They usually present as World Health Organization (WHO) grade I, and they delineate on gadolinium-enhanced MRI; the surgical goal is wide radical resection, and the course thereafter is usually benign. Fluorescein sodium (FL) tends to accumulate in areas with altered blood–brain barrier (BBB). Thus far, the results provided by prospective and retrospective studies show that the utilization of this fluorophore may be associated with better visualization and improvement of resection for several tumors of the central nervous system. In this study, we retrospectively studied the effect of fluorescein sodium on visualization and resection of gangliogliomas. (2) Methods: Surgical databases in three neurosurgical departments , with approval by the local ethics committee, were retrospectively reviewed to find gangliogliomas surgically removed by a fluorescein-guided technique by the aid of a dedicated filter on the surgical microscope from April 2014 to February 2020. Eighteen patients  underwent surgical treatment for gangliogliomas during 19 operations. Fluorescein was intravenously injected (5 mg/kg) after general anesthesia induction. Tumors were removed using a microsurgical technique with the YELLOW 560 Filter (YE560) . (3) Results: No side effects related to fluorescein occurred. In all tumors, contrast enhancement on preoperative MRI correlated with bright yellow fluorescence during the surgical procedure . Fluorescein was considered helpful by the operating surgeon in distinguishing tumors from viable tissue in all cases (100%). Biopsy was intended in two operations, and subtotal resection was intended in one operation. In all other operations considered preoperatively eligible, gross total resection (GTR) was achieved in 12 out of 16 (75%) instances. (4) Conclusions: The use of FL and YE560 is a readily available method for safe fluorescence-guided tumor resection, possibly visualizing tumor margins intraoperatively similar to contrast enhancement in T1-weighted MRI. Our data suggested a positive effect of fluorescein-guided surgery on intraoperative visualization and extent of resection during resection of gangliogliomas. Gangliogliomas comprise a small number of brain tumors . They arGangliogliomas show high heterogeneity in imaging studies. Calcification has been described on plain skull X-ray and computed tomography (CT), on magnetic resonance imaging (MRI), high T1-weighted imaging (T1WI), short-tau inversion recovery (STIR), and fluid-attenuated inversion recovery (FLAIR) signal. Low T2-weighted imaging (T2WI) signal is common, with no diffusion restriction on diffusion-weighted imaging (DWI) and apparent diffusion coefficient (ACD). Calcification may become visible on T2*/gradient echo sequences (GRE)/susceptibility weighted imaging (SWI) sequences. Contrast enhancement is a common feature ,10.The surgical goal is wide radical resection, depending on the eloquence of the tissue involved, and the course thereafter usually benign ,11. In oApart from standard microsurgical resection utilizing the operating microscope, 5-aminolevulinic acid (5-ALA) fluorescence has been evaluated as an intraoperative tool for improved visualization with limited to no benefit ,14,15. NHere, we present an alternative technique to localize and examine gangliogliomas intraoperatively, ultimately guiding tumor resection, using FL and an FL-specific microscope filter. This multicenter study, to our knowledge, is the first report on the effect of FL on visualization and resection of ganglioglioma.In this study, we systematically and retrospectively reviewed surgical and neuropathological databases at three neurosurgical departments  to identify the cohort of patients with ganglioglioma who had undergone fluorescence-guided tumor resection with fluorescein sodium (FL) at any of the centers between May 2012 and February 2020. Inclusion criteria were informed consent about the off-label use of FL, histopathologically confirmed ganglioglioma, and no known allergy to FL. The retrospective study was approved by the respective institutional review boards.We identified 18 patients  with gangliogliomas, including 15 gangliogliomas classified as WHO grade I, 1 desmoplastic infantile ganglioglioma classified as WHO grade I, one ganglioglioma/subependymal giant cell astrocytoma classified as WHO grade I, and one anaplastic ganglioglioma classified as WHO grade II. All patients underwent preoperative and prompt postoperative (within 72 h after surgery) contrast-enhanced MRI. A review of preoperative imaging determined localization and contrast-enhancing behavior of the tumor. The extent of resection was estimated by a neuroradiologist comparing pre- and postoperative imaging.Data on the clinical and neurologic condition at admission and discharge were available for all patients .Following reports published earlier ,28, a weDue to differences in institutional standards, four patients received glucocorticoids immediately preoperatively  was defined as more than 1 cm3 of contrast enhancement. Furthermore, cases were stratified by whether an identifiable tumor was left behind, due to anatomical or functional considerations intraoperatively, as well as by whether there was an intended biopsy.Gross total resection (GTR) was defined as no residual contrast on postoperative MRI, near-total resection (NTR) was defined as less than 1 cmAll procedures conducted in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.n = 18; 100%), intense homogenous or heterogeneous yellow-green fluorescent staining of tumor tissue was noted. In all cases but one (Pat. 8), intraoperative fluorescence was deemed helpful for improved resection. In the one case, fluorescent guidance was not necessary due to good localization of the lesion and intended STR. No technical difficulties regarding the use of the microscope filter were encountered during the surgical interventions.In all patients . Residual tumor tissue was seen on postoperative MRI in seven cases (33%). In those seven patients, a biopsy was intended in two operations (Pat. 7), and subtotal resection was intended in one . In three patients , GTR was deemed possible according to the preoperative MRI, but intraoperative constraints  limited the extent of resection (EOR). In one patient (Pat. 1), residual contrast enhancement was noted on the postoperative MRI. At baseline, 15/18 (83%) of patients had KPS scores of 90–100, indicating good clinical and neurological conditions. Surgical morbidity led to a postoperative decline of KPS score in five patients at discharge. No long-term follow-up data were available; such data were not relevant to the scope of this study, as we solely focused on the quality of resection validated by early postoperative MRI.We did not encounter any morbidity or mortality attributable to the use of FL. Furthermore, no major side effects related to fluorescein throughout the observation period apart from yellow-colored urine and, in some patients, slight yellow discoloration of the skin were documented.Despite the technical advancements, many surgeries of different lesions of the central nervous system (CNS) still solely rely on visual cues and tactile differentiation when resecting tumorous tissue. To name a few, the operating microscope, gadolinium-enhanced MRI, ultrasound, and neuronavigation substantially extended the armamentarium available to neurosurgeons over the past decades to safely aid maximal EOR. Fluorescence-guided surgery, fitted with the operating microscope, has been part of this evolution. Apart from its off-label use in detecting cerebrospinal fluid leaks, FL has regained interest amongst the neurosurgical community in the past decade. It has been shown to safely guide tumor resection in numerous tumors of the CNS, including but not limited to high-grade gliomas, metastases, and hemangioblastomas ,19,20,21To our knowledge, this is the first report evaluating low-dose FL in ganglioglioma surgery. The use of FL could improve tumor visualization and resection in this entity. Moreover, our observation was associated with gadolinium enhancement on preoperative MRI, which was present in all tumors in our series. Surgeon-reported visibility of tumorous tissue was clearly enhanced by FL and filter view and almost always considered helpful for complete tumor removal.The goal of GTR was achieved in 12 out of 16 75%) operations when GTR was preoperatively considered possible and intended. In 3 out of 16 (19%) of the remaining operations, residual tumor tissue was left behind intentionally to preserve function or limit surgical morbidity, and in 1 out of 16 (6%) operations, residual contrast enhancement was noted on the postoperative MRI. In a study of Varshneya et al., of 198 adult patients with low and high-grade gangliogliomas, the authors found overall gross total resection rate of 59% . Of 348 5% operatNo adversaries to the surgical workflow or ergonomics were encountered. In line with earlier experiences using FL for other intracranial pathologies, no side effects attributable to the use of FL occurred intraoperatively or during the hospital stay.The exact mechanism by which FL accumulates in gangliogliomas is not yet known. This holds for other pathologies of the CNS, evaluated with FL. The striking mutual feature is gadolinium enhancement, indicating an impairment of the BBB and likely accounting for the FL staining, as both are distributed via the same route. Glioma cells can precipitate the breakdown of the blood–brain barrier (BBB), which could enable FL to permeate into the extracellular space, possibly facilitating fluorescence ,25,35. AAlthough intraoperative evaluation of the fluorescence was subjective in this study, the report of helpful fluorescence in almost all cases, particularly at the tumor margins, taken together with a high rate of GTR, suggests a reproducible effect. FL is still undergoing feasibility tests, particularly in combination with the YELLOW 560 nm microscope filter. Final approval of the drug by the respective competent authorities is pending. In Italy, hospitals are reimbursed for the usage of the drug.Optimal dosage and timing of FL in ganglioglioma surgery, as well as its role in redo surgery, is not known. Although not all gangliogliomas are contrast enhancing on preoperative MRI ,10, all Our data suggested a positive effect of fluorescein-guided surgery during resection of gangliogliomas with contrast enhancement on preoperative MRI. We conclude that FL and the YELLOW 560 nm filter are safe and feasible tools. The use of FL and YELLOW 560 nm filter is a readily available method for fluorescence-guided tumor resection, possibly visualizing tumor margins intraoperatively similar to contrast enhancement in T1-weighted MRI.


Teaching Point: The imaging clues to differentiate idiopathic tumoral calcinosis from other calcified soft tissue lesions include: pseudotumoral appearance with mass effect, bone erosion with intra-osseous protrusion of calcification, lobulated morphology, and peri-articular location on the extensor side. Figure 1, white arrow) surrounded by hyperintense edema on fat-suppressed (FS) T2-weighted images (WI). There was a focal bony lesion on the triceps tendon insertion , hyperintense on FS T2-WI and hypo-intense on T1-WI, though surrounded by a subtle peripheral rim with a lower signal. FS T2-WI demonstrated perilesional bone marrow edema.A 68-year-old woman was referred to our department, because of a painful swelling at the extensor side of the right elbow. Magnetic resonance imaging (MRI) revealed an elongated lesion with rosary morphology in the distal triceps muscle belly and tendon. The lesion was predominantly of low signal on all pulse sequences . Computed tomography (CT) was performed to further evaluate its extent, morphology, and internal structure. CT confirmed an elongated lesion with “string of beads” morphology within the triceps muscle and tendon. The proximal part was less dense  than the homogeneously hyperdense distal part . The olecranon erosion had a subtle central calcification and a sclerotic rim .A subsequent conventional radiograph confirmed a well-defined soft tissue calcification with variable density posteriorly to the distal humerus  consists of a peri-articular, calcified soft tissue mass. There is an idiopathic, primary form and a secondary form associated with chronic renal failure and secondary hyperparathyroidism . The disOn MRI, TC is either diffuse hypo-intense on both pulse sequences or heterogeneous with signal voids and areas of high intensity signal on T2-WI, attributed to a granulomatous foreign body reaction . MRI mayMedical therapy is of limited value in idiopathic TC. Complete surgical excision may be considered in larger lesions .


