Cystic adenomyosis is an unusual form of adenomyosis, characterized by a well-circumscribed cavitated endometrial gland and stroma, ≥ 1 cm in diameter, located within the myometrium. Few cases have been reported in the gynecological literature, with confusing naming such as: juvenile cystic adenomyosis, cystic myometrial lesions, cystic adenomyoma or juvenile adenomyotic cysts. The current preferred terminology is accessory cavitated uterine mass /or malformation (ACUM). We report here the cases of two 17 and 18 -year-old nulliparous women, who complained of severe dysmenorrhea early after the onset of menarche, with none or partial efficiency of medical treatment. MRI findings, with a follow-up in one case and surgical treatment in both cases, are described with an emphasis on physiopathology. The typical MR appearance is a large well-circumscribed round mass within the external myometrium, composed by an inner cystic hemorrhagic layer surrounded by a thick fibrous crown. The first-line treatment is laparoscopic surgery with mass resection. This typical MRI pattern must be a part of the knowledge of the radiologists. MRIMagnetic resonance imagingACUMAccessory Cavitated Uterine MassAs stated by Bird It may be focal or, more frequently, diffuse with several cystic spaces filled with blood, usually small, 5 mm in greatest diameter and typically located within the junctional zone.The cystic form is an unusual finding characterized by a unique larger hemorrhagic cyst lined by endometrium and located within the myometrium. The physiopathology of this entity is incompletely understood, as its MR appearance. We report two cases with a typical MR imaging pattern and surgical and pathological correlation.Case 1: Fig. 117-This 17-year-old nulliparous girl presented disabling dysmenorrhea and abdominal cramping, leading to several hospitalization. Her first menses occurred at the age of 12 years, and her menstrual cycle was more or less regular. She was occasionally sexually active and no contraceptive pill was taken.In the past year, the pain had become more severe and refractory to any analgesic or non-steroidal anti-inflammatory drugs. There was no dyspareunia or dyschezia. Biological routine tests including complete blood cell count, urinary analysis and erythrocyte sedimentation rate were within normal limits. CA 15.3, carcinoembryonic antigen, and b-human chorionic gonadotropin levels were normal, whereas CA 125 level was slightly elevated (40U/mL).The pelvic examination found a painful mass in the left part of the pelvic cavity.Transvaginal ultrasound revealed a well-circumscribed mass along the external part of the myometrium with a heterogeneous hypoechoic center surrounded by an annular vascular thick rim a.On Pelvic MRI b and c, Laparoscopic surgery was undertaken. The mass was found in the uterus close to the left round ligament. Aspiration of chocolate-colored fluid from the cyst was done before its radical excision after an easily finding of correct surgical planes. The pathological examination confirmed the diagnosis of hemorrhagic cystic lined with endometrial glands and stroma surrounded by smooth muscle hyperplasia, consistent with a cystic adenomyoma.Case 2: Fig. 2A 2A 23-year-old nulliparous young woman was referred to our department due to long standing chronic major dysmenorrhea and dyspareunia, with no response to analgesic drugs. She had no previous medical story. Her first menses occurred at the age of 13 years. She localized the complaint mainly in the left part of the pelvic cavity, starting one week before the onset of menses and disappeared 2-3 days later.A first MRI performed in 2015 revealed a 15 mm rounded formation of the left uterine horn, with an intra-myometrial topography, distinct from the endometrial cavity, with a central hypersignal T2-weighted surrounded by a thick hypointense fibrous crown a. No perShe was re-referred in November 2020 for disabling dysmenorrhea and induration of the left uterosacral ligament on clinical examination. The intensity of the pain was becoming progressively very intense without efficiency of the medical treatment. A second pelvic MRI examination found an increased in size lesion measuring 30 mm, with a larger central fluid and a hypointense crown b. It wasThe surgical exploration revealed a round 3 × 3-cm uterine mass arising from the left lateral uterine wall and extending toward the left side of the broad ligament with smooth border. Small foci of endometriotic lesions were also noted on the left uterosacral ligament. The external layer of the mass was incised, and chocolate-colored fluid was drained. The appearance of the tissue lining the cyst wall was similar to that of a normal endometrium. The cyst wall was then enucleated, leading to its complete resection from the surrounding myometrium e and f. Adenomyosis of the uterus is a very common imaging finding Cystic lesions within the myometrium are uncommon. They are classified into two main groups: congenital and acquired. Acquired cysts include the cystic degeneration of uterine leiomyoma, the cystic adenomyosis and serosal cysts. Congenital cysts derive from Mullerian duct anomalies such as a noncommunicating rudimentary horn and a unicornuate uterus.The clinical context is very suggestive for adenomyosis. This particular cystic form occurs in adolescents and women younger than 30 years, with intense dysmenorrhea appearing shortly after puberty and more or less resistant to different medical treatment. This severe manifestation can be attributed to the recurrent intra-cystic bleeding producing a distension of the cystic cavity.Few cases have been published mainly by gynecologists and using various terms, such as juvenile cystic adenomyosis, adenomyotic cyst, cystic adenomyoma, and cystic adenomyosis Several explanations have been raised ,5.An attractive hypothesis was proposed by Takeda et al By considering patient age at symptom occurrence (younger or older than 30 years), some authors have classified these lesions into 2 categories: the adult and the juvenile form. The adult form seems to be the result from a traumatism at the endometrium-myometrium interface, e.g., after uterine instrumentation The last hypothesis consider that these two forms have the same underlying process and are related to a cystic variant of adenomyosis, sometimes associated with another localization of endometriosis or adenomyosis In the recent literature, the term of ACUM  is now preferred to the others previously cited. There are several arguments for this terminology: variable age of patient, constant appearance and localization in contrast to the multiple forms of adenomyosis. For Naftalin However, at histological analysis, all those formations present the same pattern. This is a cavity lined by an endometrial epithelium. The stroma below the epithelium is thin throughout the cyst and contains red blood cells and hemosiderin-laden macrophages. The stromal cells are morphologically similar to those of the endometrium, as in endometriosis. It is surrounded by a thick myofibroblastic wall corresponding to the reaction of the myometrium to menstrual bleeding as seen on imaging findings.Although the first-line imaging is endovaginal ultrasound, taken full advantage using 3 -D modality which shows an intra-myometrial rounded cystic image, with sharp edges and finely echogenic fluid content, its appearance is more characteristic on MRI. The advantage of MRI is to provide a precise localization and morphological aspect, a tissue characterization of the lesion as well as an evaluation of the overall pelvic cavity. There is no need for any contrast injection. An hypersignal on T1 and T2-weighted sequences reflects a hemorrhagic content and hypointensity of the surrounded tissue on T2-weighted is the typical aspect of the fibrous tissue. A liquid-liquid level can be observed within the cyst, corresponding to a bleeding content of different ages.In agreement with the recent paper of Peyron In addition, MRI provides some arguments for the differential diagnosis. A congenital anomaly with hematometry within a non-communicating uterine horn will have an oval or tubular configuration without any surrounded fibrous layer. The necrobiosis of a uterine myoma will have a different topography, also containing a T2-weighted hypersignal in but a thinner hypointense wall on T2-weighted sequences without enhancement, at the difference of ACUM if an injection is performed , 14.Several types of invasive management are possible, without consensus; since medical treatment does not work in the long term. Surgical adenomyomectomy has the disadvantage of surgical risk and weakening of the uterus. Interventional radiology techniques using cryotherapy or focused ultrasound are under evaluation.In conclusion,This uncommon uterine abnormality called ACUM has a so typical appearance on MRI, allowing the right diagnosis as well as a complete evaluation of the pelvic cavity. This feature must be a part of the knowledge of the radiologists.of Two Cases of Accessory Cavitated Uterine Mass (ACUM): diagnostic challenge for MRI I confirm that our 2 patients have been informed that their case has been proposed for publication in a scientific imaging journal. I confirm that written, informed consent for publication of their case was obtained from the patients. Our local Institutional Review Board, was orally informed of this work and had approved these data.Case report


Pediatric optic neuritis (PON) may be a clinically isolated and self-limiting event or may present in the context of underlying neurologic, infective, or systemic disease. PON has a high impact on the quality of life as it may or may not evolve into other acquired demyelinating syndromes (ADSs), such as multiple sclerosis (MS), neuromyelitis optica (NMO), or other syndromes related to the myelin oligodendrocyte glycoprotein IgG antibodies (MOG-IgG). These different PON phenotypes present variable clinical and radiological features, plasma and liquor biomarkers, and prognosis. We describe four pediatric cases presenting clinically with ON, with different etiopathogenetic pictures: one case had a probable infective etiology, while the others were associated with different demyelinating disorders . We discuss the possible evolution of presenting ON in other ADSs, based on recent literature. A careful evaluation of the clinical and investigation findings and the natural course of PON is necessary to define its pathogenic pathway and evolution. Further prolonged follow-up studies are needed to highlight the predictors of PON evolution, its potential sequelae, and the best treatment options. Pediatric optic neuritis (PON) is the inflammation of the optic nerve, manifesting clinically with acute or subacute visual loss; periorbital pain, dyschromatopsia, and visual field defects frequently co-occur ,2,3.The incidence rate is 0.15–0.57 per 100,000 person-years, less than that of adult-onset optic neuritis (AON) estimated at approximately 1–2 individuals per 100,000 per year . The majThe optic nerve inflammation may have multiple etiologies  ,5,6,7,8.The different phenotypes have variable prognostic features, ranging from the complete absence of sequelae to visual dysfunction and disability in general.To date, no prospective treatment trials have been performed for PON, so that adult outcomes from the Optic Neuritis Treatment Trial guide the clinical practice . CorticoWe describe four cases of PON undertaken by our child neuropsychiatry unit, with different phenotypes, underlying pathologic mechanisms, and evolution. We aimed to provide new information about PON, a rare condition with a relative scarcity of studies, to better understand its heterogeneity in the clinical and investigation findings and prognostic features.