft in the right iliac fossa with end-to-side arterial and venous anastomosis on the external iliac and a Gregoir-type anastomosis with the temporary placement of a double J probe. The immunosuppressive therapy was based on tacrolimus, mycophenolate mofetil, and prednisone. The postoperative follow-up was uneventful.He experienced three days of constant pain in the left inguinoscrotal area. Thirty-five days after the renal graft, he was feeling feverish without associated urinary or other digestive signs. The patient was apyretic, hemodynamically stable, with a BMI of 18. He had a sensitivity of the left iliac fossa with no palpable mass. The scar of the right graft was clean and painless, with no signs of inflammation. His complete blood count and C-reactive protein (CRP) were within normal range.Abdominal and inguinoscrotal ultrasound was performed, showing a hypogastric formation compatible with a diagnosis of appendagitis, without testicular torsion or any abnormalities on the graft.The abdominal scan without injection revealed a hanging formation on the mesosigmoid, related to epiploic appendagitis of the sigmoid. It was then determined to be a primary epiploic appendagitis Figures , 2, 3. TEpiploic appendagitis was first described in 1956 . They arContrary to the main differential diagnosis , the biological assessment is generally within normal limits. A slight elevation of the white blood count (WBC) and C-reactive protein (CRP) is rarely noted as an inflammatory response to fat necrosis .The diagnosis used to be made during surgery. However, with the advancement of imaging, the diagnosis of appendagitis is made more easily by experienced radiologists. When the ultrasound is not very contributive, the abdominal scan with or without injection of iodinated contrast product enables the diagnosis by visualizing a "shuttle" image. It is an intra-peritoneal mass attached to the colon, the density of which is slightly higher than normal fat, often oval, well limited by a thin border of higher density, enhanced after injection of the iodinated contrast product, thus achieving a "ring sign" appearance ,7.The treatment is, in most cases, conservative, based on analgesic treatment, with or without non-steroidal anti-inflammatory drugs (NSAIDs), with the resolution of the pain in a week or two. Resection of the necrotic fringe is necessary in complicated cases, with good outcomes. The use of antibiotics does not seem justified, even in immunocompromised patients, unless there are signs of sepsis or bacteremia , 3, 5.Appendagitis after transplantation was reported for the first time after liver transplantation for autoimmune hepatitis in a 71-year-old woman. Her treatment was based on mycophenolate mofetil, tacrolimus, and prednisone. An abdominal scan enabled the diagnosis. She was treated symptomatically with good evolution .Is the occurrence of post-transplant appendagitis in our patient a simple coincidence? Or are there other factors, like surgery or immunosuppression, favoring its occurrence? To our knowledge, our case seems to be the first appendagitis occurring after renal transplantation. Other studies with a larger sample of patients are necessary to arrive at a conclusion.This case helps illustrate a rare and uncommon diagnosis of abdominal pain in renal transplant patients. In the case of acute abdominal pain without a major inflammatory syndrome, it is necessary to suspect appendagitis, among the other diagnoses. The confirmation is based on medical imaging to avoid unnecessary surgical interventions. Medical treatment with analgesics avoids an increase in the duration of hospitalization, as well as unnecessary antibiotic therapy.


Calcifying odontogenic cyst (COC) is a cyst originating from odontogenic epithelium and has a characteristic ghost cell appearance. Clinically, COC is characterized by asymptomatic swelling, leading to lingual expansion, tooth migration, apical resorption, and perforation of cortical bone. COC is most often asymptomatic and is often only discovered during a routine radiological examination. This case report describes the clinical interventions to manage a patient with mesiodens impacted with COC. The procedure is aimed at evaluating the multidisciplinary management of COC at Universitas Airlangga Hospital, Surabaya. Multidisciplinary care has an important role in treating COC cases comprehensively. The calcifying odontogenic cyst is an uncommon benign cyst of odontogenic origin, characterized by an ameloblastoma-like epithelium with ghost cells that may calcify, first described by Gorlin et al. in 1962 [Clinically, it is characterized by slowly growing asymptomatic swelling. It may have a central (intraosseous), or less frequently, a peripheral (extraosseous) localization. It may cause lingual expansion, displacement of teeth, root resorption, and perforation in the cortical bone .On radiographs, COC appears as a well-limited unilocular radiolucent lesion of different sizes, shapes, and opacity levels. Impacted teeth may be associated. Root resorption, root divergence, or cortical blowing may be observed .A definitive diagnosis of calcifying odontogenic cyst can be reliably made based on a histological examination. An abnormal form of keratinization in the form of the ghost cells is the most distinguishing feature of a COC. However, their presence does not confirm the diagnosis as other lesions show similar presentation. Hence, a diagnosis of COC should only be made for a lesion in which the formation of ghost cells takes place in a typical epithelial cyst