Teratoma is a germ cell tumor, deriving from totipotent cells. Teratomas usually occur in gonads and are rarely extra-gonadal. The adrenal location is exceptional. Only few cases of primary adrenal teratomas have been reported in literature, mainly in young patients.We report the case of a 56-year-old female patient who presented with pyrosis, dyspepsia and abdominal pain that was evolving for 5 months. The abdominal computed tomography revealed a voluminous mass of the right adrenal gland and the hormonal evaluation was normal. The patient underwent an open transperitoneal adrenalectomy and the histopathological examination of the specimen confirmed the diagnosis of mature teratoma.Adrenal teratomas are commonly asymptomatic and their diagnosis depends mostly on radiologic findings. Malignant transformation is very rare. Surgical excision is the mainstay of treatment with a good prognosis.Open surgery should always be considered in large and adhering teratoma tumors of the adrenal gland. Some pathologic features and tumorigenesis of adrenal teratomas are not entirely elucidated, thus the importance of larger studies in order to comprehend this pathological entity. •The adrenal location of primary adrenal teratomas is exceptional.•The preoperative diagnosis is challenging, based mainly on imaging findings.•Open surgery should always be considered in large and adhering teratoma tumors of the adrenal gland. In adults, these tumors occur regularly in gonads and are rarely extra-gonadal. Retroperitoneal teratomas account for 4% of all primary teratomas and are more common in children, whereas adrenal teratomas are even rarer . Based oIn this paper, we report the case of a mature adrenal teratoma in an adult female patient with a systematic literature review. This case has been reported following the SCARE criteria .2A 56-year-old female patient with unremarkable personal and family history presented with right side upper abdominal pain that appeared 5 months before. The physical examination was normal. The abdominal contrast-enhanced computed tomography (CT) revealed a large spherical mass of the right adrenal gland, consisting majorly of fatty component, with minimal soft tissue density and a large calcification, sized 76 × 72 × 80 mm (depth x width x height) . The tumRoutine biological assessment, including liver and kidney function tests and complete blood count, was unremarkable. Adrenal-related hormonal assessment was normal: 24-h urine metanephrine was at 0.27 μmol/24h  and normetanephrine at 0.31 μmol/24h . In the supine position, aldosterone was at 169 pmol/l , renin was at 4.5 mUI/l  with an aldosterone-to-renin ratio at 34 (NV < 64). In the upright position, aldosterone was at 461 pmol/l , renin was at 39.7 mUI/l  with an aldosterone-to-renin ratio at 12 (NV < 64). Moreover, 24-h urinary free cortisol was normal at 630 ng/24h , and morning serum cortisol was at 14ng/ml after 1 mg dexamethasone suppression test . These results excluded a functional adrenal tumor. Tumor markers were normal: alpha-fetoprotein (AFP) was at 1.5 ng/ml , human chorionic gonadotropin (hCG) was at 1.99 mUI/ml , CA19-9 was at 5.32 U/ml  and carcinoembryonic antigen (CEA) was at 1.94 ng/ml .Thus, a transperitoneal adrenalectomy under general anesthesia, in the left lateral decubitus position, was performed by the surgical oncology department. Intraoperatively, the tumor was difficult to mobilize as it was intimately adherent to the IVC, the right liver and the right pillar of the diaphragm . The masThe postoperative gross examination showed a gray well-encapsulated mass, measuring 8 × 6 × 7 cm and weighing 247g. Upon dissection, the tumor contained hair, sebaceous material and bone fragments. Microscopically, the tumor included sebaceous and sweat glands, respiratory epithelium, cartilage and bone material . The dia3Teratomas are germ cell tumors composed of mature tissues arising from more than one germinal layer i.e., ectoderm, mesoderm, and endoderm. Teratomas occurring in early childhood are mostly extra-gonadal, while those occurring in adults are mainly found in the gonads . The retFor our systematic review of primary adrenal teratomas in adults, we searched for the terms of “adrenal teratoma” in the PubMed database. A total of 289 studies were published between June 1952 and December 2021. We excluded the cases reported in children, review articles, letters, abstracts without full text and irrelevant publications. Out of the remaining studies, we identified 40 cases of primary adrenal teratomas in adults aged more than 16 years reported in 19 publications. We extracted and summarized demographic, clinical, imaging, management and follow-up data in According to literature, primary adrenal teratomas in adults are extremely rare with only 41 cases reported cases in the 20th century, in patients aged between 16 and 75 years. They can occur in a wide age range, with a peak in the third decade of life and a mean age of 33.2 years, and seem to affect more females than males with a male-female sex-ratio of 0.31 . As regards laterality, 52.2% of the masses occurred in the left adrenal gland, while 46.3% were found in the right side including our case. One case presented with bilateral adrenal teratomas .In terms of clinical presentation, primary adrenal teratomas can be discovered fortuitously (22 cases), or can present with non-specific symptoms like abdominal pain when the diameters become large enough to exert pressure on neighboring organs (19 cases). The average tumor size was 6.23cm. The largest reported mass was sized 38 cm and weighed 10kg .Adrenal teratomas are typically non-functional tumors. In the available data, thirty patients, including our case, have benefited from a preoperative endocrine evaluation that was strictly normal, except one case of a 36-yar-old patient who had abnormal laboratory values of cortisol, adenocroticotropic hormone (ACTH), aldosterone, and vanillyl mandelic acid (VMA) due to a malignant transformation of a mature adrenal teratoma with adenocarcinoma components . Tumor mAbdominal CT is the gold standard for preoperative diagnosis. However, depending on the composition of the mass, the diagnosis is usually difficult. Classically, these tumors present as well-circumscribed masses with mixed density secondary to the presence of multiple tissue types  and a predominant fatty density . DavidsoOur patient underwent open surgery for her large adhering mass. Currently, laparoscopic surgery has become the mainstay of treatment for benign adrenal tumors dethroning open surgery, since it has lower morbidity and allows an earlier recovery with less pain . HoweverHistopathological examination is the key for conclusive diagnosis of adrenal teratoma. Mature teratomas appear as well-encapsulated tumors, with smooth appearance, mainly cystic and composed by two or three germ layers, usually comprising fully mature elements i.e., sebaceous material, hair, bone and fat components. Immature teratomas include at least 10% of undifferentiated tissue ,29.Gatcombe et al.  reportedThe overall prognosis after complete surgical excision of adrenal teratomas is favorable. However, whether the teratoma is mature or immature, a regular post-operative follow-up is mandatory for early identification and management of recurrences and metastases . The fol4Teratomas rarely present as retroperitoneal tumors in adults, particularly in adrenal glands. Their preoperative diagnosis remains difficult and its confirmation depends solely on histopathological examination. Surgical management of mature adrenal teratomas is the mainstay of treatment with an excellent postoperative prognosis. Several clinical and pathologic features of adrenal teratomas are not clearly elucidated. Further studies are necessary in order to comprehend their tumorigenesis and pathologic properties.This research was not funded.A written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.This is a case report that does not require a formal ethical committee approval. Data were anonymously registered in our database. Access to data was approved by the head of the department.Dr. Imane Assarrar wrote the manuscript.Dr. Marouane Harhar helped in writing and provided surgical data.Dr. Dounia Zerrouki helped in writing and literature review.Dr. Najat Draoui helped in writing and literature review.Dr. Achraf Miry interpreted and provided pathological data.Pr. Amal Bennani confirmed the histological diagnosis and revised the final manuscript.Pr. Siham Rouf helped in writing, supervised the redaction and revised the manuscript.Pr. Tijani El Harroudi operated the patient and revised the final manuscript.Pr. Hanane Latrech helped in writing, supervised the redaction, revised and approved the final draft for publication.All authors approved the final version of the manuscript.This is not an interventional study. We only reported the patient's findings from our database as a case report.Professor Hanane Latrech.Not commissioned, externally peer reviewed.The authors declare no conflicts of interest.