◯Neurological examination;◯Ophthalmological examination, including visual acuity assessment and fundus oculi examination;◯Serological examination: laboratory routine blood tests, infectious disease tests, autoantibodies including MOG-IgG and AQP4-IgG;◯Cerebrospinal fluid (CSF) examination;◯Visual evoked potentials (VEPs);◯Computerized visual field (CVF);◯Optical coherence tomography (OCT);◯Magnetic resonance imaging (MRI).We studied four cases of PON. The diagnostic workup performed on the four patients, after written informed consent obtained from their parents, consisted of the following:The results of the diagnostic workup are summarized in The first case is a 17-year-old girl with a family history of MS, hemicrania, and general anxiety disorder, without perinatal risk factors. At age 16, she presented with hypophagia, significant weight loss, asthenia, paraesthesia of both hands, sleep–wake cycle anomalies, and amenorrhea. These symptoms were followed, after a few weeks, by an acute onset of blurry vision in the left eye, scotomas, associated with supraorbital pain exacerbated by eye movements, progressively worsening within hours. She was diagnosed with unilateral, postinfective ON, likely secondary to Epstein–Barr virus (EBV) infection. MRI scans are shown in . She was diagnosed with ADEM followed by ON (ADEM-ON), MOG-IgG seropositive. The patient was treated with intravenous methylprednisolone, with a gradual, partial recovery of visual acuity. In the following three years, she presented two more generalized critical episodes. Antiepileptic therapy was proposed, but the family refused.The second case is an 11-year-old female with a family history of hematologic and neoplastic conditions, without perinatal risk factors. At age 3, she presented with an episode of acute disseminated encephalomyelitis (ADEM). Plasmapheresis and steroids were prompted, with a full recovery. At age 7, she showed an acute onset of periorbital pain, blurriness, and loss of visual acuity in the right eye. One month before the ON, she presented a generalized critical episode. MRI scans showed the inflammation of the right optic nerve with neuroradiological outcomes of the previous ADEM . She wasThe third case is a 17-year-old girl with a family history of intellectual disability and headache. She was born from twin birth, with an occurrence of threatened miscarriage in the first trimester. At age 7, she was diagnosed with a learning disorder and separation anxiety disorder. At age 17, she suffered from an acute visual loss in the right eye, blurred vision, supraorbital pain, and dyschromatopsia. One month before, she presented episodes of frontal constrictive headache, with cold sweating and urinary urgency, following prolonged sun exposure. She was diagnosed with MS because the acute unilateral ON was associated with MRI signs of spatial and temporal dissemination . The patThe fourth case is a 10-year-old female with a family history of factor V Leiden mutation, psoriasis, and Raynaud disease, without perinatal risk factors. At age 8, she presented with a severe loss of visual acuity in both eyes, preceded by head trauma. She was diagnosed with NMO, AQP4-IgG seropositive. The optic nerve inflammation was bilateral but more pronounced in the left side, where MRI signs of ON lasted for more than one month after the onset. The spinal cord was free from demyelinating lesions . She wasWe discuss four cases referring to the current literature on PON, evaluating risk factors for the development of other ADSs, such as MS and NMO, considering the crucial role of MOG-IgG and AQP4-IgG.The cases are all female, and two of them are in postpubertal age, in accordance with the epidemiologic data of the literature ,5.ON is frequently limited to an isolated and self-limiting event, although the diagnosis of a single isolated optic neuritis (SION) cannot be made if not after a period of follow-up, making the definition of its prevalence more difficult . IsolateON may not be isolated and may evolve into other ADSs .In our sample, case 2 presented ADEM-like early-onset ADS, with a multiphasic course (relapse with ON) and high titer seropositivity for MOG-IgG, diagnosable as ADEM-ON, a relatively new nosologic entity with monophasic or recurrent ON following a previous ADEM . The detON represents the onset symptom of pediatric MS in 20–25% of cases ,4,18. ThON is a symptom of onset in more than half of NMO cases: it is often bilateral and associated with positive AQP4-IgG, which are highly specific for NMO and are now considered a major diagnostic criterion . A groupAll cases presented clinically with the typical ON signs and symptoms, but the evolution and prognosis were different. While the etiopathogenesis of PON seems clearer for cases 1, 3, and 4, for case 2 it remains uncertain. In particular, at the moment, patient 2 can be included in the spectrum of multiphasic clinical syndromes related to MOG-IgG, considered by some authors as a distinct clinical entity, on the border between MS and NMO ,10,35. IThe recent literature helped to increase the knowledge on the evolution of PON, a rare condition with a relative scarcity of studies and without well-defined prognosis and standardized follow-up paths. This study could provide new information about PON, its heterogeneity in the clinical and investigation findings, the risk factors for other ADSs, and prognosis.Many different conditions can present clinically with ON, but their etiopathogenetic processes, response to treatment, visual outcome, and relapsing risk may vary substantially. The analysis of the sample examined in this study gives information that may help to understand the high heterogeneity of PON and the necessary evaluation of its natural course to define its pathogenic pathway and evolution. Further prolonged follow-up studies are needed to highlight the predictors of PON evolution, its potential sequelae, and the best treatment options.


Atypical choroid plexus papilloma is a rare pediatric brain tumor that has distinct clinical and pathologic features. In this case, we highlight the diagnosis and management of this rare disease. The details of case positioning and execution are discussed. The case review is utilized as an overview of histopathologic findings, to discuss clinical features of the disease, and to highlight areas warranting further investigation. In particular, we provide insight into the typical clinical course post-treatment. Choroid plexus tumors (CPTs) are rare tumors of neuroectodermal origin, accounting for only 0.4–0.6% of all pediatric intracranial neoplasms. While CA 2-year-old previously healthy male presented with two months of progressively worsening non-bilious, non-bloody emesis. The episodes occurred daily in the morning and after meals and were associated with increased fussiness, gait abnormalities, anorexia, and a five-pound weight loss. He was first evaluated by a pediatric gastroenterologist. Due to concerns about his associated behavioral changes and the positional nature of his symptoms, an MRI was ordered. Shortly thereafter, the patient then presented to the ED for intractable vomiting. The ordered MRI was completed at that time which revealed a large fourth ventricular tumor with obstructive hydrocephalus, most concerning for a medulloblastoma . NeurosuThe patient was positioned prone on a Mayfield gel horseshoe. A linear suboccipital incision was made from the inion to the spinous process of C2 and carried deep using Bovie electrocautery down to the suboccipital skull and the posterior ring of C1 until there was sufficient visualization to perform the bony opening. A pair of burr holes was made and a craniotome bit was used to perform the lateral craniotomy down to the foramen magnum and across the bottom of the skull. This ultimately created a suboccipital boneflap that was then removed. A C1 laminectomy was performed to complete the bony opening and dura was incised in a standard Y-shaped fashion. Operating microscope was used for the entire tumor resection. The tumor was hollowed out using a Sonopet on 40/50/8 and the adherent tumor capsule was carefully dissected from the cerebellar tonsils, vermis, and floor of the fourth ventricle. Several specimens were taken for pathologic evaluation. Progress of debulking was monitored using the Aloka ultrasound. The cerebral aqueduct was uncovered which restored normal CSF flow. Despite focally poor planes with regards to the brainstem and areas of the lateral walls of the fourth ventricle, a gross total resection was achieved with gradual and careful dissection. Excellent hemostasis was achieved, cavity was irrigated, dura was closed, bone flap was replaced, and the wound was closed in layers. The skin was closed with a running 5–0 Monocryl.The patient remained intubated post-operatively. No EVD or drains were required. He was sedated for post-operative MRI which revealed improved hydrocephalus and some enhancement adjacent to the resection cavity likely related to postsurgical reactive changes rather than residual tumor. He was discharged without difficulty 3 days post-surgery.On gross examination, the tumor appeared as a large, soft, friable, pink mass. Microscopic examination revealed a neoplasm with predominantly papillary  and focaThree- and six-month follow-up imaging revealed no new lesions, but there was a small area of slightly enlarging enhancement noted at the fourth ventricular roof representing scar tissue versus residual tumor which is being followed closely with serial imaging . ClinicaHistorically, choroid plexus tumors (CPT) have been classified by the World Health Organization (WHO) as either being benign or malignant based on certain histological criteria. Choroid plexus carcinoma (CPC) has high mitotic activity, high degree of cellularity, necrosis, and often invasion into the brain parenchyma while the benign choroid plexus papilloma (CPP) lacks such frank features of malignancy. Differentiating between CPC and CPP has proven challenging in some cases which prompted the creation of a third category which represents a small subset of CPT—atypical choroid plexus papilloma.This case is unique as it represents a rare subtype of an already rare tumor in a location that is uncommon in the pediatric patient population. Despite the radiographic evidence favoring medulloblastoma, a malignant tumor with a guarded prognosis, pathology confirmed that this patient actually had a much more benign, albeit rare, diagnosis of an atypical choroid plexus papilloma. Although CPT are typically supratentorial in children, our patient’s tumor was located infratentorial within the fourth ventricle. Pathologic hallmarks of choroid plexus papillomas include papillary fronds lined by bland columnar epithelium; positive staining for cytokeratin, transthyretin, and GFAP; and absence of nuclear pleomorphism and necrosis. These were all features displayed in our patient’s tumor specimens. Choroid plexus papillomas with greater than two but fewer than five mitotic figures per high power field are characterized as atypical, as was seen in our patient. Further research is warranted in regards to follow up chemotherapy and surveillance needed for these patients.