lining, presenting a basal layer of cuboidal or short cylindrical cells and an overlying layer consisting of cells that bear resemblance to stellate reticulum-like cells .The recommended treatment for COC consists of enucleation with curettage, which means enucleation followed by removal of a 1 to 2 mm layer of bone around the periphery of the cystic cavity with a sharp curette or a bone bur. This procedure is aimed at limiting the risk of recurrence. Decompression and marsupialization are conservative treatments used in large lesions with a high success rate .In this case, the cyst has involved the apical part of the surrounding teeth, so multidisciplinary treatment is needed for comprehensive treatment to remove the cyst and preserve the tooth. The purpose of this report is to present an interesting and rare case of COC associated with impacted mesiodens and to highlight the importance of multidisciplinary management of COC.A 14-year-old boy came to the Pediatric Dental Clinic of Rumah Sakit Gigi dan Mulut Universitas Airlangga with a history of fractured teeth due to an accident 5 years ago. The patient is currently on apexification treatment.Periapical radiographic examination showed #11 with an open apex , and theInterpretation of CBCT radiographic examination showed inverted mesiodens impaction on the palate of #11 and #21; the lesion was demarcated, radiopaque, oval in shape, and causing a discontinuity in the base of the nasal cavity .Based on periapical, panoramic, and CBCT, radiographs displayed unilocular, radiolucent, and well-delineated defect related to impacted mesiodens, suggesting a dentigerous cyst . The difTreatment was planned with a multidisciplinary approach with the oral and maxillofacial surgeon under general anesthesia for odontectomy of mesiodens impacted teeth and enucleation of the cyst, followed by apical resection of teeth involved teeth and closed with MTA plug.Laboratory assessment and thorax radiographic examination were done before the procedure and found no negative symptoms. RT-PCR testing for SARS-CoV-2 detection from nasopharyngeal and oropharyngeal swabs to screen for both symptomatic and asymptomatic COVID-19 was done, and the result was negative. This test was mandatory for the preoperative procedure in the hospital. After confirming that the patient is completely anesthetized, an extraoral asepsis procedure was performed using 70% alcohol and 10% povidone-iodine intraorally. A vasoconstrictor consisting of 2 ampoules of pehacain diluted with 6 ml of saline, injected with a local infiltration technique using a 21 gauge syringe, was administered, and a full-thickness trapezium flap was elevated on a periapical area of #12, #11, #21, and #22 regions. A round carbide bur under constant irrigation for cooling was used to enlarge the bony defect to the buccal window to gain access to the periapical lesion and root end of the affected tooth . Needle Surgical procedures also included germinectomy of 18, 28, 38, and 48 tooth buds.Histopathological anatomy results obtained after cyst enucleation and mesiodens collection  confirmeThe patient was recalled after one day to access the surgical site recovery and found no sign of pain, dizziness, nausea, and vomiting. On extraoral examination, there were no signs of edema, hyperemia, and tenderness. On intraoral examination, the postoperative wounds on #12, #11, #21, #22, #18, #28, #38, and #48 were well sewn and hyperaemic, and minimal debris was seen at the edges of the sutures .On palpation examination, the postoperative wound was palpated in the regions of #12, #11, #21, #22, #18, #28, #38, and #48, and there was minimal edema and tenderness at the edges of the sutures. On day 7 postoperative, the suture was removed. In #11, root canal access was completed without anesthesia and #21 with anesthesia. #11 and #21 were prepared up to sizes 40 and 50, respectively, 3 mm short of the apical foramen. In all teeth, the root canals were irrigated with 3 mL of 2.5% sodium hypochlorite (NaOCl), and distilled water at each change of file was used as an intracanal dressing. The quality of canal filling with the medication was confirmed radiographically. The teeth were temporarily restored with an intermediate restorative material.After two times of sterilization, the intracanal dressing was removed, and the root canals of #11 and #21 were filled with gutta-percha and followed by composite resin restoration. Follow-up is scheduled at 3 months, 6 months, and 1 year to assess clinical and radiographic signs of healing.The patient came after 3 and 6 months postoperative; there were no signs of swelling, redness, or tooth mobility of #11 and #21 and underwent an X-ray panoramic control which described a radiopaque area with several radiolucent areas apical to #11; #12 with an impression of the bone ossification process showed a beginning bone regeneration. There are no signs of recurrence Figures  and 9.The calcifying odontogenic cyst is an uncommon benign cyst of odontogenic origin . RetrospThe last 20 years from the following databases: PubMed, Medline Ovid, Web of Science, Scopus. The present systematic review revealed that COC patients' age ranged from two to 92 years, and the mean age at diagnosis was 30.7 (±21.0) years. Males made up 184 (51.8%) of the COC cases, while females made up 171 (48.2%) of the cases . Regarding anatomical location, a predilection for the maxilla (53.3%) was observed. The mandible was affected in 45.7% of cases, and information about the affected site was not available in only one case. COC was asymptomatic