Sarcoma is a rare type of tumor that can arise in the different types of connective tissues. Symptoms vary depending on the size, type, and location of the tumor. Management and surgery should be performed in a referral sarcoma center with a molecular biology platform and a dedicated medical staff. A preoperative percutaneous core needle biopsy (CNB) is required to tailor the medical and surgical strategies. In this report, we describe the case of a well-differentiated retroperitoneal liposarcoma (WD LPS) discovered in the context of recurrent cystitis on an abdominal CT scan, synchronously occurring, with another different type of sarcoma, undifferentiated pleomorphic sarcoma (UPS) developing in the thigh, discovered because of sciatic pain. This extremely rare condition was confirmed by the molecular analysis and justifies a specific strategy taking account of the differential risk. This event should also encourage genetic counseling. One-third of sarcomas occur in the trunk, whereas 50% occurs in the extremities with more than 150 subtypes according to WHO classification . SymptomThe patient is an 80-year-old female, hypertensive, dyslipidemic with no prior history of cancer or sarcomas. Her brother was diagnosed at the age of 73 with lung  cancer due to smoking, and the other brother was diagnosed with colon cancer at the age of 73. The patient had recurrent cystitis for which her treating general practitioner (GP) prescribed an abdominal CT scan that showed a right retroperitoneal adipocytic tumor of 11 cm in the anterior-posterior axis and 10 cm in the transverse axis, encasing the right kidney partially. Without any multidisciplinary tumor board (MTB), the initial strategy conducted by her GP was surveillance. After one year, the patient suffered from a left thigh pain that motivated her GP to prescribe a thigh MRI that showed a heterogeneous tumor of the posterior left thigh Figure . Both tuSarcomas are rare tumors ; howeverThe occurrence of simultaneous, different histologic types of sarcoma is extremely rare. Percutaneous biopsy is mandatory to tailor the treatment strategy. Biomolecular and histologic analyses are required to differentiate each tumor’s type. The grade and histological subtype guide further management. Well-differentiated retroperitoneal sarcoma does not metastasize but progresses slowly and locally, whereas the undifferentiated pleomorphic sarcoma of the thigh is at risk of local and metastatic progression. This is the reason why the tumor with the highest potential for metastasis was treated first. Adjuvant radiotherapy is the standard of care for high-grade sarcoma of the extremities, validated by two randomized studies. A recent randomized study (STRASS) did not show a significant benefit in terms of local control for RPS, although a signal of activity has been shown in LPS. Complete, en bloc, gross resection is the cornerstone of management of RPS with the objective to achieve macroscopically complete resection, with a single specimen encompassing the tumor and involved contiguous organs. Given the uncertainty regarding margins definition, a compartmental approach with the resection of adherent visceral irrespective of expected microscopic infiltration should be considered for retroperitoneal LPS. In front of two different sarcomas affecting the same patient, genetic counseling is advised.


Primary Seminal Vesicle (SV) tumours are a rare entity, with most SV masses representing invasion of the SV by malignancy originating in an adjacent organ, most often the prostate. Previously reported primary SV epithelial tumours have included adenocarcinoma and cystadenoma, with limited prior reports of inracystic papillary structures.A 35-year-old male presented with azoospermia, intermittent macroscopic haematuria, and mild right iliac fossa and groin pain. A papillary appearing seminal vesicle mass was found on imaging and seminal vesicoscopy. The mass was robotically excised with diagnosis of benign cystic papillary adenoma made.In this manuscript we describe a rare case of a benign cystic papillary adenoma of the seminal vesicle, a unique histological entity differentiated from cystadenoma of the Seminal Vesicle by its papillary component. Primary Seminal Vesicle (SV) tumours are a rare entity, with most SV masses representing invasion of the SV by malignancy originating in an adjacent organ, most often the prostate. Primary SV tumours can be considered in groups according to their origins from epithelial, mesenchymal or other tissues, or from an infectious or inflammatory cause. Other rare SV entities that may need to be considered in the differential diagnosis include borderline Mullerian type serous neoplasms. Overall, benign aetiologies are less common than a malignant cause.Previously reported primary SV epithelial tumours have largely included adenocarcinomas and cystadenomas, with few prior papers describing intracystic papillary structures –8. We reA 35 year old male was referred with an 8 month history of intermittent macroscopic haematuria, associated with mild right sided iliac fossa and groin pain. Past medical history included a prior right inguinal hernia repair and orchidopexy for undescended testis. The patient was also found to have azoospermia and was undergoing assessment in preparation for In vitro fertilization (IVF)/Intracytoplasmic Sperm Injection (ICSI). Examination of genitalia, testes and prostate was essentially unremarkable outside of an old right inguinal scar consistent with the history of orchidopexy and hernia repair. There was some thickening of the tissues around his right testicle consistent with previous orchidopexy. Initial workup of haematuria was performed. Urine microscopy demonstrated nonglomerular microscopic haematuria, cytology revealed atypical cells and the computerized tomography (CT) intravenous pyelogram (IVP) reported normal appearance of the upper urinary tract and bladder. However, it also described the incidental finding of a 7 × 3.5 × 2 cm fluid density mass at the posterior aspect of the bladder continuous with the right seminal vesicle. Blood tests were unremarkable including a PSA of 0.6 ng/ml. The seminal vesicle mass was further investigated with magnetic resonance imaging (MRI) which reported two cystic structures adjacent the bladder and appearing related to the seminal vesicle Fig. . The larMacroscopically, the right seminal vesicle contained a friable tan-brown colored lesion measuring 15 × 13 mm Fig. . On micrGiven the atypical findings the specimen was referred to Uropathologist Professor Jonathan Epstein at the Johns Hopkins Hospital  where a final diagnosis was made as a benign Cystic Papillary Adenoma of the seminal vesicle, which was considered a unique, distinct entity from cystadenoma of the seminal vesicle, which lacks the papillary component seen in this case.The patient will continue with IVF workup and treatment. His MRI and urine cytology will be repeated 6-monthly for 2 years as a precaution prior to discharge.Given the unique histological findings we conducted a literature review, searching PubMed and EMBASE databases with the search terms Cystic Papillary Adenoma and Seminal Vesicle . Ultimately, despite the broad search strategy, only limited prior reports of intracystic papillary structures were identified , 8.This raises several issues for the ongoing management of the patient. Firstly, is there a relationship between the seminal vesicle mass and the patient’s presenting problems of azoospermia, macroscopic haematuria and right sided abdominal and groin pain? While these initially appeared to be unrelated pathologies, it is not inconceivable that the SV mass may explain all three complaints; being of a significant size that could cause pain and a degree of obstruction to normal passage of sperm into the ejaculate, and vascular with potential to intermittently bleed. We anticipate further clinical review of symptoms, repeated urine microscopy and semen analyses would demonstrate resolution following excision of the SV mass if it is the cause. We do recommend the patient and partner continue with IVF treatment as planned in the interim to maximise the likelihood of achieving a healthy pregnancy in the event azoospermia persists, especially considering that the unilateral nature of the SV mass makes the hypothesis of an exclusively obstructive cause of azoospermia somewhat less likely.Secondarily while the lesion is reported to be entirely benign and excised in its entirety with clear margins, with limited prior reports of this pathology there is no definite existing evidence on which to base further follow up. Given other benign papillary pathologies of the lower urinary tract such as papillary urothelial neoplasm of low malignant potential (PUNLMP) are known to have the potential to recur, and the potential relationship of this mass to the patient’s clinical presentation, we believe ongoing surveillance is warranted but optimal modality, frequency and duration are unclear. Initially we recommend repeating urine microscopy, cytology and imaging with MRI or CT on a six monthly basis. Surveillance could subsequently be performed annually if all investigations remain normal, with discharge to a general practitioner considered at 10 years post treatment without recurrence. However, should the patient develop recurrence of macroscopic haematuria or other symptomatology they should be thoroughly re-investigated with urine cytology, CT, IVP and cystoscopy, and we stress that the benign diagnosis of cystic papillary adenoma of the SV should not falsely reassure the clinician of the absence of more sinister diagnoses, potential recurrence or progression to a more aggressive lesion.Finally, the use of a rigid ureteroscope to endoscopically visualize the mass was a useful component of initial investigation in this case. Consequently, while a technically challenging procedure, we propose that in the hands of an experienced endourologist, this technique may be useful in evaluation of similar presentations in future.