Arteriovenous malformations are congenital vascular malformations with a high flow rate. They are made up of a complex vessel system that forms a nidus by connecting feeding arteries to draining veins. Arteriovenous malformations can be fatal due to progressive symptoms and infiltrative disease. The head and neck are the most affected areas by extracranial arteriovenous malformations, followed by the limbs. Hormonal changes during pregnancy lead to the expansion of arteriovenous malformations, which may lead to aggressive progression. We present a case of a patient who had a very rare presentation of ruptured forehead arteriovenous malformations during her fifth pregnancy. A combination of radiological embolization followed by surgical excision was used to treat the condition. In plastic surgery and interventional radiology, aortic and venous abnormalities of the face are among the most difficult to treat. The treatment of these recurrent lesions necessitates a multidisciplinary approach. After a successful surgical resection and selective embolization, the reconstructive surgeon faces a Herculean task: the restoration of a facial contour that has been severely deformed due to the disruption of several clearly defined aesthetic units [th week of gestation, which was surgically excised.Vascular anomalies are disorders of the endothelium and surrounding cells that can affect the vasculature and involve any anatomical structure. The most common problem associated with vascular anomalies is psychological distress related to the disfigurement as well as functional defects because many lesions affect the head and neck . Local cic units . This arA 35-year-old Malay woman with a history of AVMs since childhood presented to the emergency department with a history of forehead bleeding. The AVMs had been present since childhood and had been growing since the age of 21. During her previous two pregnancies, the lesion expanded in size and was associated with skin abnormalities, but it shrank postpartum. The lesion was rapidly expanding with obvious skin changes over AVMs. On the 26th week of gestation, the malformations suddenly ruptured without a history of preceding trauma and profusely bled. Manual compression was done to halt the bleeding. However, it was not effective.Physical examination revealed a hyper-pigmented, raised lesion around 5x2cm, smooth surface, and regular margin on the forehead as shown in Figure Following a multidisciplinary team meeting, we determined that angioembolization before the excision biopsy would have been beneficial to the patient. Angio-embolization was performed, which revealed that the forehead AVMs are mostly supplied by the terminal branches of the left maxillary artery as shown in Figure Surgical excision was performed, and the wound was managed to be close primarily without tension. No complications were observed postoperatively, and the patient was discharged a few days later. On clinic follow-up, the scar appears flat and pink, and there are no signs of residual, as shown in Figure Feeding arteries, fistulae, or nidus, and draining veins make up AVMs. The nature of connectivity between arteries and veins has an impact on the embolic approach used and the treatment success. Over time, AVMs usually grow more symptomatic . The evoAVMs are twenty-fold more common in the intracerebral vasculature than in extra-cerebral sites. The head and neck are the most prevalent sites for extracranial AVMs, followed by the limbs, trunk, and viscera . AVMs inThe treatment of arteriovenous malformations can be risky, and the results can be poor. Selective embolization, surgical excision, or mixes of both are used as therapeutic strategies. Preoperative radiographic preparation is important as it is used to confirm the clinical impression, evaluate the extent of vascular malformation before treatment, identify associated malformations, and differentiate atypical masses. The imaging features of a lesion vary based on its clinical stage and location. Most AVMs are identified by the presence of dilated feeding and draining veins within normal or hypertrophied tissue by utilizing ultrasound, CT, and MRI . The repEmbolism aims to close the smallest of vessels from the inside out by injecting embolic material into the malformation's epicenter. However, selective embolization with occlusion of one feeding vessel causes immediately increased flow from other feeding vessels. To permanently collapse the major arteriovenous malformations, all micro-fistulas and macro-fistulas must be eliminated. Preoperative embolization is indicated to reduce blood flow and facilitate surgical excision but should not be used to reduce the extent of resection . The onlThe goal of surgery is total removal of the nidus of the AVMs as leaving behind residual or dormant anomalous channels may lead to further collateral formation, shunting, and expansion. As the overlying skin was involved, it was excised. Before resection, options for closure were considered. As the lesion was small and well-localized, a primary closure could be achieved. Another form of primary closure is either a skin graft or a local or microvascular flap. Delayed primary closure with a skin graft is particularly useful if bleeding is excessive, provided that vital structures are not exposed. Abnormal collateral vessels may be present after primary or delayed covering with a local cutaneous or musculocutaneous flap. If the AVMs are not completely removed, the abnormality will recur and may entangle the skin graft or flap. Hurwitz and Kerber in 1981 proposed that a microvascular free flap would improve the local hemodynamic state at the site of incompletely resected AVMs and thus minimize the likelihood of dilatation, collateral formation, and reappearance . Post-opPregnancy tends to accelerate the nature of AVMs' progression, which can lead to undesirable complications, especially in the head and neck region. AVMs that rupture during pregnancy can be treated similarly to those that occur in non-gravid women. Embolization followed by surgical excision offers a higher cure rate and reduces complications intra-operatively.


Abdominal tumor masses are a very rare disease in the fetus. The authors present the first reported case of neonatal multicystic adenomatoid hamartoma of the pancreas associated with well-differentiated fetal epithelial subtype hepatoblastoma and reveal clinical, histologic, and imagistic aspects. Case presentation: A 36-week-old female newborn in whom a 25-week ultrasound showed a relatively homogeneous pancreatic echogenic mass (34 × 30 × 55 mm) with compression of the inferior vena cava and retrograde dilation. Postnatal CT showed a giant pancreatic tumor mass (113 × 70 × 60 mm), with areas enhancing contrast and cystic/necrotic areas and a hypodense, hypocaptive nodule of 8 × 6 mm located at segment IV of the liver; thrombosis of the subhepatic segment of the inferior vena cava and both renal veins. Histopathological and immunohistochemical studies confirmed the diagnosis of multicystic pancreatic adenomatoid hamartoma and well-differentiated fetal epithelial subtype hepatoblastoma. Conclusions: Pancreatic hamartoma can be difficult to diagnose , with or without nonspecific symptoms. The synchronous presence of hepatoblastoma complicated the therapeutic conduct and prognosis of this case, with the diagnosis being confirmed histopathologically and immunohistochemically after liver biopsy. Fetal intra-abdominal complex tumors are extremely rare and are benign in most cases. Likewise, tumors of the fetal pancreas are extremely rare and mainly represent congenital islet cell adenoma . The autThe purpose of this case report was to show the rare occurrence of synchronous pancreatic hamartoma associated with hepatic hepatoblastoma and to report the associated imaging and infant outcome.We present the case of a premature female newborn at 36 weeks from a 32-year-old mother with two previous births. The pregnancy had a normal course until the 25th week, when an ultrasound scan revealed a pancreatic echogenic mass (34 × 30 × 55 mm), relatively homogeneous, with compressed inferior vena cava (IVC), normal amniotic fluid, lungs, liver, and kidneys a–d. PretThe patient had been admitted to the pediatric surgery department, Emergency Children Hospital “Grigore Alexandrescu” Bucharest, Romania, where the following exams were performed .The laparotomy was performed at 1 week old. Intraoperative findings were as follows: giant tumor mass on the anterior face/inferior border of the pancreas, 15 × 10 cm, polylobate, firm, with cystic areas, strongly adherent to the mesentery, duodenum, colon, ileal loops on which has a mass effect; nodular mass in the Vth segment of the liver, under the hepatic capsule, size < 1 cm, firm consistency suggestive for metastatic nodule; mesentery tumor mass, 1 × 1 cm, with a consistency similar to the pancreatic tumor .The surgery consisted of partial resection of the pancreatic tumor and complete resection of the mesenteric tumor. Dissection of the tumor was very difficult due to the intratumoral trajectory of the superior and inferior mesenteric vessels and the intimate contact with the inferior vena cava but without invading it; also, the tumor penetrated the retroperitoneal space without invading the kidneys and ureters. Subtotal resection was carried out with effraction of the tumor capsule, and a small area of the pancreas was preserved. The recovery after surgery was complicated; the patient was intubated, vasopressor treatment and insulin therapy were required. On the 12th day after the operation, the increase in the size of the abdomen required intraperitoneal drainage with the evacuation of chylous ascites.The initial surgical histopathological examination revealed a low-malignancy serous cystadenocarcinoma .2) with a total dose of 30 mg and 5-FU 400 mg/m2, then 1200 mg/m2 . After reevaluation of the histopathological stains, the final diagnosis showed that the resected tumor was, in fact, a pancreatic mixed hamartoma (solid and cystic), a benign tumor that does not require chemotherapy.The subsequent monitoring of the patient was performed in the Pediatric Oncology Department of the Oncological Institute «Prof. Dr. Al. Trestioreanu». Clinical examination at admission: afebrile, acceptable general condition, pallor, abdominal distension, peritoneal drainage approx. 20–30 mL/24 h, breast and bottle feeding, weight = 2700 g; laboratory findings: LDH = 2×, GGT = 4×. At the age of 2 months, the patient received a round of chemotherapy (FOLFIRINOX protocol), because the initial pathological examination was serous cystadenocarcinoma, and he received Leucovorin  a,b.The complex tumor masses encountered in the newborn continue to be a real challenge from a clinical, diagnostic, therapeutic, and prognostic point of view. This is due to the low number of cases reported in the literature. The peculiarity of this case was determined both by the synchronous occurrence of multicystic adenomatoid pancreatic hamartoma and hepatoblastoma in a 1-week-old newborn.Pancreatic hamartomas are extremely rare tumors in children and even more so in newborns. A systematic search of the literature identified a total of forty-five cases of pancreatic hamartomas, of which thirty-six were in adults, six in children, and three in newborns ,10. Two In prenatal life, abdominal tumors of the fetus are rare and sometimes difficult to diagnose. Although currently, there is no detailed and unanimously accepted classification of fetal tumors, the antenatal diagnosis of intra-abdominal tumors regarding their origin is based on the interpretation of ultrasound and MRI images, and postnatally on the histopathological evaluation of the surgical specimens . In newbThe rate of antenatal diagnosis of fetal intra-abdominal tumors is unknown due to the low number of cases. The use of ultrasound screening during pregnancy allowed the detection of intra-abdominal tumor masses . The ultPrenatal ultrasound diagnosis of congenital abdominal tumors is difficult to achieve depending on the tumor’s gestational age, origin, and characteristics. The use of 3D/4D reconstruction techniques, TUI Doppler, helps diagnose these tumor masses and fetal MRI, further contributing to establishing the relationship of the tumor with neighboring organs. The evaluation of the association of these congenital anomalies includes fetal MRI to increase the accuracy of the diagnosis, make a differential diagnosis, highlight the location, and establish the tumor morphology and the relationship with the adjacent structures, allowing the correct preoperative assessment and sometimes the prognosis of these tumors . ComputePrenatal management of the fetus requires a detailed ultrasound evaluation to detect any associated malformations and sometimes genetic evaluation . Of the Neonatal hamartomas are generally rare, with a higher kidney incidence. However, cases involving the liver, lungs and heart, brain, skeletal system, and gastrointestinal tract have been described in the literature. For example, a study by Kamil et al. showed on a batch of 84 tumors that only 19% had an intra-abdominal origin, with 15.4% solid tumors . On the Prenatally, monitoring of fetal condition, tumor growth rate, and compression of adjacent organs are elements that usually do not significantly influence the decision and the method of birth in the fetus’s interest. A rapid increase in abdominal circumference secondary to intra-abdominal tumor volume, Doppler changes, or prenatal detection of tumor formation increase the rate of elective cesarean section . PostnatThe synchronous presence of pancreatic and liver tumors in the newborn is unique and without a similar correspondence in the literature. The histopathological results raised many initial elements of confusion, which also influenced therapeutic management. According to the case definitions, the histopathological examination of specimens and the immunohistochemistry analysis diagnosed pancreatic hamartoma and hepatoblastoma.In the case of hamartomas, the changes can be observed either mesenchymally or epithelially secondary to stromal induction, part of the proliferative lesion. As a result, cytogenetic or molecular abnormalities may explain the possible appearance of malignant neoplasms .A study has shown a risk factor for hepatoblastoma associated with very low preterm birth (VLBW) . HepatobMost patients are asymptomatic as in our case; others may have abdominal discomfort, digestive disorders, and weight loss. The differential diagnosis of fetal intra-abdominal tumors is made in the order of their frequency with teratomas , with polycystic kidney disease and adrenal (neuroblastoma), liver, pancreatic, gastrointestinal tract, and splenic tumors ,23,32.Rare tumors are usually misdiagnosed or late-diagnosed, the diagnosis being indirectly supported by the effect of the tumor on adjacent organs . In our In Imaging diagnosis of pancreatic tumors is important because the treatment options and prognosis are different. The surgical procedure is aimed at symptomatic pancreatic hamartomas and neuroendocrine tumors. Fetal abdominal tumors benefit from fetal intervention and access to neonatal surgery after birth. The presence of circulatory changes in the IVC is secondary to the progressive compression caused by the tumor with the progression of the tumor mass.Three patients underwent a pancreaticoduodenectomy (PD)—Whipple operation, three a local resection, and one excision of the tumor with partial duodenectomy. Patients with pancreatic hamartoma did not undergo any type of adjuvant chemotherapy. In our case, the patient was initially diagnosed with serous cystadenocarcinoma and received at 2 months old a round of chemotherapy. No data have been reported on the recurrence or metastasis of pancreatic hamartomas.The association of pancreatic hamartoma with hepatoblastoma and extensive thrombosis of the IVC and renal veins has led to poor survival.Antenatal evaluation is important to determine the time of birth and subsequent therapeutic conduct. Therefore, managing these tumors involves a multidisciplinary team of obstetricians, neonatologists, oncologists, surgeons, and pathologists.This study shows the importance of early imaging diagnosis of tumor masses in the pancreas and liver in the fetal and neonatal period. The histological and immunohistochemical evaluation is the one that confirms the final diagnosis, with therapeutic management individualized through adequate prenatal counseling and multidisciplinary involvement. The synchronous presence of pancreatic hamartoma and hepatoblastoma was a feature of this case.