in 89.2% of patients. Symptom duration ranged from one to 180 months, with a mean of 18.1 (±29.8) months. Most cases exhibited a well-defined border (87.0%), with mixed (61.1%) and unilocular appearance (81.9%). The bone expansion was observed in 81.9% of cases. Lesion size ranged from 2.0 to 90.0 mm, with a mean of 29.5 (±20.8) mm. Regarding treatment, enucleation was the most common (69.9% of cases). In 194 cases (96.0%), no recurrence was reported. As regards the follow-up period, the mean duration of surveillance ranged from three to 264 months, with a mean of 42.2 (±43.6) months. Concerning treatment, enucleation was the modality of choice in most cases  .As regards treatment, conservative surgery was the main choice for COC. Herein, the two strategies most frequently reported for COC treatment were enucleation in 69.9% of cases and surgical removal or excisional biopsy in 18.5%. Furthermore, a two-stage approach was followed in 4.0% of cases. Uncommonly reported in COC therapy, this option is mainly chosen for other odontogenic cysts such as dentigerous or odontogenic keratocyst. Decompression or marsupialization is first performed to reduce intraluminal pressure and shrinkage of the cyst following a period of months. This allows bone growth and removal of the cyst in a second stage, with less risk of damage to important anatomical structures , 16.The present review identified two COCs treated only by marsupialization. The recurrence rate after conservative COC management was low for both intraosseous and extraosseous lesions .Considering the complexity of the case, we decided to manage the mesiodens impacted case with COC multidisciplinary . RadiogrIn this case, radiographic examination revealed a solitary well-defined unilocular radiolucency around the impacted mesiodens. Based on the clinic-radiological findings, differential diagnosis of benign odontogenic lesions like a dentigerous cyst, radicular cyst, and nasopalatine duct cyst was considered. In the current case, with the presence of pericoronal radiolucency associated with unerupted mesiodens, the working diagnosis of the dentigerous cyst was made.A definite diagnosis of calcifying odontogenic cyst can be reliably made based on a histological examination due to the lesion's lack of characteristic clinical and radiological features, as well as its variable biological behavior .Histological features of the lesion show a fibrous capsule with a lining of odontogenic epithelium. The basal layer is made up of ameloblast-like columnar or cuboidal cells of 4–10 cell thickness. It is covered by loosely arranged epithelial cells bearing similarities to the stellate reticulum of the enamel organ. There are varying numbers of epithelial cells that are devoid of nuclei and eosinophilic and retain their basic cell outline (ghost cells). These ghost cells can calcify, and calcifications are constant but vary in number .The results of the histopathological examination on this case show sections of cyst wall tissue lined with thin squamous cuboidal epithelium, consisting of round, mononucleated, and smooth chromatin cells. The stroma is a fibrous connective tissue with many foci of eosinophilic calcification resembling ghost cells. There were no signs of malignancy .The patient came with a history of trauma and swelling. Traumatic dental injuries to the teeth and the maxillofacial structures are a common occurrence, with the majority affecting the dentoalveolar structures. The most common types of traumatic dental injuries are crown fractures in the permanent dentition. An accurate diagnosis is essential to facilitate prompt management, which is widely regarded to have a major influence on the prognosis of the outcome.Radiographic examination is essential for the diagnosis of traumatic dental injuries. In addition to a thorough clinical examination, intraoral radiographs such as periapical radiographs and upper standard occlusal radiographs are routinely prescribed to identify the location and nature of traumatic dental injuries. The diagnostic radiographs also form part of the baseline records to allow objective assessment at the follow-up appointment .The clinical parameters observed were the presence of symptoms in the traumatized tooth, coronary discoloration, degree of tooth mobility, and the health of surrounding soft tissues, through visual examination and palpation. The radiographic parameters were the presence or absence of periapical lesion, pulp canal obliteration, and pathological root resorption, which was differentiated from physiological resorption by clinical history, patient age, and morphological characteristics of resorption. With both clinical and radiographic parameters, an analysis of both was performed to establish the condition of vitality or pulp necrosis .Moreover, it has also been suggested that trauma diagnostics during childhood is more difficult than in grown-up persons. These authors reported that the sutures of the skull are wider and that more adipose tissue covers the bones, which will complicate the diagnostics accuracy. Fortunately, intraoral radiographs give a very low radiation dose if the correct technique and good X-ray equipment are used making it ethically indicated to prescribe intraoral radiographs for adults and children whenever the clinical examination indicates that a more severe dental injury might exist .Recently, a new tomographic imaging method has started to be used, cone-beam CT (CBCT). CBCT has high diagnostic accuracy in cases of traumatic dental and maxillofacial injuries. But intraoral radiographs