Optic nerve ultrasound is an established routine supplementary diagnostic tool for idiopathic intracranial pressure but it can also be helpful in avoiding misdiagnoses. We describe a case of an obese 15- year-old girl with persistent headaches, fundoscopic findings suggesting papilledema, normal brain imaging who underwent two lumbar punctures with unremarkable cerebrospinal fluid findings before ultrasound revealed optic disc drusen as the cause of the optic disc elevation. Identification of optic nerve head edema in a young woman with chronic headaches and normal brain imaging is highly suggestive of idiopathic intracranial hypertension (IIH) . HoweverIn this case report, we describe the diagnostic work-up of an obese 15 year old girl with persistent headaches and fundus findings suggesting papilledema.2Ο. All laboratory examinations were unremarkable. No diagnosis of IIH was made at that time.A 15-year-old girl with a BMI of 32 kg/m2 was referred to an outside Institution by an outpatient ophthalmologist due to abnormal fundus findings suggesting “papilledema” in order to rule out IIH. The girl complained about recurrent headaches over the past year without any other symptoms such as transient visual obscurations, pulsatile tinnitus, blurred vision or diplopia. Neurological examination and visual acuity were normal. Past medical history was unremarkable. Brain magnetic resonance imaging (MRI) and MR venography (MRV) were normal. She underwent lumbar puncture with normal cerebral spinal fluid (CSF) findings and opening pressure measured at 23 cm Η20). An ultrasonography of the optic nerve sheath diameter (ONSD) was performed and -correlating with the measured CSF opening pressure- it was found within normal limits   with typical corresponding neuroimaging findings on brain MRI & MRV . Over thHowever, when predominately relying on interpretation of fundus findings the diagnosis of IIH can be challenging since not all optic disc swelling is due to papilledema. Examination of the ocular fundus is often misinterpreted especially in female obese headache patients resulting in invasive and costly tests, medication use and in some cases in unnecessary aggressive treatments. According to a large retrospective study overdiagnosis of IIH was documented in 34/86 patients 39.5%) with presumed IIH, especially due to errors committed in fundus evaluation mainly in obese women in childbearing age. ODD was responsible for 2/34 misdiagnoses (6%) [% with prODD are acellular deposits of calcium, amino and nucleic acids, and mucopolysaccharides in the optic nerve head with a reported prevalence in adults of 1.8 and 1% in children , 7. ODD Ocular ultrasound is very useful in the detection of ODD including buried drusen. It is a reliable, inexpensive and fast diagnostic tool. B-scan ultrasound is considered diagnostic if there is an area of hyperreflectivity present at the nerve head. ODD also cause acoustic shadowing of posterior structures .In conclusion, the routine use of sonographic measurements of ONSD values is useful in detecting raised ICP in patients with presumed IIH as it easily detects papilledema and enlarged ONSD in IIH. Furthermore, the diagnostic utility of optic nerve sonography may further increase since it may reveal ODD as the underlying cause of optic disc elevation in fundoscopy, avoid IIH misdiagnosis and prevent an extensive, invasive and unnecessary diagnostic work-up.


Rosai-Dorfman disease (RDD) usually presents with painless cervical lymphadenopathy during the two first decades of life, with or without extranodal involvement. Exclusive extranodal manifestation, and especially central nervous system (CNS) involvement, is uncommon. The etiology remains unknown and definitive diagnosis is based on characteristic histopathological and immunohistochemical features showing S100(+) CD1a(−) RDD type histiocytes with emperipolesis. Most CNS cases are dural-based masses.Herein, we present a case of RDD in an 8-year-old boy, who presented with multiple huge intraventricular masses.Regarding rare CNS involvement and lack of established evidence-based therapeutic approaches, reports of any individual case can supply further beneficial data concerning treatment approaches and long-term effectiveness of therapeutic strategies. Rosai-Dorfman disease (RDD), alternatively called as sinus histiocytosis with massive lymphadenopathy (SHML), typically shows massive, painless cervical lymphadenopathy along with high body temperature, leukocytosis, high erythrocyte sedimentation rates, and polyclonal hypergammaglobulinemia during the first or second decade of life . In overMajority of CNS instances are dural-based masses, mostly involving the two sides of the dura and mimicking meningioma . The defRDD can be misdiagnosed with different histotypes of meningioma and intracranial solitary fibrous tumors (ISFTs). A retrospective study on these rare mesenchymal neoplasms has reported a recurrence rate of about 43% for solitary fibrous tumors and an approximate recurrent rate of 27% for hemangiopericytomas . Thus, hDiffuse lymphoplasmacytic infiltrate and histiocytes of both typical and RDD-type, showing lymphophagocytosis with no destruction of cells (emperipolesis), are characteristic histopathologic features. RDD histiocytes have bigger, more hyperchromatic nuclei compared to the other type. Moreover, they are sometimes multinucleated with plentiful light pink cytoplasm, and show immunoreactivity for CD68 and S100 but they are negative for CD1a on immunohistochemistry .In several instances, RDD histiocytes undergo quick and full spontaneous resolution. In other cases, particularly those which show extensive extranodal involvement, a long clinical course may be experienced for several years or even decades. Therapy does not usually affect RDD, even though there have been several instances of successful chemotherapy . It is rA boy aged 8 years, suffering from multiple intraventricular huge masses resulting in hydrocephalus, was referred to the neurosurgery ward of the Loghman Hakim Hospital. Written informed consent forms were signed by the parents. The study protocol was approved by the Ethical Committee of Shahid Beheshti University of Medical Science, and all methods were performed in accordance with the relevant guidelines and regulations (IR.SBMU.RETECH.REC.1400.385). He had initially presented with fever, dizziness, and bitemporal headache about 1.5 years ago and was admitted in a pediatric center with an impression of meningitis. However, lumbar puncture was not performed at that time because of brain edema, bilateral papilledema as well as evidence of choroid plexus hypertrophy and abnormal extra-intracranial vascular connections. He had normal chest radiography and abdominopelvic sonography. He had been discharged in a relatively good condition after antibiotic therapy. On this admission, he presented with nausea, vomiting, and ataxia. On examination, no sign of adenopathy or organomegaly was evident. Brain MRI revealed large homogeneously enhancing bilateral ventricular lesions that seemed to configure the shape of the whole ventricles . As the Histopathologic examination revealed diffuse proliferation of histiocytic cells, some with abundant pale eosinophilic cytoplasm and characteristic emperipolesis in a densely fibrotic stroma admixed with a variable amount of lymphoplasmacytic infiltrate . StaininRosai-Dorfman disease can involve almost any organ system and affect any age group. The CNS involvement is uncommon, occurring in around 5% of cases . NeverthIso-intensity or hyper-intensity and homogeneity of RDD