Fish bone is one of the most common accidently ingested foreign bodies. Normally, it is eliminated from the gastrointestinal (GI) system without any symptomatology, only 1% of the cases will develop a perforation of the GI tract requiring surgical intervention.A 70-year-old man, presented with a 48h evolving abdominal pain, important abdominal distension, nausea, vomiting, and a last bowel movement reported 2 days ago, The abdomino-pelvic CT-scan objectified a distension of the terminal ileum measured at 30mm, The exploration revealed a sharp foreign body,at the 15 proximal centimeters of the terminal ileum, which penetrated through the wall of the ileum. The foreign body was removed and we noticed that it is a fish bone. The patient recovered well.Clinical manifestations are determined by the location of the perforation and the preoperative diagnosis is always difficult to reach. Computed tomography (CT) scan is the indicated method to identify ingested foreign bodies and surgery is the treatment of choice.Delay in diagnosis and treatment can be associated with significant morbidity and mortality. •Perforations of the gastrointestinal (GI) tract due to an ingested fish bone (FB) are rare.•Fish bone perforation of the GI is rarely diagnosed preoperatively and has a wide spectrum of clinical presentations.•Appropriate imagery techniques and a complete interrogatory will lead to the correct diagnosis.•Delay in diagnosis and treatment can be associated with significant morbidity and mortality. PerforaThis work has been reported in line with the SCARE criteria .2A 70-year-old man, without any particular pathological history, referred by family physician, to emergency with a 48h evolving abdominal pain, important abdominal distension, nausea, vomiting, and a last bowel movement reported 2 days ago. On examination, his vital signs were: temperature: 37.3 °C, pulse rate: 110/minute, respiratory rate: 24/minute and blood pressure: 110/60 mmHg. The abdominal examination revealed an abdominal sensibility at the right illiac fossa, a distension of the abdomen and an empty rectal ampula on rectal examination. The biological checkup showed hyperleukocytosis at 15700/mm3, hemoglobin at 11g/dl, platelets count at 159,000/mm3 and C-reactive protein at 180 mg/liter, the hepatic and renal check-ups were normal. An abdominal X-ray revealed the presence of hydro-aeric levels at the ileum segments without pneumoperitoneum. The abdomino-pelvic CT-scan objectified a distension of the terminal ileum measured at 30mm, upstream of a transitional level in which seats a rectangular and plane foreign body , Fig. 2.3Foreign body ingestion is a common problem worldwide with an estimated incidence of 120 per million, it is responsible of almost 1500 deaths per year, toddlers are most frequently affected, although rare in conscious and mostly accidentaI, it is fairly a common problem in psychiatric patients . 10–20% The intestine has a remarkable capacity to protect itself against perforation, when the intestinal mucosa is pricked with sharply pointed objects, the bowel wall increases the lumen of the bowel at the point of contact, permitting freer progress to the offending object, the flow of the intestinal contents and the relaxation of the bowel wall tend to make the head lead and the sharp end trail behind . Areas wFish bone perforation of the GI is rarely diagnosed preoperatively and has a wide spectrum of clinical presentations that varies from unseen passage per rectum to severe peritonitis with many clinical manifestations. Depending on the location of the damage, different symptoms can be seen: abdominal pain, vomiting, fever and occasionally melena and bowel obstruction . DifferePlain film radiography plays an insignificant role in the detection of fish bones,the sensitivity of detection in the aerodigestive tract is as low as 32%, false negatives are seen in up to 47% of cases , sensitiThe management of an ingested foreign body depends on the patient symptoms and the type and the location of the ingested object . Surgery4Perforation of intestinal structures by fish bone is a challenging diagnosis that should always be recalled in cases of acute abdominal symptoms. Appropriate imagery techniques and a complete interrogatory will lead to the correct diagnosis. Delay in diagnosis and treatment can be associated with significant morbidity and mortality.Not commissioned, externally peer reviewed.None.I declare on my honor that the ethical approval has been exempted by my establishment.Written informed consent for publication of their clinical details and/or clinical images was obtained from the patient.Abdelilah Elbakouri: writing the paper.Karim Yaqine: Corresponding author writing the paper.Mounir Bouali: study concept.Khalid Elhattabi: correction of the paper.Fatimazahra Bensardi: correction of the paper.Abdelaziz Fadil: correction of the paper.Not applicable.DOCTEUR YAQINE KARIM.The authors declare having no conflicts of interest for this article.


Pericecal hernia, a subtype of internal hernia, is a rare and unusual cause of small-bowel obstruction (SBO). We report the case of a pericecal hernia in an 80-year-old woman who presented to the emergency department with symptoms of SBO. She experienced colicky diffuse abdominal pain associated with vomiting and obstipation for over five days. Abdominal examination showed rebound tenderness in the right iliac fossa without any mass palpation.Computed tomography noted a “sac-like” structure in the right iliac fossa with dilated and ischemic small-bowel loops within. Exploratory laparotomy showed strangulated pericecal hernia and non-viable small bowel loops in the inferior ileocecal recess. Extensive resection and defect repair followed by small-bowel end-to-end primary anastomosis was made. After two days of ICU admission, patient died from septic shock.This case report highlights the need for prompt diagnosis, followed by surgical intervention to lower mortality of SBO of internal hernia origin, especially in cases where no previous surgery is noted. Internal hernias are protrusions of viscera, generally small bowel loops, through mesenteric or peritoneal defects. Cases of small bowel obstruction (SBO) due to internal hernia, and specifically pericecal hernia, are rare, with mortality of up to 50% reported in complicated cases . It reprWe report the case of an 80-year-old woman, with a known history of type II diabetes, who presented to the Emergency Department for colicky diffuse abdominal pain, associated with vomiting and obstipation for over five days. No previous surgery was noted in her past medical record. She reported no previous tobacco or alcohol use. On examination, patient was dehydrated, Glasgow coma scale score of 15/15, temperature of 37,8°C, respiratory rate of 26 breaths per minute, blood pressure of 108/78 and a pulse rate of 110 bpm. On abdominal examination, no previous surgical scar was noted on inspection, and palpation revealed right lower quadrant and hypogastric rebound tenderness without any palpable mass. Laboratory exams showed elevated white blood cell amount of 18000/mm3 and elevated C-reactive protein of 57mg/l. Abdominal CT scan showed a “sac-like” structure in right iliac fossa measuring 148x64mm, with herniated, distended and ischemic small-bowel loops within. Ascending colon was seemingly pushed upward by the herniated content  are defined as protrusions of viscera through mesenteric or peritoneal defects. IH are mainly asymptomatic with an estimated incidence of up to 2%. Almost 5.8% of SBO are thought to be due to internal hernia; pericecal hernia may cause SBO in rare cases and form about 13% of all internal hernia . PathogeStrangulated pericecal hernia is a rare and challenging condition, with high mortality rate. Our case report highlights the necessity of a prompt diagnosis. Immediate intervention should be carried out to avoid necrosis of the herniated content, once the diagnosis is suspected on clinical and radiological evaluation.