are a good start and are accurate enough if we only suspect dental injuries or minimally displaced bone fractures according to other researchers. In general, in dental trauma, CBCT should only be prescribed in selected cases, where conventional radiographs provide inadequate information for treatment planning .In this case, after the periapical radiographic examination revealed mesiodens impaction on the apical of the maxillary anterior teeth, we referred the patient for a CBCT radiographic examination to help determine the diagnosis and location of the impacted tooth and appropriate treatment plan.According to the periapical radiography, apical #11 was exposed with an open apical; then, apexification was performed by the pediatric dentist. Apexification is a method of treatment for immature permanent teeth in which root growth and development ceased due to pulp necrosis. Its purpose is to induce root-end closure with no canal wall thickening or continuous root lengthening. It can be achieved in two ways: (1) as a long-term procedure using calcium hydroxide dressing to allow the formation of a biologic hard tissue barrier or (2) as a short-term (more recent) procedure, creating an artificial apical plug of MTA or other bioceramic material .The mechanism of action of calcium hydroxide in the induction of an apical barrier is still controversial, although it is formed by cells originating from the adjacent connective tissue. The calcified barrier, even when appearing radiographically and clinically complete, is histologically porous and may be composed of cementum, dentin, bone, or osteodentin. This procedure requires multiple visits and could take a year or more to achieve a complete apical barrier that would allow root canal filling using gutta-percha (GP) and sealer .Cyst size is an important factor when formulating a treatment plan. The cystic lesions having a size < 5 cm can be managed with simple enucleation/curettage and submitted for pathologic examination  while preserving the strategic tooth or teeth involved. In the few cases that had clinical or radiographic evidence of multilocular lesions, extensive lesions with involvement of adjacent soft tissues or a history of multiple recurrent lesions should be considered to have aggressive behavior, and radical resection could be the first choice of treatment option depending on the surgeon's training, available resources, and the patient's preferences. Otherwise, conservative treatment methods such as decompression with a second surgical procedure (enucleation) and another aggressive approach than bone resection can be chosen, and the failures of this treatment must indicate the radical resection method as well as for extensive lesions without aggressive behaviors. Longitudinal follow-up should be considered , 25 Fig.The recommended treatment for COC, in general, is total excision in one step. Enucleation associated with curettage is the usual therapy when referring to the cystic variant. Hence, after removing the lesion, a layer of 1 to 2 mm of bone must be removed at the periphery of the cystic cavity .The patient underwent cyst enucleation, mesiodens impacted tooth odontectomy, periapical exploration of #11 and #12, followed by apical resection of the teeth. Apical #11 and #21 were closed with MTA and GIC and planned orthograde root canal filling for tooth 11 and pulpectomy for #21 after surgery.In this case, MTA was chosen because preclinical studies clearly showed that MTA has a high sealing capability, good material stability, and excellent biocompatibility. Multiple experimental studies in animals highlighted the mild tissue reactions observed adjacent to this material. Furthermore, histological analysis of the periapical regions demonstrated a frequent deposition of new cementum not only onto the resection plane  but also directly onto MTA. For these reasons, MTA is considered a bioactive material.In addition, germinectomy for tooth buds #18, #28, #38, and #48 was also performed under general anesthesia. General anesthesia was chosen because based on the CBCT interpretation, the lesion was in contact with the floor of the nasal cavity, and the patient also required the extraction of the four wisdom teeth.Obturation of #11 was performed one week after surgery and was followed by root canal treatment on #21. In the three- and six-month follow-ups, there were no complaints subjectively. Objectively, there were no signs of swelling, redness, or tooth mobility in #11 and #21. Panoramic and periapical X-rays showed the hermetic filling of #11 and #21, radiopaque area with several radiolucent areas apical to #11, and #12 with an impression of the bone ossification process. There are no signs of recurrence.The patient was scheduled for periodic follow-up appointments once a year to see if there are any complaints and if signs of healing have formed.This study is expected to provide insight into how to determine the diagnosis of a lesion through clinical approaches and radiographic and pathological examinations, especially when the lesion involves surrounding structures such as adjacent tooth roots. Cross-sectoral collaboration is needed to be able to handle complex cases to achieve complete care for the patient.Our case represents the classical features of the calcifying odontogenic cyst. The comprehensive and multidisciplinary approach is the best way to manage complex cases of mesiodens impaction with COC to relieve symptoms and assure total healing. A proper diagnostic setup is the starting point to develop an efficient treatment plan.