lesions of the CNS can be evident on computed tomography (CT or CAT), enhanced by contrast administration, but with no calcifications. On the other hand, MRI shows the homogeneity and isointensity of RDD lesions on T1-weighted images (T1WI) while indicating heterogeneity of RDD lesions with isointense and hypointense areas on T2-weighted images (T2WI). Gadolinium administration enhances the lesions homogeneously, usually showing a dural tail. Both CT scan and MRI methods usually indicate perilesional edema , 19.There are similarities between the RDD histopathological characteristics in extranodal manifestations and nodal involvement. The only difference is in more distinct fibrosis and less obvious emperipolesis in extranodal sites . SimilarRosai-Dorfman disease of the CNS is preferably managed by surgical resection that relieves neurologic symptoms. Adjuvant therapies are taken into account when neurological symptoms persist or lesions appear around vital structures. There are different therapies, including fractionated radiotherapy, stereotactic radiotherapy, corticosteroids, and chemotherapy, but more investigations need to be performed to find the optimal management of the disease . A systeChemotheraputic modality is used to shrink space occupying lesions alongside other treatment modalities. Rarely, RDD can be presented as two isolated lesions that originated from a single clone . SimilarRosai-Dorfman disease of the CNS can be defined as an uncommon presentation, which requires consideration in differential diagnosis of not only dural-based but also unusual intraventricular brain lesions, especially in children. Definitive diagnosis is according to the histopathology and immunohistochemistry. Surgical resection seems to be the most effective therapy, and given that the disease is rare, evidences obtained from any individual case studies can provide further important information regarding the treatment approaches and the long-term effectiveness of treatment strategies for relapse/incompletely resected lesions.Our study had a limitation regarding lack of long term follow-up. Further assessment of additional similar cases is also required for estimation of recurrence rate and efficacy of therapeutic modalities.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.The studies involving human participants were reviewed and approved by Shahid Beheshti University of Medical Sciences. The patients/participants provided their written informed consent to participate in this study.EJ and GS wrote the draft and revised it. MT, FB, and MY collected the data and performed the clinical assessment. All authors read and approved 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.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.


A 33-year-old female patient presented with abdominal distention and lower extremities edema. Abdominal wall varicosis and shifting dullness were positive on physical examination. Her liver function was classified as Child-Pugh B and a solid tumor at retro-hepatic vena cava extending to right atrium with thrombi in three hepatic veins were confirmed. The diagnosis of leiomyosarcoma with Budd-Chiari syndrome was highly suspected with preoperative ultrasound, echocardiogram, CT scan, and three-dimensional reconstruction. A zone II-III leiomyosarcoma of IVC origin was confirmed at surgery and ex vivo liver resection and autotransplantation, and hepatic vein thrombectomy with atrial reconstruction were performed under cardiopulmonary bypass (CPB). Operative time, anhepatic time, and CPB time were 12 h, 128 min, and 84 min, respectively. The patients experienced post-operative liver dysfunction and was cured with conservative therapy. Hepatic recurrence two years after surgery was managed with radiofrequency. The patient was alive with liver metastasis three years after surgery. Despite being regarded as an extremely aggressive procedure, ELRA could be considered in the treatment of advanced leiomyosarcoma with Budd-Chiari syndrome and hepatic vein thrombi.We report the first documented case of leiomyosarcoma at zone II-III of inferior  Ultrasonography showed ascites, hepatic congestion, and a solid tumor in retro-hepatic olvement Figure 1A multi-disciplinary team consisting of members from Liver Transplantation, Surgical Oncology, Cardiothoracic surgery, Cardiothoracic Anesthesiology, and Hepatology reviewed patients’ general information and reports. Despite the compromised hepatic function of the patient, surgery was considered as the only curative option for solving the extending tumor and secondary Budd-Chiari syndrome. ELRA with bench thrombectomy was planned considering tumor involvement to three hepatic veins and right atrium as well as thrombi. Gore-Tex graft replacement of the IVC was considered if IVC-sparing tumor extraction was not possible.vena cava circumferentially, freeing the IVC from the retroperitoneum as well as diaphragm. Major vasculatures were carefully dissected, and hepatic artery, portal vein, common bile duct, and intrahepatic vena cava were totally freed and suspended. No significant bleeding occurred during the mobilization of the liver. CPB was then initiated after systemic heparinization via cannulation of ascending aorta, right atrium, and femoral vein. Clamps were placed on the infra-hepatic IVC below the margin of the lesion but above the bilateral renal vein levels, as well as hepatic artery and portal vein. Then, right atrium was incised above the tumor margin, and a vent was placed in coronary sinus. The hepatic inflow and outflow were disconnected for explanting the whole liver with the tumor  solution  thrombi Video 1.Primary leiomyosarcomas of IVC originate from the smooth muscle of vascular wall and present as a rare clinical entity . They usen bloc resection with complicated vascular reconstruction are required. Although postoperative recurrence is common, a pooled data analysis of 377 surgically resected leiomyosarcomas of IVC claimed that radical resection is the best treatment modality and, in many circumstances, can present a complex procedure requiring a multi-disciplinary team to manage appropriately  of only 6%. However, long-term survival was possible after radical resection, with 5-year overall survival (OS) of 55% . In liteLong-term survival is possible after ELRA for IVC leiomyosarcoma. Hepatic veins thrombi and Budd-Chiari syndrome secondary to leiomyosarcoma does not necessarily preclude the option for ELRA. Recurrence of this rare tumor is common, and resection should be considered in light of the tumor biology and the general condition of the individual patient.The original contributions presented in the study are included in the article/The studies involving human participants were reviewed and approved by Ethical committee of First Affiliated Hospital of Xinjiang Medical University. The patients/participants provided their written informed consent to participate in this study.TT and TL drafted the manuscript. SA, Y-BH, LB, and S-SG performed literature search. Z-PW, TT, and TL analyzed the data. TT, TL, QH, JW, and J-MZ performed the surgery. J-MZ, TL and TT critically revised the manuscript. All authors contributed to the article and approved the submitted version.This work was supported by grants from Xinjiang Uyghur Autonomous Region Key Laboratory Open Research Program (2018D04002); Tian Shan Youth Program (2017Q094); Shu-Lan Excellent Project-Support Program for Overseas Study of Young Talents in Organ Transplantation, 2017; and Xinjiang Natural Science Funding (2021D01C299).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.