Sactosalpinx means a collection of fluid  in the fallopian tube. CAH  is a typical 46XX DSD (Disorder of Sex Development) due to a steroidogenic enzymatic defect. Both conditions are rare and can lead to reduced fertility rate.We describe two post-menarche virgin girls with CAH who were hospitalized for acute abdomen due to laparoscopically confirmed sactosalpinx. Case 1 recovered after conservative management, case 2 after a second-look and bilateral salpingectomy. The first case consisted of right sactosalpinx and previous peritonitis reported; the second one of bilateral symptomatic pyosalpinx and previous vaginal stenosis. Recurrent abdominal pain persisted at follow-up in Case 1: post-operative MRI (Magnetic Resonance Imaging) showed bilateral hydrosapinx that disappeared at a following ultrasound scan control. Follow-up was uneventful 36 months after surgery in Case 2, except for the surgical revision of the vaginal introitus.CAH-sactosalpinx association is a very rare but not negligible event. We suggest a conservative approach for sactosalpinx if tubal and/or ovary torsion can be excluded. Pyosalpinx is more challenging to treat, but during pediatric age we suggest starting with a conservative approach, especially in patients with CAH who have a potential low fertility rate. Careful gynecological follow-up after menarche is recommended to rule out any further causes of infertility. Congenital Adrenal Hyperplasia (CAH) and sactosalpinx, are two rare conditions that adversely affect future fertility in girls.CAH is a typical 46XX Disorder of Sex Development (DSD) due to a steroidogenic enzymatic defect. The most common form is the classic CAH due to 21-hydroxylase deficiency: an excessive adrenal androgen biosynthesis results in masculinization of the genitalia in XX offspring [17-OH-hydroxyprogesterone serum increase and genital inspection lead to diagnosis.Prenatal androgen stimulation is responsible for the presence of UGS  and clitoris hypertrophy at birth in the most of female fetus. In 46 XX newborn, genital ambiguity at birth varies from near female-like anatomy to near male-like (stage 1 to 5 according to Prader score) .Medical treatment involves the administration of steroid drugs associated with mineralocorticoids or not.Surgical treatment involves female genitoplasty (FG) consisting of UGS correction by total or partial mobilization –5 or by Both pelvic surgery and vaginal stenosis and are risk factors for sactosalpinx.We describe two cases of sexually inactive post-menarche girls with CAH and symptomatic sactosalpinx.Both cases needed diagnostic laparoscopy for diagnosis confirmation.watch and wait” approach for uncomplicated cases: patients and parents/guardians must be adequately informed about the disease, pros and cons and potential risks of conservative or surgical treatment.We discuss the “Girls affected by CAH and sactosalpinx need a rigorous clinical and ultrasound follow-up to achieve the best chances of fertility and the lowest risk of complications.A 14-years-old virgin girl was admitted to our Emergency Room for acute abdomen symptoms . Patient history was significant for diagnosis of CAH when she was 2-months-old, laparotomy and appendicectomy for complicated appendicitis when she was 8 years old, FG 1 year later. Both interventions were performed at another Hospital in Romania (no documentation available regarding the operations). Perineal examination before the onset of sactosalpinx showed: clitoromegaly, regular urethral meatus, no vaginal stenosis at vaginoscopy; no urinary incontinence, nor urinary tract infections. Menarche at the age of 12, regular menses, history of recurrent lower abdominal and ultrasonographic findings of regular ovaries and uterus. She irregularly took oral therapy with hydrocortisone  and Florinef®: 0,1 mg/day.At admission, clinical examination revealed tenderness with guarding in pelvic region especially in the right iliac fossa. Serology revealed white cell count of 18.640 μL with neutrophilia and CRP (C-Reactive Protein) of 2,8 mg/dL at admission increased up to 16.4 mg/dL. An US (Ultrasound Scan) showed a large multilocular tubular fluid collection consistent of tube-ovarian structure (diameter more than 9 × 4.7 cm) and presence of liquid film in pelvic cavity. Urgent diagnostic laparoscopy was performed, because acute abdominal pain persisted and ovary and/or tubal torsion could not be excluded. Intra-operatively, right hydrosalpinx with a gross tubular dilatation was found without adnexal torsion,  showed bilateral hydrosapinx see Fig.  that disA not sexually active 15-years-old girl was evaluated for acute abdomen and fever.Patient history was significant for diagnosis of CAH at birth, FG performed at our Institute when she was 21-months-old , surgical treatment for vaginal stenosis when she was 12-years-old and 16-years- old. Before the onset of acute symptomatology, the patient had recurrent low urinary tract infections history, no urinary incontinence. Urethral meatus was near clitoris , vaginal introitus was narrow: its surgical revision and vaginal dilatation were performed 3 year before the acute symptoms occurred. She referred menarche at the age of 14 and regular menses. Ultrasound scan was significant for regular ovaries and uterus deflected to left. The patient regularly took oral therapy with hydrocortisone  and Florinef®: 0,2 mg/day.Physical examination, ultrasound scan and blood examination at admission suggested bilateral pyosalpinx. An urgent diagnostic laparoscopy was performed at another Hospital and confirmed the diagnosis. It was decided for conservative management even in this second case. The girl started standard parenteral antibiotic therapy for pyosalpinx but symptoms persisted and bilateral salpingectomy was necessary at the second-look laparoscopy 2 weeks later. Post-operative course was regular. A second surgical operation to enlarge vaginal introitus was needed after 1 year. Follow-up was then uneventful after 3 years.Sactosalpinx is a rare cause of abdominal pain in pediatric age and the clinical picture range from asymptomatic form to acute abdomen.It occurs when a distally blocked fallopian tube fills with fluid collection of serum , blood  or pus .Occlusion of the fimbriated end of the tube may lead to tubal dilatation, usually in the ampulla and infundibulum. As far as the etiology is concerned, primary causes involve the anatomy, like abnormalities of length, mobility and structure. Secondary causes are acquired. The most common cause of hydrosalpinx is pelvic inflammatory disease, other causes include endometriosis, peri-tubal adhesion from a previous operation, tubal cancer and tubal pregnancy . These sUS and MRI guide for diagnosis. On MRI, hydrosalpinx appears as a fluid-filled C- or S-shaped tubular structure that arise from the upper lateral margin of the uterus , but oftCAH is the most common cause of 46XX-DSD due to fetal androgen exposure . It is aThe most challenging part of this intervention is the separation of the vagina from the urethra, especially in cases with a high confluence (UGS length more than 3 cm). Besides the technical difficulty, the patient is at risk for post-operative urinary incontinence, urethra-vaginal fistula and vaginal or urethral stenosis [en bloc mobilization of the urethrovaginal confluence, with repositioning of the urethral and vaginal orifices in a separate orthotopic location.To facilitate reconstruction and decrease the risk of complications, partial UGS mobilization was described , 5. RinkThe goal of surgical reconstruction for girls with CAH and UGS are recreating normal appearing and functional external genitalia, preserving bladder function, and maximizing the potential for a normal sexual and reproductive adult life. This goal may be technically harder to achieve in children with a high urethra-vaginal confluence and higher degrees of virilization.CAH and sactosalpinx are two rare in pediatric age and they both lead to reduced fertility rate.In 2011 Boukaidi et al.  reportedBoth our cases had predisposing factors to sactosalpinx: Case 1 had peritoneal adhesions occluding the fallopian right tube due to previous surgery (laparotomy for peritonitis); Case 2 had a history of vaginal stenosis and recurrent urinary infections .We recommend a conservative approach for asymptomatic hydrosalpinx and for symptomatic sactosalphinx in general, except for septic cases or no responder patients (worsening symptoms despite adequate antibiotic treatment). If symptoms are controlled by analgesic/antibiotic therapy and adnexal torsion or other associated conditions (neoplasm) are excluded at surgery, we encourage a conservative treatment and a strict clinical and radiological follow-up: ultrasound scan every 6–12 months until adult age and periodical genital visit under anesthesia to exclude predisposing factor like vaginal stenosis for CAH surgically treated patients.Vaginal stenosis, particularly when associated with recurrent urinary infectionsPrevious pelvic surgery or previous laparotomy/laparoscopy for peritonitis for example due to complicated appendicitis or other abdominal diseases.In our opinion, especially in cases with reduced fertility potential, it is important not to neglect predisposing conditions like:


Glandular neoplasms of the temporal-mastoid region and endolymphatic sac (ELS) are rare, and it is quite challenging to differentiate between an adenoma and an adenocarcinoma. ELS tumors (ELST) usually present with papillary, follicular, or solid patterns and can be further distinguished histologically and through immunohistochemistry. The microscopic features and clinical course of this neoplasm have been comprehensively explained by Heffner, who considered it “low-grade adenocarcinoma of likely ELS origin.” The papillary form more commonly affects females, and it is a more aggressive form of ELST that is destructive and exhibits extensive local spread. The tumor usually has a close association with von Hippel-Lindau (VHL) disease, but 11%-30% of the ELST cases develop in individuals without a VHL mutation. ELSTs manifest with headaches, hearing loss, ear discharge, and cranial nerve palsies. Currently, the only available curative therapeutic intervention consists of wide local excision and long-term follow-up. Because of the sensitive location of this tumor, the adjuvant radiotherapy options are still questionable. In this case report, the author presents a 74-year-old woman with a past medical history of Schneiderian papilloma and was diagnosed with papillary mucinous adenocarcinoma of the ELS not associated with VHL disease. The different parts of our body are derived from three different germ layers. Similarly, the membranous labyrinth and endolymphatic sac (ELS) are derived from the ectoderm and are found postero-medial to the temporal bone ,2. The eMiddle ear tumors are sub-classified as adenomas, aggressive middle ear neoplasms, papillomas, and mixed or papillary type ,7. The mELS tumors (ELSTs) are extremely uncommon, with only around 300 cases published in the literature . The purA 74-year-old female patient came in for a follow-up with an extensive past medical and surgical history. During the visit, her vital signs were as follows: blood pressure, 133/83 mmHg; heart rate, 88 beats/min; temperature, 97.5°F; respiratory rate, 16/min; and body mass index, 27.16. Her medications were the following: vitamin D, potassium, magnesium, aspirin 81 mg/day , glucosamine capsule, and prednisone 20 mg as needed.Past medical history: The patient has a past medical history of skin cancer, endometrial cancer, and Schneiderian papilloma of the right mastoid.Past surgical history: The patient had undergone a bilateral hip replacement, hysterectomy, hemorrhoidectomy, breast lump resection, tubal ligation, resection of Schneiderian papilloma (June 2018), and excision of skin cancer from the right leg (December 2019).In 2014, the patient presented with right-sided hearing loss. In 2017, her right tympanic membrane was perforated, with blood and abnormal tissue protruding from