Foci of splenic tissue separated from the spleen can occur as a congenital anomaly. Isolated nodules of splenic tissue are called accessory spleens or spleniculli. However, nodules of splenic tissue can merge with other organs during embryonic development, in which case we speak of spleno-visceral fusions: most often, they merge with the tail of the pancreas (thus forming spleno-pancreatic fusion or an intrapancreatic accessory spleen), with the reproductive gland , or with the kidney . Our case report describes the fusion of heterotopic splenic tissue with the right adrenal gland, which was misinterpreted as a metastasis of a renal cell carcinoma. To the best of our knowledge, this is the first reported case of spleno-adrenal fusion. Spleno-visceral fusions usually represent asymptomatic conditions; their main clinical significance lies in the confusion they cause and its misinterpretation as tumors of other organs. We believe that the cause of retroperitoneal spleno-visceral fusions is the anomalous migration of splenic cells along the dorsal mesentery to the urogenital ridge, together with primitive germ cells, at the end of the fifth week and during the sixth week of embryonic age. This theory explains the possible origin of spleno-visceral fusions, their different frequency of occurrence, and the predominance of findings on the left side. These are asymptomatic conditions; their main clinical significance lies in the confusion they cause and its misinterpretation as tumors of other organs. So far, cases or even smaller groups of patients with spleno-pancreatic, spleno-gonadal, and spleno-renal fusion have been published. Our article presents a case report of fusion of heterotopic splenic tissue and the right adrenal gland. The finding was mistaken for an adrenal metastasis of a renal carcinoma of the right kidney; the correct diagnosis was made by histology after an adrenalectomy. In the discussion, we present a possible embryological basis for this anomaly, but also for other spleno-visceral fusions in the retroperitoneum.2A 79-year-old female patient with dyspnea underwent computed tomography (CT) angiography to rule out pulmonary embolism in October 2017. There was only arterial hypertension, bilateral hip arthroplasty, and laparoscopically assisted cholecystectomy in the patient’s history. A mass on the right adrenal gland was incidentally detected on the CT scan. With this finding, the patient was referred to a urological department in a tertiary medical center. A lesion of the right adrenal gland was confirmed on the subsequent abdominal CT, and in addition, another incidental lesion was found on the right kidney. A tumor on the lower pole of the right kidney measured 45 × 38 × 34 mm, with significant heterogeneous enhancement; it was interpreted as a suspected renal cell carcinoma . The masHistological examination of the right kidney tissue confirmed a clear cell renal carcinoma pT1b, N0, M0, grade I–II . The remThe patient underwent follow-up abdominal CT in July 2018; the finding was without recurrence of the tumor. At the last follow-up clinical check in March 2020, the patient felt in good health condition.Regarding the management of such a case, we believe that spleno-adrenal fusion is an extremely rare anomaly and could not be predicted a priori. In similar conditions that are more common in clinical practice, such as splenosis or accessory spleens mimicking a neoplastic lesion, the suspicion may be confirmed by 99m Technetium heat-damaged red blood cell scintigraphy combined with single-photon emission CT.The patient provided an informed consent to all the diagnostic and therapeutic procedures. Further, the patient provided a general informed consent to the use of the results of the examination methods for the purposes of research and publication, which is included as a part of the informed consent for hospitalization in our department.33.1Foci of splenic tissue separated from the main body of the spleen can occur for several reasons. In general, they are considered asymptomatic and only very rarely is their presence accompanied by complications, such as torsion of a wandering accessory spleen or bleeding caused by spontaneous rupture . The maiSplenosis represents one of the possible causes of foci of splenic tissue occurring in different regions of the body. It is not a congenital condition, but an autotransplantation of the splenic parenchyma at the time of trauma or a splenectomy . If a foThe spleen is derived from a mass of mesenchymal cells located between the layers of the dorsal mesogastrium and begins to develop during the fifth week . The pro3.2Compared to other types of spleno-visceral fusions, fusion with the pancreas is probably the most common. Unver Dogan et al. report the occurrence of heterotopic splenic tissue within the tail of the pancreas in 0.7% of autopsies . Case reThe embryological explanation is relatively straightforward, as the spleen and tail of the pancreas have their embryological origin in the dorsal mesogastrium during the fifth week and lie in relatively close proximity to one another  11]. Th. Th11]. 3.3The connection of the spleen and reproductive glands is the second most common form of fusion; so far about 175 cases have been described . Spleno-The initial stages of gonadal development occur during the fifth week, when a thickened area of the mesothelium develops on the posterior abdominal wall on the medial side of the mesonephros. The proliferating epithelium and underlying mesenchyme create a bulge called the genital ridge  30]. Pr. Pr30]. 3.4Congenital connections of heterotopic splenic tissue and the kidney are much rarer in the literature than are those of the reproductive organs; so far about 13 cases have been published, including four in the right kidney ,34,35,36Three slightly overlapping kidney systems are formed in the urogenital ridge during intrauterine life. The first of these systems, called the pronephros, is rudimentary and nonfunctional. It is presented during the fourth week . The sec3.5Congenital connections of heterotopic splenic tissue and the adrenal gland have been theoretically assumed, but we were unable to find any documented case of spleno-adrenal fusion. There are several case reports of accessory spleens in the right suprarenal space misinterpreted as retroperitoneal neoplasms, but there was no direct connection to the right adrenal gland ,19,20. A3.6Several theories have been proposed that attempt to explain the embryological background of retroperitonal spleno-visceral fusions. To the best of our knowledge, Sneath came up with the first hypothesis in 1913 . He poinIn the context of the above facts, we offer an interpretation of our own finding. In our case, with histologically proven splenic tissue within the right adrenal gland, we primarily ruled out the possibility of splenosis, as there was no splenic trauma or splenectomy in the patient’s history (note the intact spleen in 3.7Spleno-renal and discontinuous spleno-gonadal fusion are usually mistaken for a potentially malignant kidney or testicular tumor, as splenic heterotopy in these organs is a very rare condition and the finding of imaging methods is nonspecific. Also in our case of spleno-adrenal fusion, the lesion was evaluated as an indeterminate adrenal mass and there was a high probability of metastatic adrenal involvement when a renal cell carcinoma was suspected. For spleno-pancreatic fusion or an intrapancreatic accessory spleen, which is relatively more common than other fusions, criteria have been proposed to distinguish the splenic tissue from tumors of the pancreatic tail ,43. In t4To the best of our knowledge, this is the first reported case of spleno-adrenal fusion. We believe that the cause of retroperitoneal spleno-visceral fusions is the anomalous migration of spleen cells along the dorsal mesentery to the urogenital ridge, together with primitive germ cells, at the end of the fifth and during the sixth week of embryonic age. This theory explains the possible origin of spleno-visceral fusions, their different frequencies of occurrence, and the predominance of findings on the left side. We believe that spleno-adrenal fusion is an extremely rare anomaly and could not be predicted a priori. In similar conditions that are more common in clinical practice, such as splenosis or accessory spleens mimicking a neoplastic lesion, the suspicion may be confirmed by 99m Technetium heat-damaged red blood cell scintigraphy combined with single-photon emission CT.