Follicular pancreatitis is a very rare type of focal chronic pancreatitis and is often mistaken for pancreatic neoplasms. It is histologically characterized by extensive lymphoid follicular formation with reactive germinal centers.A 50-year-old man was admitted to our hospital with 1-month history of epigastric pain. Contrast-enhanced computed tomography and magnetic resonance imaging revealed a 4.7 cm sized enhancing pancreatic head mass with anterior mesenteric soft tissue infiltration and superior mesenteric vein invasion. Endoscopic ultrasonography revealed an ill-defined hypoechoic mass in the head of the pancreas.A laparoscopic surgical biopsy was performed. Hematoxylin-eosin staining showed the acini structure destruction within the pancreatic parenchyma and different-sized lymphoid follicles with reactive germinal centers around the duct. Immunohistochemical examination showed that cells were positive for the B-cell marker CD20, T-cell marker CD3, and slightly positive for IgG4. However, cells were negative for the B-cell marker Bcl-2. Follicular pancreatitis was confirmed based on the findings of histology and immunohistochemistry.The patient was regularly followed without any specific treatment.Follow-up computed tomography revealed no change in the lesion 1 year after diagnosis.To the best of our knowledge, this is the first case of follicular pancreatitis in Korea. It is histologically characterized by extensive lymphoid follicular formation with reactive germinal centers. Patients with follicular pancreatitis are often asymptomatic and the lesions are incidentally discovered as a mass on radiologic imaging, suggestive of pancreatic cancer. Therefore, it is usually diagnosed retrospectively through a surgical approach.Herein, we report a case of a 50-year-old man with follicular pancreatitis, which was mistaken for pancreatic cancer, and the correct diagnosis was made through surgical biopsy, along with a review of the literature about this condition.3 , hemoglobin 14.5 g/dL , platelet count 270,000/mm3 , blood urea nitrogen 13.9 mg/dL , creatinine 0.95 mg/dL , serum albumin 4.5 g/dL , aspartate aminotransferase 21 U/L , alanine aminotransferase 30 U/L , alkaline phosphatase 124 U/L , and lactate dehydrogenase 293 IU/L . Total bilirubin was 0.48 mg/dL with a direct fraction of 0.19 mg/dL . Amylase was 159 U/L  and lipase was 483 U/L . Serum carcinoembryonic antigen (CEA) was within the normal limit. Carbohydrate antigen 19-9 was 68.75 U/mL . Contrast-enhanced abdominal and pelvic computed tomography (CT) revealed an enhancing mass (4.7 cm) with anterior mesenteric soft tissue infiltration in the head of the pancreas (Fig. A 50-year-old man was admitted to our hospital with 1-month history of epigastric pain. He had no history of diabetes mellitus and hypertension. Furthermore, he had no history of alcohol drinking, although he was an ex-smoker with a history of 20 pack years. On admission, his vital signs were normal. There was tenderness in his epigastrium while other physical examinations were unremarkable. Laboratory examinations revealed the following findings: white blood cell count 6000/mm–7Follicular pancreatitis is an extremely rare form of mass-forming focal chronic pancreatitis. Because of its rarity, follicular pancreatitis is often mistaken for pancreatic cancer. The clinical manifestations, diagnosis, treatment, and prognosis of follicular pancreatitis have not been validated yet. suggest that follicular pancreatitis typically occurs in patients older than 50 years with a male predominance. The clinical presentation is often asymptomatic, although elevated levels of liver enzymes, abdominal pain, jaundice, malaise, and weight loss have been observed. The most commonly affected site is the pancreatic tail, followed by the head, body, and the uncinate process. A single pancreatic mass is most common on radiologic images, followed by multiple masses and no mass. On EUS, the appearance is usually a hypoechoic mass. In our case, follicular pancreatitis manifested as a single pancreatic head mass of hypoechoic nature in a 50-year-old man with abdominal pain. To the best of our knowledge, this is the first case of follicular pancreatitis in Korea.Previous case reports–7 In our case, EUS-FNB was initially performed for the diagnosis; however, pancreatic cancer was not ruled out. Eventually, a laparoscopic surgical biopsy was performed for an accurate histologic diagnosis.Primary diagnosis of follicular pancreatitis is difficult since radiologic imaging findings strongly suggest the presence of pancreatic cancer. Most of the reported cases of follicular pancreatitis have been diagnosed retrospectively after surgical resection, sometimes with surgical complications.Histologically, follicular pancreatitis is characterized by extensive intra-pancreatic lymphoid follicles formation and dense lymphoplasmacytic infiltration. Furthermore, follicular pancreatitis is associated with diffuse or periductal Bcl-2 negative reactive germinal centers within lymphoid follicles and a low IgG4+ plasma cell count, which is similar to the findings of our case.–7 One case partially responded to steroid therapy; however, its efficacy remains unclear. Our case was regularly followed without any special treatment. Follow-up contrast-enhanced abdominal and pelvic CT revealed no change in the lesion 1 year after the surgical diagnosis. However, further follow-up is necessary to determine the long-term efficacy of this approach.The treatment of this disease is not well established yet. Most reported cases were diagnosed and treated by surgical resection, and there is no recurrence after surgical resection.In summary, the dramatic differences in the treatment and prognosis of follicular pancreatitis and pancreatic cancer stress the importance of differential diagnosis. Therefore, despite its rarity, follicular pancreatitis should not be ruled out during the differential diagnosis of pancreatic cancer. Furthermore, a high index of clinical suspicion and an accurate histopathologic diagnosis is important for identifying patients with follicular pancreatitis. If an accurate histopathologic diagnosis is made before surgery, unnecessary surgery and surgical complications can be avoided.Conceptualization: Young Eun Joo.Investigation: Wan-Sik Lee, Myung-Giun Noh.Resources: Wan-Sik Lee, Ki-Hyun Kim, Myung-Giun Noh.Writing – original draft: Wan-Sik Lee, Ki-Hyun Kim.Writing – review & editing: Wan-Sik Lee, Young Eun Joo.