the eustachian tube orifice. Right ear tympanostomy tubes were placed, which helped. The tube eventually fell out, and the tympanic membrane healed. She had a recurrence of middle ear effusion, for which a computed tomography (CT) scan of the right temporal area was performed. The CT images with and without contrast showed complete opacification of the right middle ear cavity and mastoid air cells.Because of the opacification of the middle ear and the mastoid air cells, an audiogram was recommended to test the extent of hearing loss. The results demonstrated mild sloping profound sensorineural hearing loss of the right ear with a conductive component at 2 kHz and mild sloping profound sensorineural hearing loss of the left ear. The pure tone averages were 60 dB in the right ear and 28 dB in the left ear. The word recognition scores were 36% on the right and 92% on the left. The fiber-optic examination revealed blood draining from the right eustachian tube orifice. The magnetic resonance imaging (MRI) results showed extensive fluid throughout the mastoid air cells and the middle ear with some relative enhancement.She underwent right trans-temporal craniotomy and partial resection of the mass in January 2018. Histopathology was performed on the excised mass, which was reported as an ELST. In May 2018, a follow-up MRI revealed the recurrence of the lesion, and on physical examination, the lesion was protruding through the eardrum associated with otorrhea. In June 2018, a re-resection of the lesion was done, and this time, the eardrum, malleus, incus, and external canal bones were removed. There was a portion along the petrous bone and carotid artery where the tumor could not be removed, and the tumor extended from the middle fossa dura to the sigmoid sinus.The patient then underwent radiation therapy (60 Gy in 30 fractions). In November 2019, the patient developed right facial palsy, which was treated with two weeks of prednisone without resolution. A follow-up MRI showed a new 5-mm nodule in the right nasopharynx. There was no radiographic reoccurrence of the tumor in the right temporal bone/fossa, and there was no evidence of facial nerve involvement.In January 2020, the patient underwent a right-sided nasopharyngeal eustachian tube biopsy. The biopsy specimen was sent and processed in the pathology department, and the report showed a low-grade papillary neoplasm consistent with an ELST origin. The patient was scheduled for a follow-up MRI and CT in the next six months. The details about how the biopsy specimen was processed and the histopathological diagnostic features of ELST are discussed in the Methods and Materials sections below.Methods and materialsThe biopsy specimen was processed by fixing in formalin and embedding in paraffin before sectioning and fixing on glass slides. After the slides were processed and ready, they were stained with hematoxylin and eosin (H&E). In addition to H&E, some selected slides were also stained with periodic acid Schiff.An immunohistochemistry (IHC) study was performed using the EnVision method. The process of immunostaining implies the selective recognition of antigens (proteins) in the tissue sections of cells by utilizing the fundamental rationale of antibody binding particularly to the antigens in the biological tissue. The antibodies that were used for IHC in this case of ELST were cytokeratin 7 (CK-7), pan-keratin, cytokeratin 20 (CK-20), cytokeratin 19 (CK-19), vimentin, glial fibrillary acid protein (GAF), thyroglobulin, and Ki-67. The slides were boiled under high pressure with citrate buffer  for the purpose of antigen retrieval. The proper positive and negative controls were also performed.HistopathologyLow PowerMorphologically, at low power, the specimen showed mostly papillary and focally glandular architecture. The neoplastic proliferation of the tumor cells comprised columnar and focally cuboidal cells with round to oval nuclei that show mild to moderate pleomorphism with abundant extracytoplasmic mucin Figures , 2. As mHigh PowerOn higher magnification, a single layer of columnar to cuboidal cells lined the papillary and glandular structures. Moderate to mild pleomorphism, rare mitotic activity, and necrosis were not observed. On the other hand, multiple acinar configurations with infiltrative growth patterns and desmoplastic responses were seen Figure . Unconve ImmunochemistryAs described in the Methods and Materials sections, an IHC study was performed. CK-20, which highlights papillary growth, was negative, whereas CK-7 immunostaining was positive as evident in Figures Diagnostic modalities for an ELSTRadiographyIn this case of ELST, both CT and MRI were used as the imaging diagnostic tools. The CT scan and MRI results showed opacifications in the middle ear and the mastoid air cells in 2018, during the initial presentation of the patient. After partial resection of the lesion, the patient developed a nasopharyngeal mass along with middle ear effusion, which was diagnosed through a CT scan and MRI in January 2020.The CT scan and MRI techniques are widely used to diagnose ELST . The CT Angiography and AudiogramApart from the imaging modalities, the most sophisticated procedure to diagnose an ELST in clinical practice is angiography. Compared to an angiography, an audiogram is a noninvasive test that was performed in this case of ELST. The audiogram showed that the patient had significant hearing loss in the right ear and moderate hearing loss in the left ear because of an invading ELST. A fiber-optic examination was also performed, which showed blood draining into the eustachian tube.Angiography was not performed on this patient. However, according to previous angiography results in the literature, the tumor can have a dual blood supply from intra- and extra-cranial sources, making it extremely vascularized. A jugulotympanic paraganglioma is another entity that can mimic ELST because both of these tumors are highly vascularized and can carry potential bleeding risks .Differential Diagnosis of ELSTIntrinsic temporal bone neoplasms are included in the differential diagnosis for ELSTs, with paragangliomas being the most common. Meanwhile, metastatic papillary thyroid carcinoma, metastatic renal cell carcinoma, and choroid plexus papilloma are histologically similar to ELSTs. The ELSTs are papillary cystic structures lined with a simple cuboidal or columnar epithelium and are heavily vascularized. Clear cells, as well as siderophages and cholesterol clefts, are visible (vacuolated cells). Nuclear pleomorphism is minimal, and mitoses are uncommon ,14.StagingThere are no universally accepted staging recommendations. Bambakidis et al. suggested the system described in Table Schipper et al. defined type A tumors as those with no temporal bone destruction or dura invasion, type B as those with signs of osseous labyrinth infiltration and sensorineural hearing loss, and type C as those that have invaded the sigmoid sinus and jugular bulb ,16.The patient in this case underwent resection for a tumor that extended into the sigmoidal sinus and middle cranial fossa. If we follow the proposed staging system, then it is obvious that the tumor was an aggressive one, and the patient presented as stage III at the time of treatment.TreatmentComplete resection with negative margins is the treatment of choice for ELST, but in advanced cases, this may not be possible. As the patient presented as ELST stage III, the only universally accepted treatment of choice is complete resection of the neoplastic tissue, which is recommended. Because of the complexity and advanced stage of the disease, the tumor was only partially resected. Under general anesthesia, she underwent a trans-temporal craniotomy and a partial resection of the lesion, along with resection of the eardrum, malleus, incus, and external canal bones. There were portions along the petrous bone, carotid artery, middle cranial fossa, and sigmoid sinus where the tumor could not be removed.After the surgical resection, adjuvant radiotherapy and chemotherapy were administered with subsequent follow-ups. In January 2020, her follow-up MRI results showed a 5-cm lesion in the nasopharynx that was biopsied and confirmed as a reoccurrence of the ELST. The relapse of the ELST after resection confirms the aggressive nature and high recurrence rate of this condition.Because the tumor relapsed, follow-up CT scans and MRIs were scheduled every six months, and close monitoring was recommended. From the patient's past medical and surgical history, she developed the ELST after the resection of the Schneiderian papilloma. Schneiderian papillomas are mostly benign, non-aggressive neoplasms that arise from the sinonasal mucosa. The development of ELST from a Schneiderian papilloma is quite rare. The pathological variants, classification, differentials diagnoses, etiological factors, clinical features, and treatment modalities of ELST and Schneiderian papillomas are elaborated in detail in the Discussion section.Gaffey et al. labeled tumors with papillary architecture emanating from the middle ear epithelium and invading neighboring bony structures as “primary active papillary tumors of the middle ear” . The comCentered on the clinical, radiologic, immunohistological, and electron microscopy observations, Heffner discovered a papillary neoplasm, most likely emerging from the ELS epithelium, which was a separate entity in 1989 .One in every 39,000 individuals has VHL disease. According to current research, nearly one-third of all ELSTs are linked to VHL disease . VHL is Early routine audiology screening will enable early tumor identification. The likelihood of hearing restoration surgery should occur if there is a family history of VHL disease or, in the absence of an ELST, a diagnosis of VHL disease is made. Before surgery, detection of a tumor on gadolinium-enhanced MRI is required, followed by surgical exploration in patients with VHL disease and audiovestibular signs .Hearing disturbances, vertigo, cerebellar disturbances, and facial nerve paralysis are some of the psychiatric manifestations of ELST. Almost half of the patients had hearing loss, and 44% of them had facial nerve paralysis and cerebellar abnormalities . PapillaELST is a papillary cystic neoplasm that appears similar to thyroid cancer on histology. Since the papillary part of the tumor is tiny, it is critical to separate it from middle ear adenomas, which typically do not have papillary architecture. Unlike paragangliomas, papillary tumors of the temporal bone are unmistakably epithelial. Papillary neoplasms resulting from the ELS and the choroid plexus have microscopic similarities, which may make diagnosis much more difficult. The neuroectodermal origins of the ELS and the choroid plexus are the same . Both arHeffner identified ELST as a low-grade adenocarcinoma in 1989 based on aggressive local development with bone damage, though metastasis has yet to be recorded . HeffnerComplete tumor resection is the treatment of choice. Only one patient with confirmed complete resection had a subsequent recurrence, indicating that complete resection of specific tumors is not possible without risking the devastating loss of function or death so that subtotal resection could be necessary. Patients who have had a subtotal resection may recover from postoperative radiotherapy. However, there is still a 50% chance of tumor regrowth, so careful monitoring is essential because re-resection may be needed. Subtotal resection accompanied by stereotactic radiotherapy has consistently resulted in tumor regrowth in published literature. Stereotactic radiotherapy has not demonstrated any advantage over standard fractionated radiotherapy in terms of survival or recurrence frequency .Schneiderian papillomasGlandular neoplasms of the head and neck are rare, and it is very confusing for clinicians to differentiate between the different subtypes. In our case report, the patient initially presented with a Schneiderian papilloma that was successfully resected. There is no established relationship between