Infantile choriocarcinoma is an extremely rare disease. We present a case study of a 1-month-old male with choriocarcinoma diagnosed simultaneously with his mother. On admission to hospital, the disease was very advanced and massive progression and multi-organ failure caused the death of the patient despite the implemented treatment. It was too late to save the child’s life, but early enough to save his mother. The authors believe that the serum levels of hCG should be determined in every newborn with anemia and liver tumor, especially when the mother has a positive history of miscarriage. Primary infantile or neonatal choriocarcinoma is an extremely rare disease and the most cases involve metastatic disease to the fetus from an intraplacental chorioncarcinoma . When noRetrospective analysis of infantile choriocarcinoma showed that maternal choriocarcinoma was diagnosed in approximately 60% of these infants. Most of these mothers were asymptomatic during pregnancy, even though primary intraplacental choriocarcinoma was developed in uncomplicated third-trimester placentas. Only the development of disease in the mother or infant retrospectively confirmed intraplacental choriocarcinoma .Chorioncarcinoma (CC) next to the pre-malignant conditions like complete or partial hydatiform moles and the malignant ones like placental trophoblastic tumor  belongs to gestational trophoblastic disease (GTD). The exact frequency of CC is less ambiguous than GTD, but some data shows the incidence to be around 1:50,000 deliveries . In the All forms of GTD come from the elements of the normal human placenta, but the exact pathogenesis of choriocarcinoma has not been fully explained. Choriocarcinoma develops from abnormal trophoblastic population cells with hyperplasia and anaplasia. CC is characterized not only by a proliferation of cytotrophoblasts and syncytiotrophoblasts but also by an extensive angioinvasion of myometrium ,7. HistoThere are two forms of choriocarcinoma: gestational and non-gestational. Most of them are gestational in origin, while non-gestational ones are exceedingly rare. . The gesThe exact diagnosis, staging, risk assessment and treatment depend on the type of GTD and the level of hCG are given in the clinical practice guidelines . NeverthWe present a case study of a 1-month-old male with choriocarcinoma diagnosed simultaneously with his mother. On admission to hospital, the disease was very advanced and massive progression and multi-organ failure caused the death of the patient despite the implemented treatment. It was too late to save the child’s life, but early enough to save his mother. The authors believe that in every newborn with anemia and liver tumor, the serum levels of hCG should be determined, especially when the mother has a positive history of miscarriage.A 1-month-old male Caucasian was transferred from the local pediatric ward to the Department of Pediatric Oncology, Hematology and Transplantology because of a suspected liver tumor. In children at this age, hepatoblastoma is the third most common (after neuroblastoma and nephroblastoma) malignant tumor of the abdomen. This was therefore the first suspected diagnosis. Vomiting with traces of blood was observed twice in the two preceding days before admission. In the pediatric ward, anemia was diagnosed and an abdominal ultrasound revealed a liver tumor with rich vascularization. The patient was a full-term neonate, born from a 3rd pregnancy, the 2nd birth, Apgar 10, with a birth weight 3095 g and was a caesarean section due to a lack of progress in delivery. The first pregnancy was complicated by gestational diabetes in the mother, and the second one ended in a miscarriage in the 3rd month of pregnancy (that was 4 months before the third pregnancy).The ultrasound examination performed on admission in the oncological ward showed a massive hypodensive lesion in the right lobe of the liver, which was 5 cm in diameter. The lung X-ray indicated three circular metastatic lesions. Chest and abdominal computed tomography (CT) showed numerous (about 50) metastatic lesions in both lungs, and a liver tumor 5.2 cm × 3.7 cm × 4.8 cm in the right lobe. Lung window axial CT image shows multiple pulmonary rounded nodules of variable size, scattered throughout both lungs .An abdominal nonenhanced (A) and enhanced arterial phase (B) coronal view, arterial phase (C) and portal phase (D) axial view indicate on the hepatic mass with bleeding (A), and central necrosis . After gAn abnormal blood tests were anemia (Hb 6.1 g/dL), thrombocytopenia (77.000/uL), high BNP (1259 pg/mL), elevated total bilirubin (1.71 mg/dL), elevated LDH (651 IU/L), low fibrinogen (88 ng/dL), AFP  and an extremely high hCG level .2 with a dose reduction acc. to the body weight) was started. Despite two days of chemotherapy and intensive treatment, the clinical condition deteriorated rapidly, massive malignant progression in the lungs (X-rays), in abdomen with intestinal and lymph nodes infiltration (USG), as well as in the CNS (USG) was observed. In fourth day of hospitalization, the patient died due to multiple organ failure. The post-mortem histopathological examination confirmed the diagnosis of disseminated choriocarcinoma.The results of the CT and hCG tests authorized the urgent start of chemotherapy. A central catheter had to be inserted before treatment. Unfortunately, after surgery, due to significant respiratory efforts against tachypnoe and tachycardia, the infant was transferred to the ICU and chemotherapy with ethoposide . The mother was treated with chemotherapy for 6 months . Currently, one year after the occurrence of GTD, despite being advised not to conceive until her follow-up is complete, she is pregnant again.The occurrence of choriocarcinoma in infancy (infantile choriocarcinoma) is an immensely rare. The age of the presented patient was typical for infantile choriocarcinoma manifestation. First symptoms of infantile choriocarcinoma usually occurs at a median age of 1 month .Typical early symptoms are anemia, developmental delay, hepatomegaly, hemoptysis or respiratory failure, while there may be also signs of precocious puberty. Our patient was admitted to the hospital because he presented vomit stained with blood. Physical examination showed an enlarged liver and in laboratory tests, anemia was found. Abdominal ultrasound examination revealed a tumor with rich vascularization. Because hepatoblastoma is the most common primary liver tumor in children and is usually diagnosed during the first years of life, suspicion of this tumor has come to the fore. Further imaging and deterioration of the child’s condition forced increased investigations. AFP level was in normal range according to age, which could rule out diagnosis of hepatoblastoma while an extremely high hCG level indicated CC. Such an elevated level of human chorionic gonadotropin is strongly typical for metastatic CC. In all 30 cases reported in Blohm and Göbel’s study, hCG was always elevated . As GetrHowever, in some cases the liver may be the primary site of the choriocarcinoma as well ,18. Yet,A history of miscarriage increases the chances of having molar pregnancy, and hence choriocarcinoma as well. Genetic disorders of gamets might be the basis for both miscarriages and mole pregnancy. For example, diandric triploids are the most frequent chromosome abnormality in infertile males with oligo-, crypto- and azoospermia . MoreoveThe most frequently observed symptom of the maternal disease is intermittent vaginal bleeding after delivery , which aWomen undergoing chemotherapy are advised to avoid pregnancy for 1 year after completing the chemotherapy. During that year, patients are at high risk of the disease recurrence. In addition, a physiological increase of hCG levels during pregnancy may make it difficult to detect and diagnose CC relapse as early as possible. However, women who become pregnant and desperately want to have a baby can be sure of a likely successful outcome .Although the incidence of CC in infants is extremely rare, about a quarter of them show symptoms after birth and the diagnosis can be easily confirmed with hCG. The authors recommend that in every newborn with anemia and liver tumor, the routine hCG testing should be offered both for an infant and for a mother, especially when the mother has a positive history of miscarriage. In addition, particular emphasis should be placed on the histopathological examination of excrecta after miscarriages and deliveries, especially since GTD and intraplacental CC can be easily underdiagnosed because most women are asymptomatic with a macroscopically normal placenta.


Erdheim-Chester disease (ECD) is a rare, idiopathic, systemic non-Langerhans cell histiocytosis involving long bone and visceral organs. Central nervous system (CNS) involvement is uncommon and most cases develop as a part of systemic disease. We present a rare case of variant ECD as an isolated intramedullary tumor.18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) showed an intramedullary mass lesion at T2-T4 with no systemic lesion, which was heterogeneous enhancement pattern with cord swelling and edema from C7 to T6. Gross total removal was achieved for the white-gray-colored and soft-natured intramedullary mass lesion with an ill-defined boundary. Histological finding revealed benign histiocytic proliferation with foamy histiocytes and uniform nuclei. We concluded it as an isolated intramedullary ECD. The patient showed self-standing and walkable at 18-month with no evidence of recurrence and new lesion on spine MRI and whole-body FDG-PET/CT until sudden occurrence of unknown originated thoracic cord infarction.A 75-year-old female patient with a medical history of diabetes and hypertension presented with sudden-onset flaccid paraparesis for 1 day. Neurological examination revealed grade 2–3 weakness in both legs, decreased deep tendon reflex, loss of anal tone, and numbness below T4. Leg weakness deteriorated to G1 before surgery. Preoperative magnetic resonance imaging (MRI) and We experienced an extremely rare case of isolated intramedullary ECD, which was controlled by surgical resection with no adjuvant therapy. Histological examination is the most important for final diagnosis, and careful serial follow-up after surgical resection is required to identify the recurrence and progression to systemic disease. Erdheim-Chester disease (ECD) is a rare, idiopathic, systemic non-Langerhans cell histiocytosis (LCH). It is characterized by foamy histiocytes surrounded by fibrosis, and mainly affects middle-aged adults , 2. The 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) confirmed the single lesion on T2-4 with a maximum standardized uptake value (SUVmax) of 3.14 and no systemic lesion  revealed an intramedullary mass lesion on T2-T4 with cord swelling and edema from C7 to T6. The lesion was iso-intense on T2-weighted images (WI) and iso- to slightly low-intense on T1-WI with heterogeneous enhancement , adult-onset xanthogranuloma, xanthoma disseminatum, Rosai-Dorfman disease (RDD), and ECD . It can max) of the intramedullary lesion when compared with the higher FDG-uptake seen with malignant lesions. Naito et al. [max was higher in CNS lesions of eight ECD patients (5.1–16) than in this patient [Primary or metastatic spinal cord tumors, multiple sclerosis, and sarcoidosis should be considered as differential diagnoses . Old ageo et al.  demonstr patient . These pV600E mutation, although there were foamy histiocytes with CD68 (+) and CD1a (-). Haroche et al. [V600E mutation was detected in 54 % (13/24) of ECD and 38 % (11/29) of LCH, which might benefit from targeted therapy. Negative BRAFV600E mutation in ECD is required to differentiate with RDD and JXG, which are also negative for BRAFV600E mutation [V600E varies depending on the biopsy sites; there was only 13 % (6/46) of positive BRAFV600E in CNS [V600E, we cannot exclude the possibility of ECD because negative BRAFV600E is not identified. Rather, the histological findings are suitable for the confirmation of ECD.In this case, we identified the patient was negative for BRAFe et al.  reportedmutation . HoweverE in CNS . ConsideThe prognosis of ECD appears to be significantly worse than that of other types of histiocytosis, which is owing to the extent of visceral involvement. A progressive course is commonly associated with a high mortality rate, which is found to be 22 % in the literature , 9, 24. Histiocytosis classification has been revised recently with the following five categories: (1) Langerhans-related, (2) cutaneous and mucocutaneous, (3) malignant histiocytosis, (4) Rosai-Dorfman disease, and (5) hemophagocytic lymphohistiocytosis and macrophage activation syndrome . ECD is We experienced an extremely rare case of isolated intramedullary ECD; there were no new lesions or recurrence at 1-year follow-up period after gross total removal of isolated intramedullary ECD lesion without adjuvant therapy. Histological examination is the most important for final diagnosis, and careful serial follow-up after surgical resection is required to identify recurrence and progression to systemic disease.