The clinical behaviors, prognosis, and appropriate treatments of papillary tumors of the pineal region (PTPR) are not fully defined due to the rarity of these tumors. At diagnosis, PTPR may present with clinical symptoms, including headache with obstructive hydrocephalus, diplopia, vomiting, and lethargy, as well as neurological signs, including Argyll Robertson pupils and Parinaud’s syndrome due to compression of the dorsal midbrain, specifically the periaqueductal region with horizontal nystagmus. Radiological assessment of pineal region lesions is challenging, with a wide range of potential differential diagnoses. PTPR typically presents as a heterogeneous, well-circumscribed mass in the pineal region, which might contain cystic areas, calcifications, hemorrhages, or protein accumulations. Here, we report three female pediatric patients with PTPR treated in King Fahad Medical City (KFMC) in Saudi Arabia. Histological and immunohistochemical diagnosis was confirmed by analysis of genome-wide DNA methylation profiles. This case series expands on the available reports on the clinical presentations of PTPR and provides important information on the responses to different treatment modalities. Papillary tumors of the pineal region (PTPR) are very rare neuroepithelial tumors characterized by a papillary architecture and epithelial cytology. First introduced in the World Health Organization classification of central nervous system tumors in 2007 and classified as WHO grade 2–3 ,2..PTPR has no sex predilection, although some reports suggested a female preponderance. Fewer than 200 cases are reported to date, and pediatric cases are very rare and have an average patient age of 11.6 years (range 1–18 years) ..PTPR has an uncertain origin; it does not arise from the pineal gland but is thought to arise from the ependymal cells of the subcommissural organ (SCO), which is located below the posterior commissure at the level of the cerebral aqueduct, just anterior to the pineal gland. The SCO contains specialized cytokeratin-positive and nestin-positive ependymal cells and is involved in the secretion of glycopeptides and regulation of CSF during development. Its glycopeptide content is thought to be the source of the intrinsic T1 hyperintensity commonly reported in PTPR .DNA methylation-based tumor classification emerged as a promising tool for CNS tumors. Two methylation groups for papillary tumors of the pineal region (A and B) are defined. In “PTPR Group A”, which is equivalent to PTPR Group 1 in the report of Heim et al. , loss ofhttps://www.molecularneuropathology.org), (accessed on 1 May 2021), and reports were produced as shown by Capper et al. [.Here, we report a case series involving three PTPR pediatric patients treated in King Fahad Medical City. All were female, and the histological and immunohistochemical diagnosis was confirmed by methylation CNS classifier profiling. Samples were processed in our institutional genomics facility, which combined samples from multiple sources for processing by an Illumina Infinium HumanMethylation450 BeadChip (450k) array or by a MethylationEPIC BeadChip (850k) array. Standard quality controls confirmed adequate tumor purity/quality, bisulfite conversion, and DNA quality. IDAT files were uploaded to either version 11b2 or 11b4 of the online CNS tumor methylation classifier  was placed. The patient was transferred to KFMC for further evaluation. A brain CT and MRI identified a pineal region mass of 1.6 × 1.9 × 2 cm A–C. MRI The patient underwent an endoscopic radiotherapy frontal stereotactic biopsy of the pineal tumor only. Histopathology revealed a grade 2–3 papillary tumor of the pineal region (PTPR) F,G. MethDue to the MRI findings suggestive of spinal seeding metastasis, the patient underwent craniospinal irradiation treatment. A follow-up MRI 4 months after radiation intracranial pressure revealed a significant decrease in the size of the heterogeneous residual lesion along the surgical bed  J,K. The She remained in good health until 4 months, when she was admitted to the neurosurgical ward with increased somnolence and decreased activity. MRI showed a progressive increase in the size of the residual tumor in the pineal region, now engulfing the tectal plate and obstructing the cerebral aqueduct E,F. AddiShe underwent a second craniotomy with partial resection and debulking of the progressed tumor. Postoperative results reveal a small residual lesion H.Again, histopathological examination confirmed PTPR with a higher mitotic rate (over 15/10 hpf) accompanied by foci of necrosis, raising the possibility of a high-grade transformation (not shown). CSF analysis was negative for metastases. The patient received external beam radiation therapy (EBRT) localized to the brain at 59.4 Gy/33 Fx.She recovered well, with no neurological deficits and was discharged in a good condition. Due to the aggressive pineloblastoma-like behavior of the tumor and recurrent relapse, she was initiated on the chemotherapy protocol of Medulloblastoma Saudi Arabian Pediatric Hematology Oncology Society (SAPHOS), with a total of six maintenance cycles alternating (A&B) as follows:Cycle A: Cisplatin 90 mg/m2/day, day 1 and oral etoposide 35 mg/m2/day P.O. days 1–21 of a 4-week cycle.Cycle B: Cyclophosphamide 1 g/m2/day, days 1 and 