Leiomyosarcoma is a rare tumor that could originate from the gastrointestinal tract, uterus, kidney, retroperitoneum, and the soft tissues of the extremities. It accounts for only 1% of all gastrointestinal mesenchymal tumors and primary leiomyosarcoma of the stomach is extremely rare. Most cases reported as leiomyosarcoma of the stomach before the development of KIT immunohistochemistry might be gastrointestinal stromal tumors (GISTs) of the stomach and only 18 cases of leiomyosarcoma of the stomach have been reported since early 2000s. We report here a patient with leiomyosarcoma of the stomach treated by laparoscopic and endoscopic cooperative surgery (LECS).A 59-year-old man was referred to our hospital for an early gastric cancer, which was initially treated by endoscopic submucosal dissection. Six months after his initial treatment, a follow-up esophagogastroduodenoscopy revealed a small polypoid lesion at the lesser curvature of the proximal stomach, which appeared to be a hyperplastic polyp. However, one and a half years later, the lesion grew and showed more irregular surface. Biopsy at the time revealed smooth muscle cell proliferation suggestive of leiomyoma. Three years later, the lesion grew even larger and biopsy showed pleomorphic spindle cells. Immunohistochemical study showed positive staining for alpha-smooth muscle actin and desmin, but negative for c-kit and CD34. Ki-67 labeling index was nearly 60%. Based on these findings, the diagnosis of leiomyosarcoma was established. The patient subsequently underwent a partial gastrectomy by LECS. The patient is currently in good condition without recurrence or metastasis at 12 months after surgery.Leiomyosarcoma of the stomach is extremely rare. This is the first report of leiomyosarcoma of the stomach treated by LECS. We could also follow its appearance change through endoscopic examination for 3 years. Leiomyosarcoma is a rare tumor that could originate from the gastrointestinal tract, uterus, kidney, retroperitoneum, and the soft tissues of the extremities . PrimaryA 59-year-old male without significant chief complaints and medical history was referred to our hospital for treatment of early gastric adenocarcinoma. The patient underwent endoscopic submucosal dissection (ESD) to resect the lesion. Pathological examination demonstrated complete resection of the tumor. The resected tumor was confined within the mucosal layer without lymphovascular invasion. Six months after ESD, a follow-up esophagogastroduodenoscopy (EGD) was performed and found a small polypoid lesion at the lesser curvature of the proximal stomach, which appeared to be a benign polyp Fig. a.Fig. 1ESubsequent EGD at 1 year after ESD, the polypoid lesion was clinically diagnosed as hyperplastic polyp based on its size (3 mm) and shape  or combination of laparoscopic and endoscopic approaches to neoplasia with non-exposure techniques (CLEAN-NET) were developed as a full-thickness resection without intestinal perforation . NEWS orOur report indicates that mesenchymal tumors of the stomach including leiomyosarcoma can be difficult to establish the diagnosis from conventional endoscopic biopsy specimens. LECS can be useful to establish the pathological diagnosis and simultaneously allow a curative resection of the gastric submucosal tumor.Leiomyosarcoma of the stomach is extremely rare. This is the first report of leiomyosarcoma of the stomach treated by LECS. We could also follow its appearance change through endoscopic examination for 3 years.


A 3-week-old suckling piglet spontaneously died after septicemic colibacillosis. At postmortem examination, bulging and ulcerated lesions were seen, affecting the oral mucosa on the inner surface of the lower lip. After histopathological investigation, the diagnosis of congenital oral squamous cell carcinoma was made. To the best of our knowledge, this is the first case of congenital oral squamous cell carcinoma ever described. A relationship has been shown or suggested between papillomavirus infection and oral squamous cell carcinoma in humans and animals. However, next-generation sequencing study did not demonstrate any papillomavirus sequences in the case reported herein. Lymphosarcoma, nephroblastoma, melanoma, and hepatic tumors are considered to be the most common porcine neoplasms, while other tumors are only sporadically observed in this animal species [Cancer is widely recognized as a major cause of morbidity and mortality in small companion animals . The occ species . The rel species . The preMycoplasma hyopneumoniae, and porcine reproductive and respiratory syndrome virus .The event occurred in a small farrow-to-finish pig herd located in central Italy, the breeding stock consisting of 70 sows (Landrace x Large White x Duroc) and 2 boars . All pigs were routinely vaccinated against Aujeszky's disease , according to the Italian law. In addition, sows were vaccinated against colibacillosis and clostridiosis , while piglets were vaccinated 15 days from birth against porcine circovirus type 2, Escherichia coli in pure culture from the spleen, thus supporting the diagnosis of septicemic colibacillosis.In May 2020, the herd experienced a severe episode of colibacillosis in suckling piglets, with a relevant increase of mortality. Several piglets were autopsied for diagnostic purposes. Among them, a 3-week-old piglet was examined. The animal showed a moderate body condition. At necropsy, acute enteritis, systemic enlargement and hyperaemia of lymph nodes, mild splenomegaly, pulmonary oedema, and disseminated petechiae on the visceral pleura, as well as on the external surface of the kidneys, were seen. On the basis of the postmortem pathological findings, septicemia was suspected. Bacteriological investigation yielded the isolation of Notably, bulging and ulcerated lesions were also seen, affecting the oral mucosa. The largest lesion was seen on the inner surface of the lower lip, close to the right second and third incisors , while sOn the basis of the above microscopic findings, taking into account that tumors are defined as congenital when detected in foetuses and newborns until 2 months of age , the finUnfortunately, we did not sample any other tissues or organs; thus, we cannot rule out the presence of metastases. We nonetheless remark that no microscopic evidence existed regarding the presence of neoplastic emboli inside the lymphatic and blood vessels; furthermore, lesions resembling neoplasms were not grossly observed at postmortem examination.Bacillus spp. (1718 reads) and circoviruses (360 reads), respectively, followed by less represented taxa. No papillomaviral DNA sequences were found in any of the reads.Thereafter, an aliquot of the formalin-fixed tissue sample was submitted for an in-depth biomolecular investigation, in order to confirm/rule out the presence of papillomaviruses or, more in general, oncogenic viruses. To this aim, DNA was extracted by the Maxwell® RSC DNA FFPE Kit , using the Maxwell 16 Instrument , according to the manufacturer's protocol. Genomic libraries were prepared by means of the Illumina DNA Prep  and then sequenced on the MiniSeq Instrument (Illumina Inc.), using MiniSeq Mid Output Reagent Cartridge v2 (Illumina Inc.), 300 cycles, and standard 150 bp paired-end reads. Raw reads were trimmed by Trimmomatic . MetagenPteridium aquilinum) [http://www.oie.int). More commonly, cases of posttraumatic, necrotizing stomatitis by Fusobacterium necrophorum are observed in piglets. Formerly, traumatic injuries often resulted from tooth clipping; such a procedure was widely abandoned in Italy and not performed in the pig herd studied. As above stated, we also mistakenly suspected posttraumatic lesions. However, microscopic findings leave no margin for doubt, as further confirmed by two skilled veterinary pathologists, who remained blind to signalment and case history.Oral SCCs are commonly diagnosed in cats and dogs, much less frequently in horses and sporadically in sheep . In geneuilinum) . In pigsuilinum) ; in thisTwo cases of oral SCC have been described thus far in pigs, both affecting aged pet pot-bellied pigs. The first case was observed in a 10-year-old female pig, and SCC appeared as an ulcerated mass which infiltrated the hard and soft palate and metastasized to the draining lymph node and to the lungs . The secAs reviewed in detail in a previous work, porcine congenital tumors are uncommon and mostly of mesenchymal origin. Despite their very low prevalence, some congenital tumors in pigs  have been studied thoroughly as models in comparative oncology, because of their mode of inheritance .On an interesting note, a report regarding newborn piglets in China showed a very high incidence of enzootic lingual papillomatosis (>100.000/year) . We consOverall, infectious diseases represent the most relevant issue in the field of porcine health management, leading the diagnostic approach to mostly be focused on viral and bacterial pathogens, often by means of biomolecular tools. Histopathology is not routinely carried out for diagnostic purposes in farm animals, at least in the Italian context. In spite of this, the present case report highlights that microscopic investigation is always of value to integrate with other laboratory tests, allowing for a more correct understanding of misleading postmortem findings and providing more reliable data about the prevalence and the main features of cancer in the different animal species.