We present to you a rare case of Crohn's disease involving the ovary in a 28-year-old nulligravida Eritrean patient. This is considered a rare manifestation of Crohn's disease that is believed to be due to fistulization between the ovary and intestines. Crohn's disease (CD) is an inflammatory bowel disorder (IBD) that is progressive in nature, resulting in certain complications such as fistulization or stricture formation that once developed may require surgical intervention . The cliThere are no firm diagnostic features for CD but typical signs include perianal fistulas, large skin tags or abscesses on physical examination , small bA 28-year-old single nulligravida Eritrean patient presented in 2013 to the emergency department with right lower quadrant pain for 3 weeks. Her pain started gradually; it was colicky and throbbing in nature and radiated to her right leg. The pain was aggravated by fatty and spicy food, occurred on and off, and was partially alleviated by painkillers. The pain was associated with nausea, vomiting, bloating, alternating diarrhea and constipation, and episodes of bleeding per rectum. The patient had her first menstrual period when she was 11; she had regular cycles for 7 days until recently, as her period lasted only for 3 days in the two cycles preceding her presentation, with increased premenstruation pain. She reported that she had no weight loss, no loss of appetite, no night sweats, but had palpitations while her other systems were unremarkable. She only complained of eczema and her past surgical history was of an anal fistula in 2010; she had no blood transfusion and no known food or drug allergies, and she is a nonsmoker. Her younger sister was known to have ileal Crohn's disease.She was diagnosed in the ER with appendicitis and underwent emergency appendectomy. After appendectomy, she continued to complain of continuous abdominal discomfort. In March of 2017, the patient returned to the hospital due to recurrence of pain associated with loss of appetite. A CT scan of the abdomen revealed a right adnexal mass inseparable from a thickened and stenosed distal ileum. An exploratory lower midline laparotomy was done that revealed an abscess in the right side of the abdomen in the vicinity of the distal part of the small intestines along with ovarian swelling. She underwent abscess drainage and right ovarian cystectomy only, as the ileum itself was inflamed but with no fat creeping and was not fibrotic, indicating that it itself was not diseased but showed signs of inflammation due to proximity to the diseased ovary. The patient tolerated the procedure well, had an uneventful hospital stay, and was discharged from the hospital three days later.Bacilli and fungal organisms. The final pathological impression was of a diffuse multifocal necrotizing granulomatous inflammation that was consistent with CD (see The histopathological examination of the right ovarian mass revealed infiltration of the ovarian stroma by a mixture of inflammatory cells composed of lymphocytes, plasma cells, neutrophils, and histiocytes, with formation of multiple microabscesses. Collections of epithelioid histiocytes with formation of granulomas were also noted. Some of these granulomas showed central suppuration. There was no evidence of malignancy. Ziehl-Neelsen and Grocott's methenamine silver stains were performed and found to be negative for acid-fast h CD see .There are 18 reported cases of ovarian CD in the literature. Gawron et al. reported a case in 2017 that described a 27-year-old nulligravida patient that was known to have CD who presented with fever, pelvic pain, and a palpable mass. Transabdominal ultrasound confirmed this mass to be located in the right iliac fossa, and hence, a tubo-ovarian abscess was suspected. As a result, the patient underwent diagnostic laparoscopy; a right-sided oophorectomy was performed and upon examining the surgical sample, a granuloma was identified and was suggestive of CD .In August 2016, a total of three cases of ovarian CD were also reported, there were pelvic abscesses documented in the first two cases because of fistulization with the terminal ileum, and an ovarian abscess was noticed in the third case as a consequence of disease adjacency . Rindos Yabushita et al. also presented a case of ileal/ovarian cystoma fistula due to CD. A 22-year-old woman was diagnosed at the age of 20 in the hospital with CD of the large and small intestine; she had right lower abdominal pain and thus was admitted for investigations; GI contrast studies showed no fistulas but CT examination suspected an intestinal ovarian cyst fistula. Surgery was performed and revealed that the ovarian cyst became a lump with the ileum and was diagnosed as ileo-ovarian fistula; partial resection of the ileum and resection of the appendix were performed .Ouakaa-Kchaou et al. reported a case of a 32-year-old Caucasian female that presented with a 1-day history of lower right quadrant abdominal pain that was associated with vomiting and fever; the patient was medically and surgically free with no family history of IBD; a diagnosis of acute appendicitis was in consideration; therefore, the patient was referred to surgery; during laparotomy, a thickened segment of the terminal ileum was adherent to the right edematous enlarged ovary with direct fistulization seen between them accompanied by an ovarian abscess with an ileo-ileal fistula, which led to the suspicion of CD. This led the surgeon to perform an en bloc right salpingo-oophorectomy with a standard ileo-cecal and ileo-ileal fistula resection. On histopathology, a section of the small intestine showed patchy mucosal ulceration, submucosal fibrosis, lymphoid aggregation, fissure ulcers, and transmural inflammation, while the right ovary showed extensive necrotizing inflammation with multinucleated giant cells and epithelioid consistent and confirming CD. Tuberculosis, actinomycosis, and malignancies were excluded. The patient is reportedly doing well after 4 years of follow-up and treatment with azathioprine .Andreani et al. described a case of an ovary with an intact capsule that was macroscopically abnormal in a 28-year-old female patient complaining of a 1-year history of lower abdominal pain, weight loss of 9 kg, and unformed stools. The patient was medically and surgically free and had a negative family of IBD, and her general examination and abdominal examinations were negative .Monneuse et al. reported a case of a 37-year-old female patient diagnosed with CD who came complaining of lower abdominal pain on admission. A right ovarian cyst was associated with inflammatory changes of the pelvis and involved the terminal ileum and sigmoid and was detected by an abdominal gynecologic ultrasound .McCluggage et al. reported four cases of ovarian granulomas that were related to CD. Three of the four cases had similar features, two of them with granulomas that had central suppuration with microabscess formation. The author described them as having fissure ulcerations, lined by inflammatory granulation tissue that extended from the ovarian surface into the parenchyma, and vegetable materials were also identified. In the fourth case that involved the left ovary, the ovarian cortex had several small, well circumscribed, and nonnecrotizing granulomas .Allen and Calvert reported a case of a 58-year-old female patient presented with lower abdominal pain and diarrhea leading to a right hemicolectomy for CD; the patient was symptom-free for 23 years; during her presentation, the disease had recurred and led to the resection of 17 cm of the ileum. 5 years later, the patient had multiple short Crohn's related strictures with fistulas and abscesses into the pelvis; two areas of the ileum measuring 10 cm and 20 cm were excised; one year later, the patient presented with lower abdominal pain and subacute obstruction. The patient was unsuccessfully treated with several courses of steroids and azathioprine, leading to further investigations that showed a short area of Crohn's ileitis and two strictures. The patient underwent a stricturoplasty and a further segment of the ileum next to an inflammatory mass surrounding the ovary was excised; postoperatively, the patient did well. The resected tubo-ovarian mass weighed 48 grams measuring 5 x 5x 3 cm; it was pale with irregular areas of necrosis. Histology showed florid necrotizing and suppurative and nonsuppurative granulomatous salpingo-oophritis, due to fistulization from the adherent bowel. Multiple acute abscesses and fragments of vegetable material were found, adjacent to the fallopian tube and between these areas were many granulomas same as the ones found in Crohn's ileitis [Goldberg et al. described that vesicular fistulas and salpingitis are rare complications of CD after presenting a case of oophoro-vesicular-colonic fistula secondary to CD. Interestingly, the patient reported that during menstruation, she experienced bleeding from the bladder. A single stage oophorectomy, sigmoid resection, and repair of the fistula were done; the patient had complete resolution of her symptoms and preservation of fertility .Honore presented a case of a 30-year-old nulliparous woman who complained of nonspecific symptoms, diagnosed radiologically to have distal ileitis suggesting of CD; the patient had a partial ileo-colectomy along with excision of the adherent right adnexa. Suppurative oophoritis was found in relation to an ileo-ovarian fistula and a noncaseating granulomatous inflammatory response similar to what was observed in the ileum .Frost et al. reported a case of a 26-year-old black female who complained of abdominal pain for 1 year; the patient was diagnosed clinically with cystic ovaries and underwent an exploratory laparotomy that revealed ileo-cecal CD with multiple adhesions to the bowel, peritoneum, and right adnexa; a biopsy of the right ovary and an appendectomy were performed. The ovary showed multiple cysts lined by granulosa cells with scattered epithelioid granulomas beneath the serosal surface .Brooks and Wheeler reported a case of a 30-year-old female patient that was known to have CD who underwent resection of the bowel and adherent adnexa. Granulomatous salpingitis, oophoritis, and enterocolitis were identified histologically. A few features that were not usual for CD included the presence of an adnexal mass and florid atypical proliferation of the tubal epithelium that are similar to the changes that can be seen in tuberculous salpingitis .In this case report, we present a case of ovarian CD, a rare complication that is likely a result of direct fistulization between the bowel and the ovary. As presented, similar cases were previously reported by authors from the United States, Canada, the United Kingdom, Europe, Tunisia, and Japan. Similar to many of these reported cases, our patient presented as a case of acute abdomen that was misdiagnosed as appendicitis and led to surgery. After persistence of symptoms postoperatively, the diagnosis was questioned and an exploratory laparotomy was performed and revealed an enlarged ovary. Following resection of the ovary, the histopathological examination revealed features consistent with CD.Crohn's disease is a progressive disease that can result in fistulization between the bowel and other organs. Involvement of the ovary in females with Crohn's disease is a rare complication that can be missed on initial presentation. A high index of suspicion and careful examination of histological specimens are needed to accurately reach the diagnosis. Management should be individualized but surgical intervention is usually required.


Ectopic spleen is extremely rare. Most cases are congenital, acquired ectopic spleen may be a consequence of surgery or trauma to the spleen. The ectopic spleen in the gastric wall we reported is even rarer.We report a 41-year-old female patient, with a past history of splenectomy, who presented with heartburn. Gastroscopy revealed a swelling in the fundus in the stomach. Ultrasonography and computed tomographic examination suggested the possibility of gastrointestinal stromal tumor. We performed endoscopic resection of the mass. Pathological examination of the resected mass showed ectopic spleen.When a patient with a history of splenectomy presents with a gastric submucosal tumor, ectopic spleen should also be considered in the differential diagnoses. And minimally invasive endoscopic treatment can achieve the purpose of diagnosis and treatment for unobvious submucosal tumors. Ectopic spleen is a rare condition that was first described in 1667 by a Dutch doctor, Van Horne . In prev12/L, platelet count 117.0 × 109/L, and normal blood coagulation parameters. Esophagogastroduodenoscopy (EGD) revealed a swelling protruding into the gastric fundus. Endoscopic ultrasonography (EUS) showed an approximately 20.0 mm × 9.6 mm, oval, slightly hypoechoic mass without calcification, originating from the fourth layer, and protruding into the lumen of the stomach; there was no obvious blood flow signal within the mass, and the boundary was clearly seen. Based on these findings, our provisional diagnosis was gastrointestinal stromal tumor (GIST). Computed tomography (CT) of the abdomen and pelvis showed a nodular soft tissue shadow on the greater curvature of the fundus of the stomach; the picture was consistent with GIST. The spleen was absent. We decided to resect the mass, and performed endoscopic full-thickness resection (EFR) under general anesthesia on January 22, 2019. During surgery, a 2.0 cm × 1.8 cm swelling, with smooth overlying mucosa, was seen in the fundus of stomach. A submucosal injection of 1:10,000 adrenaline + methylene blue + sodium hyaluronate was administered at the apex of the lesion, and the lifted mucosa was incised with Dual-Knife . The tumor was found to be located in the muscularis propria. IT-Knife nano  was used to remove the tumor completely. A small perforation was seen at the bottom of the wound, and so an over-the-scope clip (OTSC) was used to close the wound. Postoperative pathological examination of the excised mass showed ectopic splenic nodules. (Fig. A 41-year-old woman with a history of splenectomy 8 years previously for aplastic anemia was admitted to the 900th Hospital of PLA, Fuzhou, China, with complaints of repeated attacks of heartburn over the last 6 months. The attacks generally occurred after meals. On physical examination, a 13-cm surgical scar was seen on the left lower abdominal wall. No mass was palpable. Laboratory findings revealed red blood cell count 3.59 × 10Ectopic spleen is very rare, with an incidence of 0.05%-0.5%; there are only sporadic reports in literature –4. AccorMost cases of ectopic spleen are probably congenital , 7. AcquThe ectopic spleens in previous reports were generally located in the pelvic cavity, with a few in the left upper abdomen . There hAn asymptomatic ectopic spleen, without complications, does not require immediate intervention. However, surgical treatment may be necessary for patients with recurrent abdominal pain, and especially those with suspected torsion, acute infarction or ischemia, rupture, or bleeding of the ectopic spleen . Our patTo summarize, in patients with a history of splenectomy, ectopic spleen should be considered in the differential diagnoses of a gastric submucosal tumor. EFR appears to be a safe and effective minimally invasive method for removal of gastric ectopic spleen.