Schneiderian papillomas and ELSTs in the literature .The nasal mucosa is lined by pseudostratified ciliated columnar epithelium, also known as the respiratory epithelium, and is derived from the ectoderm. The respiratory epithelium lines the sinonasal tract and is also known as the Schneiderian membrane, which is a source of three histologically and structurally different types of papillomas: inverted, fungiform, and oncocytic papillomas. In turn, these are collectively known as Schneiderian papillomas .The classification, pathology, taxonomy, and etiology of sinonasal tract papillomas are disputed. In 1854, Ward et al. described these papillomas for the first time, and over several years, more than 50 various terminologies have been used to describe these clinical entities . Almost All three subtypes are collectively called Schneiderian papillomas and are very rare, occurring in only 0.4%-4.7% of all sinonasal tract tumors. . Nasal pAlthough there is uncertainty in the etiology of these papillomas, the inverted and fungiform subtypes have been suggested to be viral in origin. The different occupational and environmental factors, toxic agents, smoking, injury, and inflammation may affect the development of these lesions, but these are not very potent factors ,25.Fu et al. have reported nine different cases where patients with Schneiderian papillomas also had a history of anogenital papillomas. However, another study found that patients with sinonasal tract papillomas do not have an increased risk of growing papillomas in other sites of the body .The prevalence of the different types of Schneiderian papilloma changes according to the institution. Fu et al. (1992) jointly reviewed 728 cases of Schneiderian papillomas. They reported that among the different types of papillomas, 6% were oncocytic , 32% were fungiform (6%-50%), and 62% were inverted (47%-78%) [Types of Schneiderina papillomaOncocytic Schneiderian Papilloma (OSP)(1) Among the three different forms of Schneiderian papillomas, the OSP is very uncommon, and it is morphologically similar to the inverted variant. Histologically, these two entities are different and do not share features . The mosEtiologyEtiologically, an OSP is not associated with any viral infection. Many of the patients diagnosed with OSPs have tested negative for the human papilloma (HPV) virus through a polymerase chain reaction. This is in contrast with the other two subtypes of Schneiderian papillomas, the inverted and fungiform types, which show a close association with a viral infection. The most commonly affected age group is the middle-aged population, and this lesion is uniformly distributed between males and females .HistopathologyMicroscopically, the OSP epithelium is comprised of several layers of tall columnar cells, mostly two to eight cells in a layer. The cells have a slightly granular cytoplasm, which is suggestive of oncocytes. Barnes et al. have suggested and proven that the cells show similar characteristics with that of oncocytes but harbor abundant mitochondria and have very high amounts of cytochrome C oxidase content, thereby clearly attaining their oncocytic characteristics . The nucFungiform Papilloma(2) FPs usually affect males between 20 and 50 years of age and are almost 10 times more prevalent in males. On gross examination, FPs are papillary, exophytic, or warty types of lesions that are usually gray, pink, or tan. They typically grow on the anterior part of the nasal septum and compared with OSP, they do not involve the paranasal sinuses . FPs areMRI and CT are the radiological tools used to diagnose Schneiderian papillomas, but the imaging results of FPs are usually not significant. Etiologically, it was suggested that the FPs are caused by HPV, particularly by types 6 and 11 and infrequently by types 16 and 57b. A total of 79 cases of FP were evaluated by polymerase chain reaction for the presence of HPV, and 45 cases (57%) were positive ,37.FPs should be differentiated from other very common keratinizing appendiceal papillomas such as warts and verruca Vulgaris near the nasal cavity. The presence of ciliated pseudostratified columnar epithelium with less keratinization can help diagnose and distinguish FPs from appendiceal papillomas .The treatment of choice for FPs is complete surgical resection. The local recurrence of FPs is 22%-50%, which has been reported in the literature, and the most common cause of recurrence is incomplete surgical excision .HistopathologyOn gross examination, FPs can grow from a few millimeters to up to 2-3 cm and are divided into three subtypes: squamous, septal, and exophytic papillomas. Microscopically, the FPs are composed of a central core of fibro-vascular tissue covered by ciliated pseudostratified columnar epithelium roughly 8-20 cells thick. Commonly the FPs originate from the inner nasal mucosa, which is why the epithelium lacks keratinization properties. In some rare instances, if inflammation, infection, or trauma to the nose or the papilloma is too severe and causes the lesion to push out of the nose, it can be dried by air and may attain keratinization characteristics ,22. Most Inverted Papilloma (IP)(3)The third and last type of Schneiderian papilloma is an IP. This differs from OSP and FP in many aspects, but they all share a common site of origin, which is the sinonasal tract mucosa. IPs are classified based on their histological characteristics, and they account for 1%-4% of all sinonasal tract malignancies ,39. The PathologyOn gross examination, IPs appear as a polypoid mass that is bulky, soft, mildly firm, and granular and are pink to brownish to gray. The most frequent site of origin is the sinonasal tract, specifically the lateral nasal wall and septum. The involvement of the ELS, lacrimal system, paranasal sinuses, and nasopharynx is rare, but they have been reported as sites of origin ,41.Sign and SymptomsThe clinical presentation of IP is pretty similar to the other types of Schneiderian papillomas. Patients usually present with nasal obstruction that can be unilateral or bilateral, increased sinus pressure or infection, facial pain and pressure, headache, rhinorrhea, and epistaxis ,42. AparEtiologyThe definitive etiology of IPs is anonymous. Similarly, to FP, IPs are also associated with HPV infection. Remarkably, HPV strains 6, 11, 16, and 18, and rarely HPV 57. The Epstein-Barr virus (EBV) is also thought to be involved in the pathogenesis of IPs. Macdonald et al. have reported 13 out of 20 cases of IPs that were associated with EBV and confirmed by PCR . Indeed,HistopathologyHistologically, IPs are commonly nonmalignant lesions and rarely complicated by carcinomas, especially squamous cell carcinomas, mucoepidermoid, spindle and clear cell carcinomas, and warts ,43. TheyELST is a unique neoplasm that originates from the ELS epithelium and can be malignant or benign. Malignant lesions exhibit an aggressive nature, with local destruction of tissues but a low risk of distant metastasis. Early wide surgical excision with negative margins is recommended for small lesions and has been the treatment of choice for many years. Because of the structural ambiguity of the ELS and distinct tumor territorial expansion patterns, complete local excision for massive tumors becomes highly challenging. Alternatively, in advanced cases where lesions invade the neighboring structures, radiotherapy, and gamma knife surgery may be the only options.Almost 20% of ELSTs are associated with VHL disease, but there was no VHL mutation in our case. The patient had a history of Schneiderian papilloma two years ago, which then developed into papillary mucinous adenocarcinoma of the ELS. A Schneiderian papilloma is a benign tumor arising from the nasal mucosa and has very low malignant potential and local spread. The development of papillary mucinous adenocarcinoma of the ELS in a patient with a history of Schneiderian papilloma is also sporadic. More cases and studies are needed to prove the association between Schneiderian papillomas and ELSTs.The case was identified because of its unusual presentation. Patients with head and neck neoplasms should be histopathologically diagnosed at an early stage for them to receive early treatment and follow-up. More research needs to be done to better understand the pathological and molecular mechanisms involved in the malignant transformation of ELST and the relationship between Schneiderian papillomas and papillary mucinous adenocarcinomas of the ELS.


Angiosarcoma is a rare, highly malignant tumor prone to recurrence and metastasis. Angiosarcoma is insidious in the initial stage, and its clinical manifestation lacks specificity. The diagnosis is based on histopathology and immunohistochemistry findings.A 73-year-old man was hospitalized following complaints of persistent cough 6 months and hemoptysis for 2 months. Anti-infective treatment was ineffective. A CT-guided percutaneous core needle biopsy of pulmonary lesions revealed organized pneumonia, and the removed skin of purpuric rash area on the left calf revealed vasculitis. Chest CT was used during the patient follow-up. Hormonal therapy combined with immunoglobulins did not lead to improvement, and there was rapid progression of the lung lesions. Subsequently, the patient underwent a surgery, the diseased tissue was separated and removed completely beside the left submandibular gland under local anaesthesia. The immunohistochemical staining indicated CD31 (+) and CD34 (+) confirming a diagnosis of metastatic angiosarcoma. The expression of PD-L1 was 70%, therefore, anlotinib and pembrolizumab treatments were initiated. The patient eventually died.Angiosarcoma is a malignant tumor in the clinic that lacks typical and specific signs and symptoms. The diagnosis depends on immunohistochemistry, which requires repeated biopsies of multiple sites in highly suspected cases. Angiosarcoma is a rare tumor that accounts for less than 2% of all soft tissue sarcomas . It is hAngiosarcoma has a high mortality rate and lacks typical and specific signs and symptoms, which pose as challenges for its diagnosis and treatment by clinicians. Herein, we report the rare cause of pulmonary nodules in an elderly man. The patient had a persistent cough and hemoptysis. Owing to the non-specificity of clinical features, his condition was misdiagnosed as vasculitis and Wegener’s granuloma successively. To determine the cause, he underwent a biopsy several times. Eventually, the cause was confirmed following the resection and biopsy of a neoplasm beside the left submandibular gland, whose immunohistochemical staining suggested CD31 and CD34 positivity, indicating metastatic angiosarcoma. We discuss this case including its clinical features, diagnosis, and treatment. We also discuss the lessons we learned and the challenges experienced. It can play a certain warming role in clinical work and provide evidence for clinicians and radiologists who lack knowledge about it, to diagnose this rare entity.9/L, 82.6% neutrophils), C-reactive protein (CRP) of 92 mg/L, and erythrocyte sedimentation rate (ESR) of 95 mm/h. CT scan of the chest showed scattered high-density nodular shadows of different sizes with clear borders in both lungs on January 10, 2020. The diameter of the largest nodule was approximately 1.5 ×1.6 cm. Some of the nodules were surrounded by ground-glass shadow (18F-fluoro-2-deoxy-D-glucose positron emission tomography-computed tomography (18FDG-PET/CT) showed that multiple solid nodules of different sizes were diffused in both lungs, with a maximum standardized uptake values (SUV) of 4.29  scan was performed using a 64-slice spiral CT  on Aug 1, 2019  and methylprednisolone (40 mg daily) were given. However, cough and blood sputum persisted. The CT was used for the patient follow-up, and the imaging revealed worse findings than the previous one, on February 17, 2020 , and the pathology suggested organized pneumonia. Experienced physicians in the departments of respiratory diseases, hematology, rheumatology, infectious diseases and radiology participated in