2, vincristine 1.5 mg/m2, days 1, 8, of a 4-week cycle with G-CSF SQ at 5 μg/kg/day, daily for at least 10 days, starting 24 hours after the last dose of chemotherapy in each cycle.The patient tolerated chemotherapy well. More than one year post chemotherapy, she is clinically well, and her last MRI showed improvement of the postoperative changes, with no definite enhanced residual or recurrent masses at the surgical bed, and no intracranial or intraspinal CSF seeding metastasis I.A 6-year-old girl presented with a 3-month history of headache and vomiting. A CT scan conducted at a local hospital indicated a pineal tumor with obstructive hydrocephalus. She underwent surgical endoscopic ventriculostomy with EVD insertion, which was complicated by postoperative meningitis and was treated with a full course of antibiotics. Thereafter, a permanent VPS was inserted and the case was referred to KFMC for proper surgical management.A pre surgery MRI showed a well-circumscribed, enhanced mass measuring 1.5 × 1.4 × 1.4 cm in the AP, CC, and transverse diameters, respectively, showing moderate diffusion restriction in the pineal region and extending into the inferior and posterior aspects of the third ventricle A–E. SpinHistopathology analysis revealed a PTPR J,K with Methylation class analysis identified a papillary tumor of the pineal region group B L. CSF cyAs discussed in the multidisciplinary neuro-oncology tumor board, the patient was planned for observation and MRI follow-up. She remained well until one-year post-diagnosis, when she came to the ER with headache, lethargy, and signs of raised intracranial pressure (ICP), and was admitted to the PICU.3 in AP, TR, and CC, respectively. The lesion was situated in the posterior aspect of the third ventricle and embedded in the proximal cerebral aqueduct, causing secondary obstructive hydrocephalus with a mass effect on the tectum, which was displaced posteriorly . She is currently stable, and MRI follow-up showed a stable residual mass in the pineal region (not shown).In this report, we describe a case series of three female pediatric patients, each with distinct clinical behaviors and treated in different ways, reflecting the heterogenicity of the tumor and lack of a consensus on proper therapeutic and management plans.The radiological findings of PTPR in our case series mostly match those reported in the literature. However, our cases showed a heterogeneous signal intensity on T2WI with diffusion restriction and heterogeneous enhancement.Although case #2 showed multiple large areas of high signal intensity on T1WI, other cases mostly presented with isosignal intensity on T1WI, with hyperintensity observed along the periphery of the lesion. Most of the high T1 signal intensity in our cases is attributed to hemorrhage, as the high signal on T1WI was decreased in the follow-up studies with a greater susceptibility effect, but other areas remained unchanged, and did not show any susceptibility and were not suppressed in fat saturated sequences, indicating proteinaceous content.In a study by Chang et al. , the autSimilarly, a case report study on a 17-year-old patient found a T1 hyperintensity within the lesion after the authors excluded fat content and calcification. The authors concluded that the glycopeptides secreted from PTPR were likely the cause for this T1 hyperintensity .Interestingly, images in our young pediatric patients showed a diminished appearance on T1WI relative to that reported for older patients, which might indicate a thus far unexplored intrinsic difference in PTPR tumors dependent on the patient’s age.The clinical behavior, prognosis, and appropriate treatment of PTPR in pediatric patients are yet to be fully defined due to the rarity of these tumors and the limited number of reported cases in the literature. PTPR in adults has a high recurrence rate of up to 67–73%, with a reported 5-year progression-free survival rate of only 27%, while a lower recurrence rate of 47% is reported in children [Surgery is the primary therapy for PTPR, and the extent of surgical resection is the only clinical factor significantly associated with better overall survival. Incomplete surgical resection and tumors with higher mitotic and proliferative activity (as measured by Ki-67 expression) are associated with a poor prognosis . Focal aThere is no proven benefit of chemotherapy, and different chemotherapy regimens are reported in recurrent refractory cases, including procarbazine, lomustine, vincristine, and temozolomide .At the molecular signaling level, mTOR kinase inhibitors, such as everolimus, are reported to be effective in controlling recurrent PTPR tumors with chromosome 10 deletions and specifically with inactivating mutations in the PTEN (phosphatase and tensin-like protein) tumor suppressor gene on chromosome 10, which is involved in the PI3K/Akt/mTOR signaling pathway. PTEN and its downstream targets may respond to everolimus with or without temozolomide .Based on the DNA methylation patterns, we could discriminate two separate clinical behaviors. First case: The DNA methylation class was PTPR Group B. The patient presented with spinal leptomeningeal dissemination, which is rarely reported in PTPR. Tumor control with CSI radiation therapy alone was excellent, with further tumor volume reduction