Rapunzel syndrome, defined by the presence of a trichobezoar extending from the stomach to the small intestine, is a rare cause of intestinal obstruction. It usually presents with vague symptoms; however, it can also present with complications such as perforation, peritonitis and obstructive jaundice. We report a rare case of a 37-year-old woman with Rapunzel syndrome complicated by acute cholangitis and pancreatitis and analyse the diagnosis and management of this complicated pathology. Although she reported a history of trichotillomania and trichophagia, she had been asymptomatic for ten years. We review the steps of diagnosis, highlighting the importance of a thorough clinical history and detailed examination, with supporting evidence from the contrast-enhanced computed tomography (CECT) scan. She was successfully managed with gastrotomy and trichobezoar removal. She had an uneventful postoperative recovery and was discharged after psychiatric counselling. To our knowledge, this is the first case of Rapunzel syndrome in a young female presenting with both cholangitis and pancreatitis. Named after the fairy-tale Rapunzel, where the titular character has long hair, the first case was described in 1968 .Patients are generally asymptomatic until the bezoar becomes very large and causes intestinal obstruction. Over 70% of the cases are females younger than 20 years and present with nausea, vomiting, pain and abdominal distension . Very ra2A 37-year-old female patient presented to us with complaints of continuous, dull abdominal pain for six months. She reported an acute exacerbation of the epigastric pain with radiation to the back for five days. She had a history of fever for the past five days with a maximal temperature of 101°F, for which she was treated with acetaminophen tablets (500 mg). Additional complaints included halitosis and early satiety. Ten years ago, she was diagnosed with trichotillomania with anxiety and trichophagia. She reported receiving behavioural therapy and medication  for about one year, following which she had no recurrence of symptoms and was not on any medication. She had no history of pica or any eating disorders.The height and weight of the patient were 162 cm and 54 kg, respectively, and the body mass index was 20.6. On clinical examination, she had icterus. Abdominal examination revealed a mobile mass in the epigastrium extending to the right hypochondrium with visible gastric peristalsis. Abdominal ultrasound showed a large heteroechoic intragastric mass. Upper gastrointestinal endoscopy revealed that her entire stomach was filled with hair and the scope could not be negotiated into the duodenum. Laboratory investigations revealed increased bilirubin levels (5.2 mg/dL) and elevated total leukocyte count . The lipase  and amylase  levels were increased. The AST and ALT levels were 53 U/L  and 58 U/L (7–50 U/L), respectively. The ALP level was 158 U/L , and the GGT level was 150 U/L . ESR was also elevated at 50 mm/h, and the CRP levels were not evaluated.The contrast-enhanced computed tomography (CECT) scan revealed an intraluminal mottled mass of mesh-like appearance occupying the entire stomach and extending beyond the second part of the duodenum, delineated by the oral contrast circumferentially, suggestive of a trichobezoar . The lacHence, due to evidence on the CT scan, a preoperative diagnosis of a trichobezoar was made. The large size and high density of the bezoar made fragmentation and removal by endoscopy, a high-risk procedure with high chances of failure. Failure of endoscopy could cause fragmentation and dislocation of parts of the bezoar. Written informed consent was obtained from the patient to perform the surgery, after a clear presentation of all available treatment options.Given the large size of the trichobezoar and the presence of cholangitis and pancreatitis, the patient was scheduled for surgery. The abdomen was entered by a midline incision. The anterior gastrotomy was done between stay sutures which revealed a large, entangled mass of hair in the stomach with the tail extending to the second part of the duodenum . The tai3Trichobezoars are conglomerations of hair that accumulate in the gastrointestinal tract. Trichobezoars are usually present in the stomach. Rarely, there might be an extension into the small intestine called Rapunzel syndrome presenting with intestinal obstruction, as seen in our case . First rThis condition remains asymptomatic for a long time with symptoms appearing only in the late stages. Symptoms are very vague, such as abdominal pain (46.66%), nausea and vomiting (44.44%), obstruction (20%), abdominal distension (8.88%) and weight loss (8.88%) . The cliMajority of the patients have a psychiatric history of trichotillomania, trichophagia or pica  . It is eIn a very small number of cases, it goes undetected for a long period of time causing biliary or pancreatic obstruction, gastric perforation or intussusception. Pancreatitis due to obstruction of Ampulla of Vater by the bezoar has been reported in only four cases of Rapunzel syndrome thus far ref. . IrritatUltrasound may show an echogenic mass, but a CT scan is the diagnostic tool of choice since it provides a clear image of the bezoar delineated by the contrast dye, as seen in our case  13]. En. En13]. Treatment depends on size and location. Successful management involves complete removal as well as ensuring the prevention of recurrence. Previous literature shows that only small bezoars can be successfully removed by endoscopy. There is still a risk of esophagitis and perforation, which is higher with large bezoars . For giaThe case reveals that Rapunzel syndrome can have varied, often non-specific presentations as well as a myriad of complications such as cholangitis and pancreatitis, and it must be high on the differential diagnosis for patients with a psychiatric history.4Rapunzel syndrome is a very rare condition usually seen in young females with trichotillomania and trichophagia. Hence, we report a rare case of Rapunzel syndrome presenting with both acute cholangitis and pancreatitis. A history of psychiatric illness, a thorough clinical examination and confirmation of the diagnosis using investigations is key to making an accurate diagnosis. Surgical removal is the first line treatment for giant bezoars and is associated with the least complications. Hence, when a female patient with a history of psychiatric illness presents with features of intestinal obstruction, a high clinical suspicion must be held for trichobezoar, and regular post-operative psychiatric counselling is of utmost importance to prevent a recurrence.


World Health Organization (WHO) grade I meningiomas are slow-growing and typically benign brain tumors that can often be easily removed by surgery and rarely become malignant. We report the case of a WHO grade I meningioma in a 67-year-old man with multiple extracranial metastases. Meningioma is a benign tumor arising from the arachnoid cells of the leptomeninges and is one of the most commonly diagnosed primary brain tumors in adults . DistantA 67-year-old white man was diagnosed with an incidental parasagittal meningioma in the left frontoparietal lobe measuring 4 x 2 x 2.5 cm on imaging. He initially declined surgical resection and opted for observation. On reimaging six years later, the tumor had grown to 5.5 x 3 x 3 cm and had extended to the superior sagittal sinus and the left premotor and motor areas Figure . Eight yTwo years after completion of the external beam radiation therapy, the patient presented with a sudden transient episode of aphasia. An MRI of the brain showed a slight interval increase in the size of the mass, and he underwent a left frontoparietal craniotomy. Pathologic examination revealed a WHO grade I meningioma.The patient experienced recurrence with increase in size of the meningioma with new bilateral temporal lobe lesions, and was subsequently treated with fractionated stereotactic radiosurgery (SRS). Shortly after completing SRS, he presented with persistent lower back pain. An MRI of the spine with and without contrast revealed lytic lesions in the C4, T11, and L3 vertebrae, suspicious for malignancy Figure . A biopsThe patient was subsequently treated with SRS to the right temporal tip lesion adjacent to the dura and the T11 lesion. Subsequently, he complained of left-sided mid-thoracic pain radiating down his thigh, difficulty with ambulation, and stool incontinence, with MRI consistent with spinal cord compression Figure . He was Surgical resection with the goal of a gross total resection is the mainstay of treatment of meningiomas ,4. RadiaPathogenesis of grade I meningioma metastasis includes tumor invasion of the venous sinuses ,8 and suThe possibility of extracranial metastasis presents challenges when considering the appropriate observation practices for patients with grade I meningioma, with some authors advocating for full body CT imaging as an option for extracranial examination . Recent Grade I meningioma has been recognized as one of the most common intracranial neoplasms, with only a few cases of metastasis described. In patients presenting with aggressive disease or multiple recurrences, further investigation into potential immunohistochemical targets that could signal tumor malignancy should be considered. Checking molecular signatures that herald aggressive behavior may also help prevent repercussions due to diagnostic error.