Background: Intravascular fasciitis is a rare disease that is a reactive proliferative lesion of myofibroblasts. There are rare reports that intravascular fasciitis has invaded the jugular vein as seen in this case.Case Presentation: A 41-year-old female presented with right neck dull pain for 20 days. The appearance of the subcutaneous mass was oval, pink hyaline, well-demarcated, and measuring ~5 mm in diameter. Microscopically, the mass was composed of spindle cells arranged in intersecting fascicles. Immunohistochemical stains showed that the spindle cells were positive for smooth muscle actin and negative for S-100, Desmin, MyoD1, and elastin stains. The nuclei of the spindle cells were relatively uniform, and mitotic activity was observed. The overall morphological and immunohistochemical features are consistent with intravascular fasciitis.Conclusion: Due to the rapid growth and vascular invasion, intravascular fasciitis created a high risk of misdiagnosing it as a sarcoma or thrombosis. Reporting this uncommon case, we raise awareness of this non-neoplastic lesion, and careful, light microscopic examination combined with immunohistochemical staining aids in the diagnosis of intravascular fasciitis. Intravascular fasciitis (IVF) is a rare benign lesion arising from reactive proliferation of myofibroblasts inside the vascular lumen or in the superficial or deep fascia with involvement of small- and/or medium-size blood vessels. IVF often occurs in the upper extremities, head and neck, and lower extremities. Five cases involving the large vein are previously reported in the literature and are commonly misdiagnosed as venous thrombosis or malignant sarcoma. Here, we report a rare case of IVF that was located in the right internal jugular vein. Meanwhile, we review the literature on this rare disease.A 41-year-old female presented with right neck dull pain for 20 days. She denied any associated difficulty with headache, dizziness, pain, fever, or swelling. She was a nonsmoker and non-drinker and denied any drug abuse. Physical examination revealed a firm, well-defined, tender, non-mobile mass in the right neck with dimension about 10 × 10 × 10 mm. The mass did not move up or down with swallowing. Laboratory data were within normal limits. A computed tomography angiography (CTA) demonstrated the right jugular vein filling defect at the level of the cricoid cartilage–thyroid plane . The grain situ hybridization (FISH) showing rearrangement of the USP6 locus  and excluding gene amplification of the MDM2 . S100, Desmin, MyoD1, and elastin stains were negative. Fluorescent the MDM2 . Our resNodular fasciitis (NF) is a benign lesion arising in the subcutaneous tissue, skeletal muscles, and fascia. IVF is a rare variant of NF, which was first described by Patchefsky and Enzinger in 1981 . In totaIt can develop in almost any site and is most commonly encountered in the head and neck, followed by the upper extremities, lower extremities, and trunk , 18. TheMicroscopically, IVF is mainly composed of a proliferation of plump spindle cells inside the lumen or associated with the wall of arteries and veins of all sizes, arranged in short intersecting fascicles, a storiform pattern, or a unregular manner. The lesions could appear as intraluminal or extraluminal growth with or without infiltration of surrounding connective tissue and infiltrative borders with entrapment of skeletal muscle observed in some cases. The background stroma was dense or myxoid and frequently showed a well-developed, slit-like capillary network. Scattered lymphocytes, red blood cells, and inflammatory cells often were seen in the background. Multinucleated giant cells were often present. Mitosis sometimes occurred, but no abnormal mitotic figures were found.t was detected in our case. The fusion gene (MYH9-USP6) was detected in 92% of NF and found to be highly sensitive and specific, favoring a neoplastic nature  for the publication of any potentially identifiable images or data included in this article.GC and CC were wrote the manuscript and were assistant in surgery. XW was involved in editing the manuscript. BH was chief operating surgeon. 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.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.


A 63-year-old male with non-small-cell lung cancer (NSCLC) developed a tracheomediastinal fistula after endobronchial ultrasound transbronchial needle aspiration while on treatment with bevacizumab. This vascular endothelial growth factor-specific angiogenesis inhibitor is a first-line treatment for unresectable or metastatic NSCLC and has been reported to cause fatal non-gastrointestinal fistulas. Respiratory tract fistulas are a known rare complication after bevacizumab therapy characterized by a high mortality rate. Respiratory tract fistulas (RTFs) are a known rare complication of bevacizumab therapy that are characterized by a high mortality rate . Risk faA 63-year-old man with a 40-pack-year smoking history presented with superior vena cava syndrome secondary to a large right paratracheal mass Figure , 1B. He Treatment with definitive chemoradiation with carboplatin, pemetrexed, and pembrolizumab was initiated. After two cycles of chemotherapy, with a mixed response, new metastatic foci were found on imaging scans. He was referred to pulmonary medicine for repeat EBUS-TBNA of the right paratracheal mass to obtain tumor tissue for analysis of molecular markers.Following the repeat EBUS-TBNA, the patient was started on chemotherapy with carboplatin, paclitaxel, bevacizumab, and atezolizumab every three weeks. Interval imaging demonstrated an overall improvement in metastatic disease, along with a mediastinal air-filled cavity/fistula within the location of the 4R node Figure , 1D. BecBevacizumab is a humanized monoclonal antibody that targets vascular endothelial growth factor and thereby inhibits angiogenesis -3. It isThe incidence of RTFs is not well described, but it is believed to be low. However, Kanzaki et al. described that of seven patients who underwent treatment with lung resection and perioperative bevacizumab for metastatic colorectal cancer, two (29%) developed late-onset pulmonary fistulas , which sThus far, the reported bevacizumab-associated fistulas have included the following types: malignant tracheal-mediastinal-parenchymal-pleural fistula , tracheoRisk factors that have been associated with RTF formation after bevacizumab treatment include recent invasive procedures such as EBUS and surgery, damage to esophageal and respiratory tract structures after chemotherapy and/or radiation therapy, squamous cell carcinoma, central location and cavitation of the tumor -5, superAlthough the definite pathophysiology of RTF is unknown, it has been hypothesized that impaired wound healing and vessel formation secondary to bevacizumab can lead to the formation of fistulas after damage to the mucosa . Some haFistulas affecting the airway have been managed in various ways based on the patient’s individual situation . Case reIn our patient’s case, we believe that both the two cycles of bevacizumab and the chemoradiation therapy he received contributed to the formation of the fistula. The reported literature about bevacizumab-associated RTFs continues to lack detail. Therefore, it is important to analyze all patients with RTFs as a group to better understand the pathophysiology of this complication, its true incidence and prevalence, and what preventive measures should be taken. Follow-up of the resolved fistulas is needed to determine the long-term outcomes and whether any specific treatment has advantages over the others.