continuing over time posttreatment.The second case: The DNA methylation class was PTPR Group A, but the patient had an aggressive course with recurrent local tumor growth, despite radiation therapy and repeated surgical resections without spinal seeding metastasis. Interestingly, this patient responded well to pineoblastoma-like intensive chemotherapy, indicating that there are instances where chemotherapy is indicated in the treatment of PTPR.The third patient: The DNA methylation class was PTPR Group B. The patient also had local recurrence with microscopic CSF spinal dissemination, and was treated with CSI radiation therapy.Currently, the clinical behavior of PTPRs can be compared to that of ependymoma, with gross total resection as a mainstay of treatment, followed by surveillance neuroimaging. Adjuvant therapies, such as radiation with or without chemotherapy, should be considered when complete surgical resection is not feasible.Based on our case series, we recommend using the DNA methylation patterns to detect PTPR Group B, which is reported to have a more aggressive course and was present in our patients with spinal metastasis that needs to be controlled with CSI radiation therapy.Future therapeutic strategies will undoubtedly benefit from further molecular and mechanistic insights into the effects of the chromosomal changes associated with individual PTPR tumors. This might open the door to individualized medicine approaches that allow therapeutic targeting of specific signaling pathways or molecular mechanisms that play critical roles in the development of an individual’s PTPR tumor.


Clear cell sarcoma of the kidney is an uncommon pediatric renal malignant neoplasm that is typically characterized in 2–3-year-olds by aggressive behavior and late relapses. Our literature review revealed fewer than ten previously reported cases of CCSK with inferior vena cava thrombus, with only five in the pediatric age group.3), associated with dilatation of the left renal vein. Contrast abdominal computed tomography showed a mass measuring 7 × 5 × 3 cm3 with the presence of thrombus extending into the inferior cavity down to the right atrium that was initially diagnosed as Wilms’ tumor. Radical right nephrectomy with excision of the thrombus was undertaken. Histological immunostaining revealed a diagnosis of the tumor as clear cell sarcoma with vascular tumor thrombus extending to the inferior vena cava.We report the case of a 14-year-old Syrian girl who complained of mild pain in the left lumbar region pain with hematuria. On physical examination, a mass was palpated in the left flank. Abdominal ultrasonography revealed a left renal mass (7 × 5 × 2 cmClear cell sarcoma and Wilms’ tumor are similar in terms of typical age of appearance, clinical features, and histopathology, but with different methods of treatment and prognosis. The differential diagnosis of such masses is thus very important. We present the case of a patient with clear cell sarcoma with unusual age, with complete removal of the thromboses in the inferior vena cava and the right atrium. Clear cell sarcoma of the kidney (CCSK) is an uncommon pediatric renal malignant neoplasm  . CCSK isA 14-year-old Syrian female was admitted to the Urology Department of Tishreen University Hospital with 2-month history of mild pain in the left lumbar region pain with hematuria and weight loss of over 10 kg in that interval. She was treated with antibiotics without improvement. There was no previous medical or familial history.On physical examination, her temperature was 38.3 °C, oxygen saturation 98%, and arterial blood pressure was 11/8 (systolic pressure/diastolic pressure). She was stable with normal physical examination except for pallor and left lumbar region mass.On laboratory analysis, erythrocyte sedimentation rate (ESR) and lactate dehydrogenase (LDH) were elevated. Complete blood count (CBC), C-reactive protein (CRP), renal and liver function, and glucose were normal.Urinalysis showed 50–60 red blood cells Table .Table 1L3 with dilation of the left renal vein. Intravenous contrast computed tomography (CT) scan of the abdomen showed a mass with size of 7 × 5 × 3 cm3 arising from the anterior part of the left kidney with tumor thrombus in the inferior vena cava extending to the right atrium without metastases  with monitoring of the patient’s vital signs . The patient was systemically heparinized in the intensive care unit. The postoperative course was uneventful; after 72 hours, she was referred to the urology department. Seven days later, she was discharged from the hospital in good condition. The histopathology of the resected renal mass revealed a tumor cell with clear cytoplasm, indistinct nucleoli, and abundant extracellular matrix. There were cystic and focal areas of necrosis , and radical nephrectomy with or without radiation is used .According to our literature review, the therapeutic strategy for intravascular thrombosis in the cases of pediatric tumors is based on preoperative chemotherapy to achieve intravascular tumor regression with delayed resection of the intravascular thrombosis during a laparotomy . PediatrThe present case report describes a patient with CCSK with vena cava thrombus. Initially, surgical treatment followed by chemotherapy was believed to be the treatment of choice.