Hibernoma is a rare benign tumour that was first described by Merkel in 1906. It arises from remnants of brown fat and has a differential diagnosis of lipoma and liposarcoma. This is a case report of a 31-year-old male with a slow-growing mass in the left flank that produced constant pain radiating to the groin. Computerised tomography localised the mass within the external oblique muscle, which showed some heterogeneity and low attenuation. The mass appeared hypodense to muscle on T1 and hyperdense to muscle on T2 weighted magnetic resonance images. Prominent vascularity of the mass was noted. Finally, the lesion was found to be a ‘typical’ hibernoma on core-needle biopsy. It was surgically resected with a cuff of muscle. He recovered without complication, and there is no clinical evidence of recurrence at 6 months. Hibernoma is a rare benign tumour of brown adipose tissue that promotes non-shivering thermogenesis. Brown adipose tissue is presented in the foetus and is gradually replaced by white adipose tissue with advancing age. It persists, however, in varying amounts throughout adult life and may be found in the neck, axilla, mediastinum, periaortic and perirenal zones. In the foetus, brown fat has also been identified in the interscapular area, posterior abdominal wall, suprailiac and peripancreatic adipose tissue, and near autonomic ganglia, in addition to the sites described in adults. It is not surprising, therefore, that hibernomas have often been reported to be present in these locations. In this report, we describe a case of hibernoma with unusual site of presentation in the anterior abdominal wall. This has rarely been reported in the English literature. Differential diagnoses for hibernoma include benign lipoma and malignant liposarcoma. Careful investigation and diagnosis are required because of the very different management of the differential diagnoses.A 31-year-old male was referred to our general surgery team for diagnosis and management of a growing mass in the left flank region. The patient reported incidentally discovering the mass a month ago, and it has since grown and become hard to palpate. He reported a 7/10 sharp constant pain over the flank, which radiates to abdomen and left groin. There were no associated changes to gastrointestinal function or genitourinary symptoms. A systems review was unremarkable. There were no relevant family or social histories. On examination, the abdomen was soft with some tenderness around the firm 5 cm ovoid mass deep in the left lumbar region. The mass was not reducible, and there were no organomegaly or external hernias. The rest of the examination was unremarkable.. Appearances were inconsistent with a simple lipoma and magnetic resonance imaging (MRI) was recommended to rule out sarcoma. The abdominal MRI showed a fairly homogenous mass within external oblique muscle with well-circumscribed borders. The lesion was hypodense to muscle on T1 and hyperdense to muscle on T2 weighted images  scan of the abdomen was done to confirm the diagnosis. Portal venous phase abdominopelvic CT scan showed an ovoid mass within the external oblique muscle with heterogenous attenuation. There was no calcification, inflammatory changes or evidence of direct invasion into the muscled images . There wAt surgery, a 7-cm linear skin incision was made over the mid-portion of the mass. The mass was localised within the external oblique muscle. It was completely excised with a cuff of muscle by dividing the external oblique muscle. The external oblique muscle, aponeurosis and skin were reapproximated in layers.in situ hybridization was used for assessment of MDM2 gene (12q15) amplification, which was negative. This was performed to rule out any atypical lipomatous tumour.Macroscopically, it was a fatty mass measuring 48 × 39 × 35 mm, which on cross-section did not reveal any necrosis, haemorrhagic or fibroseptae formation. Microscopically the lesion was composed of lobules of brown far with abundant macrovesicles . There wThe postoperative course was uncomplicated. He was reviewed as an outpatient at 2 weeks and again at 6 months. There were no ongoing symptoms or evidence of recurrence.Hibernomas usually occurs in the second to fourth decade of life and a higher female prevalence . HoweverHibernomas are typically fatty hypervascular lesions that are grossly similar to lipomas. They are well-defined, encapsulated mobile masses. The colour varies from tan to red-brown, depending on the amount of intracellular lipid. While adult adipocytes have an eccentrically placed nucleus within a clear cytoplasm, hibernoma has a central nucleus with a multivacuolated and granular eosinophilic cytoplasm .Four morphologic variants of hibernoma have been identified: typical 82%), myxoid (8%), lipoma like (7%) and spindle cell %, myxoid. There iHibernomas, though rare, should be considered in the differential diagnosis of slow-growing, fibrofatty tumours. Hibernomas and lipomas are difficult to differentiate on imaging. Therefore, histopathological analysis is always necessary for a correct diagnosis. Complete surgical excision is the recommended modality of treatment because of the discomfort that is often associated with the tumour. There is no reported local recurrence, metastasis or any malignant transformation.None declared.None.


Background: Adrenocorticotropic Hormone (ACTH)-dependent Cushing's Syndrome (CS) is most often caused by a pituitary adenoma. Although rarely, it can also result from pituitary corticotroph cell hyperplasia (CH). Reports on concomitant pituitary lesions including ACTH-producing adenomas and Rathke's cleft cysts (RCCs) have been published. Positron emission tomography (PET), using 11C-labelled-methionine (MET) as a tracer and co-registered with magnetic resonance imaging (MRI) has been shown to be useful in the diagnosis of pituitary collision lesions, however, its role is still under investigation. In this work we present the case of a patient in whom CS was caused by non-adenomatous CH within the wall of an RCC.Case Summary: In 2015 a patient with signs and symptoms of CS was referred to our Department. Biochemical studies repeatedly showed elevated midnight serum cortisol and ACTH levels. Magnetic resonance imaging of the sellar region revealed an RCC and MET-PET/MR showed heterogeneous labelled-methionine metabolism in the vicinity of the cyst's wall. Transsphenoidal surgery resulted in rapid, complete and lasting relief of symptoms. Histopathological examination demonstrated an RCC and CH.Conclusions: Concomitance of pituitary focal lesions is a rare phenomenon. Methionine-labelled PET/MR may be useful in the diagnosis of collision sellar lesions, including CH. Corticotroph cell hyperplasia can present as mild and fluctuating hypercortisolaemia. Rathke's cleft cyst (RCC) is a frequently encountered lesion of the sellar region, found in about 20% of autopsies . SymptomThe diagnosis of CS has remained a challenge in clinical practice, with limited sensitivity and specificity of laboratory and imaging tests. For this reason clinical examination, and sometimes long-term observation, can be key to correct diagnosis. However, conflicting with this is the fact that early implementation of appropriate treatment can be invaluable, because this can prevent the development of numerous organ complications which can lead to increased mortality and reduced quality of life .11C-labelled methionine as a tracer and co-registered with MRI (MET-PET/MR), has been reported to provide valuable information in the diagnosis of pituitary adenomas  findings. Recently, positron emission tomography (PET), using adenomas , 4. Howein vitro studies have contributed to an understanding of the pathophysiological basis of corticotroph adenomas (CA) as well as their varying clinical picture. It is hypothesised that silent corticotroph adenomas (SCA) originate from the intermediate lobe, while adenomas causing full-blown CS originate from anterior lobal cells , plethora, and dorsocervical fat pad . Adrenocorticotropic Hormone levels were above the upper limit in the morning and at midnight (59.2 and 58 pg/ml). Correct inhibition of cortisol production was found in the 1 mg overnight dexamethasone suppression test  and urinary free cortisol level was within the normal range .11C-methionine 20 min after injection of 720 MBq of the tracer. Time of acquisition was 15 min per bed. Positron emission tomography reconstruction was done using OSEM 3D. Magnetic Resonance sequences T2 Blade, T2 TSE were performed for the whole brain and T1 MPR, T1 TSE, T2 TSE sequences were performed for the sella. Slice thickness was 1–2 mm. The study showed a 6 × 3 mm cyst anterior to the pituitary stalk and the structure of the pituitary gland as being slightly thicker on the left side. Heterogenous 11C-methionine metabolism was observed around the cyst with a peak of tracer uptake on the left side of the cyst's wall  of the chest and abdomen and scintigraphy of somatostatin receptors were performed. No pathology was found with these tests. The patient was rehospitalised in July 2016. Loss of the physiological circadian rhythm of cortisol secretion was again observed by high midnight serum cortisol level (10.6 μg/dl). Complete suppression of cortisol was again found in the low dose DST and urinary free cortisol measured twice was within normal limits. The other hormone levels were: Thyroid-Stimulating Hormone (TSH): 1.75 μIU/ml, L-Thyroxine (FT4): 1.26 ng/dl, Prolactin: 22.08 ng/ml, Follicle-Stimulating Hormone (FSH): 3.34 IU/ml, Luteinizing Hormone (LH): 1.45 mIU/ml, Growth Hormone (GH): 0.04 ng/ml .2). She developed stage 1 hypertension and treatment with ramipril was initiated. Proximal muscle weakness worsened and the patient was unable to hold a hairdryer sufficiently long to blow-dry her hair. After consideration of the whole clinical picture, and the results of the imaging tests, and after neurosurgical consultation, the decision was made to perform transsphenoidal pituitary resection. This surgery was performed in January 2017. During the operation, after opening of the sella, a cystic lesion in the upper part of the pituitary gland was exposed. The structure of the gland surrounding the cyst wall had a morphology resembling a pituitary adenoma.During the next few months the patient's overall health status worsened. Due to problems with concentration and short term memory she was unable to continue work in a bank. The patient's body mass had increased by 3 kg despite attempts at a dietary regime (BMI 32.7 kg/mLow ACTH (6.78 pg/ml) and cortisol (0.64 μg/ml) levels were found postoperatively. Secondary hypothyroidism, secondary hypogonadism and postoperative diabetes insipidus were diagnosed. Histopathological examination demonstrated an RCC, numerous ACTH+ cells and to a lesser extent TSH+ cells. Adrenocorticotropic Hormone positive cells had penetrated the connective tissue of the RCC. Reticular fibres retained a regular pattern indicating CH . The diaAfter the operation, the patient gained a significant and rapid improvement in mood and concentration and was able to resume work. Increased appetite, myopathy, hypertension resolved, and the plethora and dorsocervical fat pad disappeared. Over the next few months, a mass reduction of 21 kg was observed. The presented case provides an example of the coexistence of two focal lesions in the pituitary and addresses the diagnostic difficulties concerning this rare entity. To our knowledge, it is the first description of CH concomitant with an RCC. Additionally, the authors did not find any other description of the use of MET-PET/MR in diagnosing CH in humans.Collision lesions in the pituitary have previously been observed and knowledge about this phenomenon is primarily based on case reports, which may suggest an element of randomness. The challenge in diagnosing concomitant lesions results from the fact that an adenoma, located in a pituitary compressed by a cyst, may be undetectable by conventional MRI . In fact11C is therefore a promising tool in localising regions with increased peptide synthesis, including pituitary adenomas. Studies have shown a