Primary pulmonary leiomyosarcomas (PPLs) are extremely rare tumors of the lungs. They can present with non-specific symptoms or can also be asymptomatic with clues towards diagnosis being found on routine examination or radiographs. We present a case of a 54-year-old woman who presented with worsening shortness of breath and spells of dizziness. Her chest radiographs showed right-sided pleural effusion and CT revealed a large enhancing pleural mass with compression atelectasis and mediastinal shift. She underwent a thoracoscopy and right pleural biopsy. Histopathology and immunohistochemistry were most consistent with leiomyosarcoma. An extensive search for a possible primary in other sites was unrevealing, thus diagnosing the patient with PPL. She was managed with surgery and radiotherapy. Primary sarcomas of the lungs are exceedingly rare tumors. They comprise less than 0.5% of all lung malignancies . These mA 54-year-old woman with a known past medical history of diabetes mellitus presented with worsening shortness of breath and dizzy spells for the past few days. On initial presentation, the patient had tachypnea to 29 breaths per minute, tachycardia to 110 beats per minute, hypoxia with oxygen saturation to 82 to 84% on room air, afebrile with normal blood pressure, and negative orthostatic measurements. The patient was initially resuscitated and placed on a non-rebreather with an appropriate response. The review of systems was negative, with no recent travels or sick contacts. Physical exam was notable for decreased breath sounds over the right middle and lower lung bases.Further investigation showed moderate to large right-sided pleural effusion on chest X-ray. Labs were only significant for mild high sensitivity C-reactive protein elevation and mild leukocytosis with relative lymphocytosis. Multiple differentials were raised, including both infectious and non-infectious etiologies including pneumonia, pulmonary embolism, tuberculosis, and malignancy. Computed tomography (CT) of the chest was performed, which showed a large right-sided enhancing pleural mass along the parietal pleura measuring 6.8 x 4.1 x 6.4 cm along with compression atelectasis from large pleural effusion and mediastinal shift to the left , Wilms tumor protein (WT-1), with focal positivity for smooth muscle antigen, and calretinin which highlighted the presence of some entrapped mesothelial cells. The morphology and immune profile were most consistent with leiomyosarcoma (LMS) Figure .A CT scan with contrast for extremities, abdomen, and pelvis was also performed to stage further the tumor, which was negative in pursuit of any concomitant or primary malignancy. Thus, the diagnosis of primary pulmonary leiomyosarcoma (PPL) was made with probable endobronchial origin. For multidisciplinary management, the patient was then referred to thoracic surgery, oncology, and radiation oncology.We described a case of PPL in a patient who had presented with dyspnea; the clue towards an underlying malignant pathology was apparent from the radiograph. PPL can present with a broad range of unspecific symptoms that range from cough, fever, chest pain, wheezing, hemoptysis, or thoracic symptoms due to tumor expansion into the chest cavity . HoweverPulmonary sarcomas are most often metastatic from other sites . The chaPPL may originate within the pulmonary parenchyma, within a bronchus, or within the pulmonary artery . Only thSurgical resection of the tumor is the preferred modality of treatment . A studyIn conclusion, PPLs are extremely rare tumors that may only be apparent for the first time on routine examinations or radiographs. Patients usually present with non-specific symptoms like shortness of breath, cough, hemoptysis, or because of compression of thoracic organs. Definitive diagnosis can be made by histopathology and immunohistochemistry; PPLs stain positively for markers of smooth muscle differentiation. Surgery is the favored modality of treatment. Complete resection of the tumor is associated with a good prognosis.


Pseudoaneurysm is a rare vascular complication of trauma, causing an incomplete tear of the vessel wall. We present a clinical case report arising from the distal branch of facial artery in an infant. Facial artery pseudoaneurysm is a rare complication of facial trauma and can easily be misdiagnosed especially in the paediatric age group. Prompt investigation and diagnosis with timely and apt intervention is the key to the successful management of facial artery pseudoaneurysm. Pseudoaneurysm is a rare vascular complication of trauma, causing an incomplete tear of the vessel wall . The comWe present a case of facial artery pseudoaneurysm along with its clinical, radiological findings and management. To our knowledge, this is the first reported surgically managed case of distal facial artery pseudoaneurysm in an infant.A one-year-old girl presented with a right cheek mass that had been growing for three weeks. She had fallen from her bed one month ago. Clinical examination revealed a 3x2.5 cm, oval, ill-defined, reddish-purplish colored mass involving the right cheek and extending from the medial canthus to the right malar region, causing facial disfigurement . On palpColor Doppler USG revealed a well-defined vascular cystic lesion in the right cheek highly suggestive of a pseudoaneurysm. CT images were suggestive of a large vascular mass; however, the exact origin of the lesion could not be confirmed .  The chAneurysms are classified as true, false, or dissecting. True aneurysm is a dilation of all the three layers of the intact vessel wall . False aIncomplete tear of the involved vessel wall causes blood to flow into the surrounding tissue resulting in tamponade and clot formation . HemorrhFacial artery pseudoaneurysms are rare because of the small diameter of the facial artery and its deep and protected location . Trauma,The diagnostic tool of choice is arteriography . ArterioNon-invasive treatment modalities include compression and observation, but this modality is time consuming . InvasivClassically, treatment of pseudoaneurysm has always been open surgical exploration with vessel ligation, but with the recent advancements in minimally invasive surgery, endovascular approach has become more popular. Endovascular management entails treatment aimed only at the aneurysm or the entire vessel from which the pseudoaneurysm arises . Percutaneous embolization is performed by direct injection of thrombin under ultrasound guidance. This transforms the pseudoaneurysm into hematoma, which then resorbs in due course of time . Using tIn the present case, mass was the result of a blunt trauma. Considering the superficial location of the mass, and the limited facilities for endovascular surgery in our center, surgical excision and ligation of the feeding vessels were planned. Outcome was good and no recurrence was reported in the 18-month follow-up period. The involvement of the distal facial artery makes this case all the more interesting and unique.We recommend keeping pseudoaneurysm as a differential diagnosis in palpable pulsatile swellings of the head and neck. And an algorithm based on site , presentation, availability of facilities and clinical expertise should be formulated and considered for management of this uncommon entity. Although arteriography with embolization is the preferred method, surgical resection is also a safe and effective method for the treatment of head and neck pseudoaneurysms. An open procedure can be considered in cases of superficial swellings, failure of endovascular approach, or as in our case, non-availability of endovascular surgery resources.Facial artery pseudoaneurysm is a rare complication of facial trauma. Prompt investigation and diagnosis with timely and apt intervention is the key to the successful management of facial artery pseudoaneurysm. Surgical resection is a safe and effective treatment method and can be considered in cases of superficial swellings, failure of endovascular approach, or as in our case, non-availability of endovascular surgery resources.


Sphenoid sinus aspergilloma (SSA) with visual loss has rarely been reported. Timely recognition and prompt surgical intervention are crucial to avoid permanent neurological consequences. Aspergillus spp (A 51-year-old male was assessed for sudden, non-traumatic, painless vision loss in the left eye of 4 h duration preceded by retro-orbital headache for 2 days. He had no significant past medical conditions. Examination revealed decreased visual acuity to light perception in the left eye and no other focal neurological deficits. Urgent CT brain imaging followed by MRI revealed a left sphenoid sinus mass with dehiscence of the posterior wall of the clivus and the greater wing of the sphenoid compressing the optic nerve canal suggesting aspergilloma or tumour A and 1B.llus spp . On postAspergillus fumigatus is a commonly isolated species, while headache is the main symptom Aspergilloma is rarely affecting the sphenoid sinuses and can remain indolent; thus, the diagnosis is often delayed until complications occur Ethics approval and permission was obtained to publish the case reports from the institutional review board which is in line with international standards.No funding was received towards the publication.GA: Clinical management, data acquisition and manuscript writing. MA supervised all the aspects and contributed to final manuscript editing. MP and AA: Contributed to data acquisition and histopathology reports. WG: Clinical management, contribute to data acquisition, manuscript preparation and final proof reading.Written informed consent was obtained from the patient to publish this report in accordance with the journal`s patient consent policy.The authors declare that they have no competing interests.