multidisciplinary discussions, and systemic vasculitis was first considered for which hormone therapy (methylprednisolone 80 mg every 12h) was recommended. During the treatment period, a soft, mobile, non-tender mass was detected beside his left submandibular gland, with the diameter of 1.7 ×1.3 cm using ultrasound scan . When magnetic resonance imaging (MRI) scan was performed using a 3.0 T scanner  with a neck matrix coil, the lesion demonstrated heterogeneous hyper-intensity on axial and coronary fat-suppressed T2-weighted images, as well as on the axial diffusion weighted image. Axial T1-weighted image showed slight hypo-intensity around the left submandibular gland Figure 32), and the patient could not tolerate the surgery.Since hemoptysis did not relieved obviously, the patient was advised to undergo a biopsy again. A bronchoscopic lung biopsy was performed using endobronchial ultrasound-guide sheath, which pathology suggested chronic inflammation. However, he had a severe hemoptysis. Re-examination by CT scan showed that there was an obvious halo sign around the focus, which indicated a lesion combined with bleeding  and its receptor (VEGFR) may be responsible for angiosarcoma formation. Recent studies have shown that VEGF and VEGFR play an important role in tumor angiogenesis, and the overexpression of VEGF is observed in 21%–25% of all patients with soft tissue sarcoma . TargetiThe fatality rate is quite high, the overall 5-year survival rate of angiosarcoma is approximately 35%. Angiosarcoma of the skin is easy to detect, and when it does not involve the main organs, the prognosis is better than that of other parts. According to current literature reports, individuals aged <70 years old, with a tumor diameter <5 cm, located on the trunk have a good prognosis, and approximately 60% of these individuals survive for more than 5 years. Highly aggressive angiosarcomas with a median survival time of the disease of 7 months, have an overall poor prognosis , 27. TimWhile reviewing the literature, we discovered that our patient had a few different and some similar characteristics. We report a rare cause of pulmonary nodules: angiosarcoma. The patient had persistent cough and hemoptysis. The clinical manifestations were highly similar to pulmonary thromboembolism, vascular malformations, lung cancer, severe pneumonia, and tuberculosis; thus, misdiagnosis was easy. The initial symptoms of the patient were consistent with those reported in the literature , 28, theDuring the imaging examination, the CT scan in this patient showed multiple solid pulmonary nodules with a ground-glass shadow initially and hemorrhagic changes and pleural effusion in the later stage as the disease progressed. FDG-uptake on PET/CT scans was lower than that of general malignant tumors. The atypical imaging changes of malignant tumors, which was not consistent with the previous imaging findings of angiosarcoma, indicated that the biological characteristics of angiosarcoma were different from those of other malignant tumors, thus, enhancing the challenges of clinical diagnosis. Moreover, repeated pathological examination and biopsy of different sites showed no sign of a malignant tumor, these were conducive to the occurrence of misdiagnosis or diagnostic error. Eventually diagnosis of angiosarcoma using biopsy, histology and immunohistochemistry were both consistent with angiosarcoma, as reported in the literature , 14.Presently, there are no obvious effective treatments, and the disease progresses rapidly. The patient was treated with anlotinib as suggested by experts, there were many reports showing that the drug could treat several types of malignant tumors –26. PembReviewing the experience in this case, despite multiple clinical findings pointing toward a malignant entity, the negative results of two lung biopsies and non-specificity of the clinical features and the atypical imaging changes of malignant tumors, lead to a delay in the diagnosis of the cause. The following lessons can be learned. First, symptoms were cough and hemoptysis, accompanied by fever and an increased inflammation index, which is a characteristic of infectious diseases. Moreover, most of lesions in CT imaging are prone to inflammatory changes. According to clinical experience, infectious disease was considered first, and the opportunity for early diagnosis was missed. Second, malignant tumors generally show hypermetabolism in PET/CT examination, which is inconsistent with the reports. This shows that PET/CT has limitations in the diagnosis of low-metabolic malignancies. Third, biopsy has a certain negative rates, it depends on the puncture method, location and the size of the lesion. In this case, the patient underwent lung biopsies, twice, and both did not indicate a malignant tumor. We assumed that the error in pathological results were owing to fewer samples obtained by puncture biopsy and various degrees of inflammation around the lesions probably, which might have affected the correct diagnosis , 29. TheWhen the clinical features lack of specificity, physical examination plays an important role in the diagnostic process as it they may uncover an alternative cause of the patient’s symptoms. The clinicians must pay attention to the new signs and symptoms. While examining a case with clinical findings not matching the pathology, the pathologist should be consulted.Angiosarcoma lacks typical and specific signs and symptoms. The diagnosis depends on immunohistochemistry, which requires repeated biopsies of multiple sites in highly suspected cases. The present case provides evidence of a rare cause of pulmonary nodules and hemoptysis and play a certain warning role in the diagnosis and treatment of similar diseases. It also contributes to the existing knowledge in the subject and will aid clinicians and radiologists in diagnosing this rare entity.The original contributions presented in the study are included in the article/Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.PW was a major contributor in writing the manuscript. LX and YY were main physicians for the patient, and proofread and revised the manuscript. All authors contributed to the article and approved the submitted version.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.


Peritoneal lymphomatosis is extremely rare and associated with poor prognosis. Most practitioners only pay more attention to peritoneal carcinomatosis. However, peritoneal lymphomatosis can be neglected and misdiagnosed. We report a teenager with 10 days of abdominal distension and pain accompanied by computed tomography scan suggesting diffuse thickening of the peritoneum and omentum and abdominopelvic effusion. Tuberculous peritonitis and peritoneal carcinomatosis were initially suspected. However, it was finally confirmed as non-Hodgkin’s B-cell lymphoma by omentum biopsies. He achieved complete remission after chemotherapy and autologous stem cell transplantation. But unfortunately, he suffered a relapse and died 10 months after diagnosis. Following a review of the literature, it can be concluded that the discovery of lymphomatosis in peritoneum is a rare finding. Lymphoma should be considered in the differential diagnosis of unexplained peritoneal thickening on computed tomography, and this case emphasizes the importance of early pathological diagnosis to make sure that the right treatment can be started opportunely. Non-Hodgkin’s lymphoma (NHL) includes more than 90 distinct genotypes of hematologic malignancies. About 90% of NHL cases are of B-cell origin, most commonly seen in diffuse large B-cell lymphoma (DLBCL) . DLBCL hA 16-year-old boy was admitted to our hospital with a 10-day history of abdominal distension and epigastric pain, accompanied by anorexia, nausea, vomiting, dyspnea, palpitations, as well as low fever and night sweating. He had always been in good health with no significant medical or malignant family history.On physical examination, the patient showed tachycardia, abdominal tension, tenderness, rebound tenderness in the upper quadrant, and shifting dullness. No palpable masses and no extremity edema were noted. Neither lymphadenopathy nor hepatosplenomegaly was detected.9/L (reference range: 3.50–9.50×109/L), neutrophils 8.03×109/L (reference range: 1.80–6.30×109/L), and monocytes 1.10×109/L (reference range: 0.10–0.60×109/L). The values of lymphocytes, hemoglobin, and platelets were normal. Fecal occult blood test was positive. The laboratory tests revealed a high uric acid level of 1,240.2 μmol/L (reference range: 208.0–506.0 μmol/L), aspartate aminotransferase value of 57 U/L (reference range: 15–46 U/L), and the serum carbohydrate antigen 125 value of 758 U/ml . Other values of blood urea nitrogen, creatinine, electrolytes, Epstein-Barr virus (EBV), human immunodeficiency virus (HIV), as well as other tumor markers , and hepatitis markers were negative. Assistant examinations associated with tuberculosis  were negative. Additionally, abdominocentesis and ascitic fluid examination were performed as well. Orange-yellow turbid fluid with predominance of mononuclear cell indicated exudate.Peripheral blood counts elevated in white blood cell 10.71×10Cervicothoracic and whole abdominal computed tomography (CT) scan without any intestinal involvement, tumor formation, or enlarged lymph nodes revealed diffuse thickening of peritoneum and omentum, thoracic, abdominal, and pelvic effusion  regimen with rituximab. During the first course of chemotherapy, the patient experienced signs and symptoms concerning for tumor lysis syndrome, including oliguria, hyperuricemia, hyperkalemia, hyperphosphatemia, and hypocalcemia. Follow-up CT reexamination demonstrated remission of his condition , which is described and explained in few past cases and studies seen in , 11–13. There usually are two ways of peritoneal invasion in malignant lymphoma . First, The differential diagnosis of peritoneal thickening includes peritoneal lymphomatosis, peritoneal carcinomatosis, tuberculous peritonitis, mesothelioma, and metastatic tumors, such as ovarian, breast, and gastrointestinal carcinomas , 21. PL As imaging findings are non-specific, it has been proven that only pathology can make a definitive diagnosis . AathiraPeritoneal lymphomatosis is rare and aggressive. Based on these previous experiences and literature reviews, chemotherapy regimens for peritoneal lymphoma are similar to those for DLBCL using CHOP with or without rituximab based on staging , 10. TweIn conclusion, PL is a rare presentation of extranodal involvement of lymphoma with known poor prognosis. With no characteristic clinical presentations and specific imaging, PL is often misdiagnosed, and the delay of the diagnosis reduces the possibility for optimal treatment and chance of survival. In such ambiguous cases, early histological diagnosis is the primary tool of the rapid and effective treatment.The original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding authors.Ethical review and approval was not required for the study on human participants in accordance with the local legislation and institutional requirements. Written informed consent to participate in this study was provided by the participants’ legal guardian/next of kin. Written informed consent was obtained from the individual(s), and minor(s)’ legal guardian/next of kin, for the publication of any potentially identifiable images or data included in this article.MZ did the data collection and wrote the original draft. ZW, ZY, BN, SY, and HY contributed to patient’s care and did a critical revision of the manuscript. XG contributed to the pathology description and images. HY and XG contributed to the final version of the manuscript. All authors contributed to the article and approved the submitted version.This work was supported by Kuanren Talents Program of the Second Affiliated Hospital of Chongqing Medical University and Young Medical High-end Reserve Talents Program of Chongqing (to HY).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.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.