very high sensitivity of this method in localising these tumours . In addition, as reported by Alwani et al. , high evThyroid-Stimulating Hormone positive cell proliferation in our patient, although found to a significantly lesser degree compared to ACTH+ cell proliferation, remains difficult to explain. As mentioned above, the patient also suffered from primary hypothyroidism, so the proliferation of this cell line could have been a physiological response to hypothyroidism. Another explanation is that the resected pathological lesion was plurihormonal. Immunochemistry for transcription factors is currently unavailable in our centre and was not performed.The authors are aware of the limitations of the study. Inferior Petrosal Sinus Sampling (IPSS), which is a gold standard in differentiating ectopic from central CS, was not performed as it is not available in our centre. However, studies have shown that IPSS may give false negative results in cases of cyclical CD , 63, 64.Cushing's Syndrome still remains an underdiagnosed disease . As RCCsMethionine-labelled PET/MR may prove useful in detecting pituitary-cell proliferation and the usefulness of PET in detecting hyperplasia has recently been demonstrated in an animal model by Balcerzyk et al. . HoweverConcomitance of pituitary focal lesions is a rare phenomenon. Methionine-labelled PET/MR may be useful in the diagnosis of collision sellar lesions, including CH. Corticotroph cell hyperplasia can present as mild and fluctuating hypercortisolaemia.The datasets generated for this study are available on request to the corresponding author.Ethical review and approval was not required for the study on human participants in accordance with the local legislation and institutional requirements. The patients/participants provided their written informed consent to participate in this study. Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.KS: collected data, performed literature search, and drafted the manuscript. EA-M, KS, LS, BM, and AS: performed medical and surgical procedures. AS: made critical revision and contributed to the writing of the manuscript. PN: prepared histopathological results. All authors revised, approved the final manuscript, and agreed to be accountable for the content of the work.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


The diagnosis of type IV branchial cleft cyst (BCC) according to the Bailey classification is very challenging due to lack of specific clinical manifestations in the early stage of the disease. Here, we present the transoral surgical route of endoscopic resection of second BCC in the parapharyngeal space (PPS) with good outcomes.A 21-year-old man with a 1-year history of snoring complained about sore throat for 1 month and a fever that lasted for 3 days.9 white blood cells (WBCs)/L and 14.94 × 109 neutrophils/L. A cervical computed tomography (CT) examination revealed a mass with liquid density of 6.2 × 4.0 × 7.7 cm3 in the left parapharyngeal space (PPS) and pharyngeal cavity stenosis. Postoperative pathology showed the existence of lymphoepithelial cysts (left PPS), which was in accordance with the diagnosis of BCC.On admission, physical examination revealed a temperature of 39°C, pain when swallowing accompanied with a lump sensation in the throat, and inability to open mouth more than 3 cm. Blood testing revealed 19.29 × 10The patient was administered 1.5 g ceftazidime every 12 hours, anti-inflammatory drugs, and incision drainage was performed subsequently. Then, endoscopy-assisted resection of the left PPS was performed via the transoral route. We used low-temperature plasma and an 8-Fr Foley catheter with a water capsule during the surgery.After resection of the mass, the patient's blood results returned to within the normal range and his symptoms improved. Five days postoperatively, the incision made in the palatine arch of the pharynx opened up by 1 cm, and eventually the wound and laceration healed. Normal oral eating was restored, and no complications were observed.Magnetic resonance imaging (MRI), and color Doppler ultrasound can be useful to diagnose BCC in PPS, which rarely occurs in the clinical setting. Extended endoscopy provides a satisfactory surgical field for trans-oral resection allowing complete resection of the BCC without serious postoperative complications. Second branchial cleft cysts (BCCs) are developmental cyst and are the rarest type of PPS tumors. Bailey et al classified BCCs into 4 types based on location: Type I is located in the anterior edge of the sternocleidomastoid muscle and the deep surface of the latissimus dorsi muscle; type II is the most common type of BCC and is located behind the submandibular gland on the superficial surface of the sternocleidomastoid muscle and the lateral side of carotid space; type III BCC is located between the carotid bifurcation and the external carotid artery in the lateral wall of pharynx; and type IV is located in the interval of the pharyngeal mucosal space. The diagnosis of type IV BCC according to the Bailey classification can be very challenging due to the lack of specific clinical manifestations in the early stage of the disease; thus, this type of tumor can be easily misdiagnosed. Furthermore, considering the anatomical position of the PPS, the important neurovascular structure is complex, the operation is difficult to perform, and the associated risk of operation is high. In particular, when that tumor is very close to the skull base, it is difficult to expose the upper end, and attempting to separate the tumor without direct visualization can easily lead to the rupture of the cyst, resulting in incomplete resection and serious complications caused by the neurovascular injury.Head and neck tumors of the parapharyngeal space (PPS) are sporadic and have an incidence of approximately 0.5%. Based on previous retrospective findings, including 1293 cases,With the development of endoscopic procedures, there is a better possibility to remove some tumors located in PPS via an intraoral approach. In the present study, we reported a single case of a patient with a giant BCC  in the PPS who underwent endoscopic resection. We utilized low-temperature plasma and an 8-Fr Foley catheter with a water capsule during the surgery, which consequently improved the postoperative outcomes.22.19 white blood cells (WBCs)/L and 14.94 × 109 neutrophils/L. All other values were within the normal range.A 21-year-old man with a 1-year history of snoring who had a sore throat for 1 month and fever for 3 days was admitted at our hospital on September 20, 2018. Physical examinations revealed a body temperature of 39°C, no difficulty in breathing, hoarseness, and pain when swallowing, accompanied with a lump like sensation in the throat. No facial numbness was observed. He could not open his mouth more than 3 cm, the congestion in the pharyngeal mucosa was reddish, and the bilateral tonsils were hyperemic. The mucous membrane of the left palatal arch was swollen, smooth and the swelling crossed the midline. The patient had soft, weak neck, with pressure pain on the mass. A clear boundary was observed around the left submandibular gland. In addition, the patient had a history of upper respiratory tract infection. Peripheral blood testing showed 19.29 × 103 in the left parapharyngeal space and pharyngeal cavity stenosis. The bilateral pharyngeal recess, pharyngeal orifice of the eustachian tube, skull base, and the cervical spine remained intact, and the cervical lymph nodes were not enlarged  and neutrophil (3.58 × 109/L) counts returned to the normal range, and bacteriological culture of the puncture fluid yielded no microbial growth.The patient was administered 1.5 g ceftazidime every 12 hours and anti-inflammatory drugs. Puncture incision and drainage was performed at the most swollen portion on the left side of his mouth and pharynx. His symptoms remarkably improved after 200 ml of the purulent discharge was drained. The patient then underwent cervical computed tomography (CT) examination, which revealed a liquid density of 6.2 × 4.0 × 7.7 cm9 leukocytes/L and 2.73 × 109 neutrophils/L.On March 13, 2019, the patient complained of having a pharyngeal foreign body sensation, yet he had no fever, swallowing pain, expiratory dyspnea, and no obstruction when opening his mouth. He was admitted to the hospital again and underwent CT and magnetic resonance imaging (MRI). The cervical spine CT scan showed a well-defined, homogeneous, cystic mass in the left PPS with the dimensions 5.2 cm × 4.2 cm × 7.6 cm; this mass displaced the carotid sheath and its structures on the posterolateral side. An MRI of the pharynx and larynx revealed that the volume of the left PPS was approximately 5.1 cm × 2.9 cm × 7.6 cm, with an abnormal signal shadow, uneven signal intensity, high signal intensity on T2WI, high signal intensity with lipid suppression, clear boundary, separation of internal shadow, and limited diffusion. The left pharyngeal recess could not be seen, whereas the right pharyngeal recess, the pharyngeal orifice of the eustachian tube, and the skull base and cervical vertebrae were not damaged  were explained in detail to the patient, after which he signed a written informed consent. Endoscopy-assisted resection of the left PPS via the transoral route was performed on March 15, 2019, after the patient was placed under general anesthesia. The patient received 1.5 g of ceftazidime 30 minutes before the start of the operation. Briefly, his head was pushed back and an opening device manufactured by Davis Stage Cementing Collars and Equipment  was used. The device was placed in the same position as that used for adenoid removal, following which the catheter was inserted into the nasopharynx via the contralateral nasal cavity and fixed in place by hanging on to the soft palate. Consequently, the mucosal and submucosal tissues were cut in the arc line  with the iso-ionizer MC403 . A 0° nasal endoscope  was used. The palatine and lingual muscles were then bluntly separated, as well as the supra-pharyngeal constrictor muscle located in the medial PPS, and the cyst wall and with the outer capsule of the tumor capsule by electrocoagulation. To prevent the rupture of the thin capsule wall and appearance of the residual recurrence of the capsule wall during separation, middle curved hemostatic forceps were used to properly separate the space around the capsule wall. Consequently, an 8-Fr double cavity water capsule enclosed in a Foley catheter was inserted along the lateral dorsal membrane of the cyst. Next, 3 to 5 ml of water was injected into the capsule to enlarge the space, allowing the hemostatic forceps to adequately re-separate the surrounding space after the bipolar electrocoagulation from the surrounding tissue under the guidance of direct vision. Water was then pumped through the catheter to enlarge the capsule, and the hemostatic forceps and the water capsule were alternately moved forward to expand the cavity. This created a satisfactory visual field to observe, distinguish, and protect the important blood vessels and nerves, and achieve meticulous blood-free separation for endoscopic endonasal surgery.The medial, anterior, and superior skull base, and the lateral and posterior regions of the tumor were separated. Bipolar electrocoagulation or gauze compression were used to stop the bleeding in case of intraoperative bleeding, ensuring smooth proceedings. Nevertheless, during the separation of the lower pole of the cyst, the cyst wall was accidentally ruptured when the root pedicle was severed by a plasma knife. After aspirating approximately 200 ml of milky white capsular fluid, the capsule was completely removed along the capsule wall guided by endoscopic vision, and the cavity was carefully examined. After sufficient hemostasis was achieved, the cavity was repeatedly washed with 2% hydrogen peroxide solution, dilute iodophor, and saline. Finally, the left tonsil was resected. No active bleeding was found in the cavity, and the cavity was sprayed with ceftazidime powder spray. One piece of yarn and 1 piece of gelatin sponge were used for filling the cavity, and the PPS was closed and the pharyngeal contractile muscle, submucous membrane, and mucous membrane were individually sutured. One rubber drainage tube was retained in the subpolar incision of the surgical cavity, and the other end of the thread from the left nasal cavity was fixed in the nasolabial groove to prevent shedding. Intraoperative bleeding was approximately 20 ml. To prevent postoperative dyspnea caused by swelling of the oral and pharyngeal mucosa, a prophylactic tracheotomy was performed and antibiotics and steroid hormones were administered.3The limitation of mouth opening occurred immediately after the surgery due to stimulation of internal pterygoid muscle, but this gradually resolved after a few days. The drainage strip and normal flow nasal cannula were withdrawn at 3 and 5 days postoperatively, respectively. Five days postoperatively, the incision made in the palatine arch of the pharynx opened up by 1 cm. Subsequently the wound and laceration healed, normal oral eating was restored, and no complications were observed. After 1 week, the patient recovered and was discharged from the hospital. Postoperative pathology showed the presence of lymphoepithelial cysts (left PPS), which was in accordance with the diagnosis of BCC Fig. . A follo4The main findings of this study are as follows: although cervical incision surgery is the most common approach to treat BCC in PPS, for this particular case, endoscopy-assisted minimally invasive surgery with a catheter water capsule was found to be the better treatment option. Compared to conventional cervical surgery that entails creating a large incision, the endoscopy-assisted and catheter-based surgical approach can fully expose the surgical field, reduce surgical injury, and complete resection without inducing scar formation. A CT, MRI, and color Doppler ultrasound can be useful to diagnose a BCC in PPS, which is a rare occurrence in the clinical setting. Most of the PPS tumors are of salivary gland origin. Some researchers have suggested that shifting of the soft palate to the opposite side and the bulging of the pharyngeal wall as well as swallowing obstruction and in some cases dyspnea, tinnitus, ear tightness, and hoarseness may be indicators of PPS tumor.,6 Neurogenic tumors are common when the tumor is located in the PPS. These tumors often manifest in the deeper part of the ipsilateral submandibular gland and as medial mass of the lower jaw. When the tumor compresses the adjacent intracranial nerve and sympathetic trunk, then hoarseness, dysphagia or dyspnea, tinnitus and ear tightness, Horner sign, and soft palatal deviation caused by ipsilateral pharyngeal lateral wall compression can appear. Further growth of the tumor can lead to invasion into the brain through the foramen ovale, anterior hypoglossal nerve tube, jugular foramen, and other structures.–10Most PPS tumors are benign and asymptomatic, while a small percentage of these tumors may be malignant. Due to their anatomic location, PPS tumors represent a therapeutic challenge and can be diagnosed using physical and imaging examinations. explained the existence of parapharyngeal cysts without sinus or fistula. They found that the branchial arch appears at the beginning of the fourth week in human embryos, and is bilaterally symmetrical. One to 4 pairs of branchial arches appear at the end of week 4, while the fifth pair is rarely observed in humans. There are 5 pairs of branchial sulci between the arches. The first pair of branchial arches develops into the maxillary eminence and mandibular eminence, which are involved in the formation of facial surfaces. The second pair is involved in the formation of the hyoid bone and neck, and it grows rapidly to the tail, gradually covering pairs 3 and 4. The space between the second pair and the other lower branchial arch spaces is called the cervical sinus, and this undergoes atresia as part of normal development and forms the neck. If abnormalities in the normal embryonic development lead to non-closure of the cervical sinus, the residual epithelial tissue forms a branchial cyst. Depending on the sites, if the cyst is located between the mandibular angle and the hyoid, it originates from the second branchial cleft, and this type of BCC has a high incidence of occurrence. Bailey divided the second BCC into 4 types according to its relationship with cervical tissue. Of the 4 types in the Bailey classification, type IV is the rarest. Some researchers consider that the retained branchial plate can cause ectoderm- endoderm adhesion in the absence of mesoderm arch insertion or branchial plate mouth rupture, and that the epithelial lining of the cyst can be formed under the endothelium of the palatine tonsil, and no fistula can enter the oropharynx. Some researchers have proposed the theory of “benign lymphoepithelial cyst,” considering that most cyst specimens have lymphoid tissue. It has been considered that epithelial cells of the salivary gland that became trapped in the cervical lymph nodes during the embryonic stage have cystic lymph node lesions, or “benign lymphoepithelial cysts.” Furthermore, Jehad et al reported the rare case of a second BCC in a 9-year-old child and found lymphoid accumulation in the cystic wall with scaly epithelial lining.The cause of BCC remains unclear. Some researchers believe that BCC originates from the residue of branchial cleft epithelial cells; others consider it to be caused by 1 or more factors such as incomplete branchial sulcus closure, rupture of the closure membrane between the branchial sulcus and pharyngeal capsule, abnormal development of the branchial organ, and retention of the cervical sinus. Paczona et alThe second BCC grows slowly and often discovered incidentally as painless masses in the neck or parotid gland, which can be accompanied by intermittent distension and pain in the neck or pharynx, especially when swallowing. When complicated with upper respiratory tract infection and inflammation, the swelling becomes aggravated with obvious pain, the mass increases rapidly, and can even rupture. Some patients develop fever and hoarse voice or dyspnea. A combination of cervical CT and MRI scans can be used to examine the approximate location, size, range, smoothness and texture of the tumors, and the anatomical relationship of the tumors with the surrounding blood vessels and nerves. When infection or bleeding occurs, the fibrosis of the capsule wall thickens, and a large number of cholesterol crystals and foreign body giant cell reactions occur in the wall. Consequently, the capsule wall becomes more closely adhered to the surrounding tissue, leading to difficulty in the separation of the capsule wall during surgery.In the present study, we report a patient with a giant BCC (approximately 8 cm in diameter) in the PPS, who was treated with endoscopic resection. After conservative treatment, cervical spine CT showed a well-defined, homogeneous, cystic mass in the left PPS with the dimensions 5.2 cm × 4.2 cm × 7.6 cm, displacing the carotid sheath and its structures on the posterolateral side. The cystic mass compressed the left tonsil anteriorly, leading to severe narrowing of the oropharyngeal passage. Although the patient's symptoms were obviously alleviated, one-stage resection was recommended for complete resection and to prevent recurrence. Hence, endoscopy-assisted oral resection of the cyst was selected based on the following considerations:1.,15The cysts were big and located on the medial side of the internal carotid artery, resulting in the outward displacement of large vessels and intracranial nerves. Under endoscopic guidance, the main vessels, nerves, small lesions, and microvessels were carefully identified. This would not be possible using the conventional cervical approach, which may significantly increase the risk of massive bleeding and adjacent craniocerebral nerve (IX–XII) injury.2.Low-temperature plasma equipment can be used to wash, stop hemostasis, cut synchronously, and solidify the small blood vessels around the tumor, which can effectively reduce bleeding and shorten the operation time. The tumor can be exposed by a submucous incision of pharyngeal contractile muscle on the side of the pharyngeal wall. The submucous incision requires less suture time. Although the cervical approach has a wide field of vision, electrocoagulation and suction devices should be used during the operation, and the skin, subcutaneous tissue, and latissimus cervicalis muscle should be cut open in turn and then sutured after the operation. It is obvious that the endoscopic transoral approach has great advantages over the cervical approach in terms of operation time and bleeding volume.3. In these patients, the BCC was located in the narrow PPS and the tension was very high. Once the capsule was opened, all the fluid was instantly ejected, leading to the unclear identification of the capsule wall epithelium and recurrence of the residual cyst wall epithelium. In order to achieve complete resection, we used hemostatic forceps to “explore the route,” and Foley catheter rotation and water capsule separation for cavity expansion were used for “soft” separation and pole electrocoagulation was used for separation of adhered tissues. The alternative “move-forward” method can be used to achieve complete resection of cysts along the capsule. Although the capsule fluid was ejected by accidental rupture of the capsule by ultrasonic knife when the polar root pedicle was severed, it ensured complete removal of the residual cyst epithelium of the root pedicle under endoscopic guidance.Simple incision, puncture, and cyst aspiration or injection of sclerosing agents can lead to the residual cyst epithelium retention and increase the risk of recurrence. A previous study reported the resection of a large tumor after volume reduction by opening the capsule to extract part of the capsular fluid. Similarly, there have been successful reports of ethanol injection therapy in patients who do not wish to undergo operation.4. but the author believes that recurrence may still occur postoperatively due to infection and pouch mouth atresia. In this case, although the operative cavity was filled with absorbable hemostatic material and indwelling drainage strip after cystectomy, the incision still opened up after operation. To sum up, we believe the negative pressure drainage tube should be retained to promote the healing of the PPS and to prevent incision opening after the surgery. The upgrade of incision grade makes it necessary to combine broad-spectrum antibiotics with antianaerobic antibiotics after caliber surgery, and steroid hormones are used to prevent dyspnea due to edema. Meanwhile, based on our personal experience, we find that general anesthesia via contralateral nasal intubation is more beneficial than direct oral intubation for this surgery. Broad-spectrum antibiotics with antianaerobic antibiotics, and steroid hormones can be used to prevent dyspnea due to edema.The oral approach provides the best aesthetic effect, as it avoids the scar and functional impairment caused by a long neck incision. Yet, when using this approach, the styloid and surrounding adjacent muscles should be used as anatomical landmarks during medial approach surgery. Recent studies have described the treatment of cyst bagging via oral approach,In summary, a CT, MRI, and color Doppler ultrasound are useful techniques to diagnose BCC in PPS, which is rare in the clinical setting. The extended use of endoscopy and 8-Fr catheter water capsules for separation of the outer wall of the cyst are satisfactory surgical approaches for trans-oral resection, allowing for complete resection of the BCC with no serious postoperative complications. However, preventive tracheotomy is still recommended to prevent the occurrence of postoperative complications of dyspnea caused by bleeding and swollen mucous membrane.Conceptualization: Shan Gao, Qin Xu.Data curation: Shan Gao, Qinchuan Yi.Formal analysis: Shan Gao, Qin Xu, Qinchuan Yi.Investigation: Shan Gao.Project administration: Qin Xu.Writing – original draft: Shan Gao.Writing – review & editing: Qin Xu, Qinchuan Yi.