Pericardial synovial sarcomas are sporadic tumors. Herein, we report a case of primary pericardial synovial sarcoma originating from the right pericardium. Missed diagnosis delayed surgical treatment. Eventually, the tumor occupied the almost entire pericardial cavity. The pericardial tumor was surgically removed as soon as possible after admission. In this paper, we aim to provide details that can help further understand the differing symptoms and presentations of pericardial synovial sarcoma and highlight the importance of consideration of this disease in similar cases where the etiology of pericardial effusion is unknown. Pericardial synovial sarcoma (PSS) is a rare malignant tumor. Thus far, only a few cases of PSS have been reported. Synovial sarcoma (SS) is also reported in some uncommon sites such as the pleura, heart, kidney, lung, prostate, liver, gastrointestinal tract, and peripheral nerves. The diagnosis of SS in these unusual locations is challenging and requires additional diagnostic procedures such as immunohistochemistry, electron microscopy, and molecular genetic techniques for confirmation of the diagnosis. The case cardiac tamponade presented as the initial symptom, but no obvious evidence supported the diagnosis of pericardial synovial sarcoma. After 5 months, the anterior mediastinal tumor was found using transthoracic echocardiography. Non-random occurrence of tx;18) was consistently found in SS. In some cases, the only cytogenetic abnormality noted is the t, suggesting that this is a key molecular event in tumor development 8 was con.Mycobacterium tuberculosis  revealed a large amount of pericardial effusion, pleural effusion, left lung swelling, and right lower lung swelling . The patrculosis . The patrculosis . He was rculosis . Five movia a median sternum incision. The size of the mass was about 20 × 15 × 19 cm  at our hospital indicated that the right anterior mediastinum mass had compressed the right heart  and the  × 19 cm . The mas × 19 cm ; further × 19 cm . It was  × 19 cm . Thus, fThe incidence of heart tumors is quite hidden, and most of them are caused by accidentally discovered cardiac lumps. These occasional phenomena often means thrombus or neoplasms, which usually occur in specific clinical environments . The incSS is an uncommon malignant tumor mainly seen in children and adolescents; its 10-year overall survival rate is about 0–20% , 7. The The morbidity rate of young people with PSS is relatively high, and the most common site of the disease is in the pericardium . Even inThe reason for the initially missed diagnosis in our case was the absence of tumor cells and related markers in the pleural fluid. Besides, the patient's symptoms improved significantly after symptomatic treatment. However, the initial beginning stage of the tumor can be seen on the CT performed initially. After 5 months, the tumor had grown up, leading to aggravation of the patient's condition. It was visible on echocardiography and compressed the right heart and the root of the great artery.As regards the treatment of PSS, on the one hand, due to the invasiveness and expansibility of the tumor, it is generally impossible to remove the tumor completely. On the other hand, there is no systemic study on this topic due to extremely low incidence of the disease; thus, no optimal treatment strategy has yet been proposed. Besides surgical resection, radiotherapy and chemotherapy are also used; this, however, depends on the clinician's discretion and the patient's condition. In general, surgical resection should be performed as soon as possible once the tumor diagnosis is confirmed, especially when the possibility of malignancy is high. Resection can be performed based on the preoperative MRI evaluation, with the premise of negative margin. Because positive margin is an independent risk factor for cardiac tumors, we recommend MRI before surgery to evaluate the degree of disease and assess whether it can be resected on the premise of negative margin. If this is not possible, second-line radiotherapy and chemotherapy can be considered; evaluation can then be performed and resection undertaken after the tumor has shrunk in size until it can be resected with a negative margin . ReportsP = 0.029). The survival rate at 12 months was 96.5% (±3.8 SE) in patients treated with chemoradiotherapy (CRT), while it was 21.9% (±10.8 SE) in those not treated with CRT (Log-Rank test: P ≤ 0.0001) , while that following incomplete resection was 55.0% (± 11.5SE) (Log-Rank test:  0.0001) . Thus, i 0.0001) –22. Putn 0.0001) . The age 0.0001)  this fin 0.0001) –27. The Herein, we describe a case of a patient with PSS. The missed diagnosis highlights the necessity and difficulty of differential diagnosis in patients with such tumors. Histopathology is key to the diagnosis of PSS. In our case, no abnormality in the pericardial effusion was noted, and our patient presented with atypical clinical symptoms. Although primary PSS is not common, when the etiological diagnosis of pericardial effusion is unknown, further tests need to be carried out to help rule out or confirm the diagnosis of PSS.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.YL, ZT, and LX contributed to conception and design of the study. YL completed the data collection and wrote the first draft of the manuscript. YL, KG, TX, HG, RL, SH, and LW wrote sections of the manuscript. All authors contributed to manuscript revision, read, and approved the submitted version.This study was supported by the National Science Foundation for Young Scientists of China (8150020951) and the Natural Science Foundation for Young Scientists of Hunan Province (2016JJ4099).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.


Interstitial obstruction in newborn infants can be caused by several factors such as malrotation, meconium plug syndrome, meconium ileus, Hirschsprung's disease, atresia and stenosis. Neonates who have been diagnosed with an interstitial obstruction are in need of immediate treatment; otherwise, they can deteriorate rapidly. Surgery remains the mainstay of treatment in most cases. Pediatric gastrointestinal tumours are very rare, especially in newborn infants. Their management is usually different as compared to adults.We present the case of a newborn infant who was born with interstitial obstruction. At the 31 weeks scan, a significant dilation of the small bowel was observed and the diagnosis of interstitial obstruction was made. When born, the newborn was transferred to a specialised unit and underwent a laparotomy. The findings were consistent with a tumour causing the obstruction; the histology reported this tumour as benign lymphoid hyperplasia.Pseudolymphoma is a very rare cause of fetal interstitial obstruction, and it should be considered in the differential diagnosis. The gastrointestinal tract is divided into two segments: the small intestine and the large intestine, known as the colon. The small intestine is made up of three parts: the duodenum (which is connected to the stomach), the jejunum and the ileum.An interstitial obstruction in a newborn infant can be caused due to several conditions such as atresia and stenosis, annular pancreas, volvulus (twisting of the bowel on itself), duplication cyst, meconium ileus, meconium plug syndrome, Hirschprung’s disease, neoplasia, trauma and some other rare causes such as necrotising enterocolitis . NeonateA fetal bowel obstruction is usually discovered in two ways. A routine scan during gestation  may show that a certain segment of the bowel is abnormal, which could be either dilated or larger than normal. While in the uterus, the fetus swallows amniotic fluid. Therefore, a narrowing of the gastrointestinal system could slow or even stop the flow of the amniotic fluid, causing swelling of the bowel, which will make the bowel appear large in the scan. In this case, the normal movements the bowel makes become exaggerated as the bowel tries to propel fluid through the blockage. These movements, also called peristalsis, can be seen over several seconds with ultrasound. A bowel obstruction could also be discovered when polyhydramnios is present in a scan. Because of the obstruction, the normal flow of the amniotic fluid will be stopped. Subsequently, the amniotic fluid stays outside the fetus and inside the uterus. The uterus can be enlarged due to the high amount of amniotic fluid. If the fetus is diagnosed with an interstitial obstruction, a thorough evaluation is significant. This evaluation may include an ultrasound, fetal echocardiogram and amniocentesis.The management of an intestinal obstruction will almost always be surgical. With the advent of neonatal intensive care and multidisciplinary care, morbidity and mortality rates are low. Tumours of the pediatric or neonatal gastrointestinal tract are extremely rare. Their infrequent presentation at treatment centres has not allowed for the development of standardized treatment protocols and prospective review .A 32-year-old Caucasian pregnant woman  presented at the antenatal department due to reduced fetal movements the day before. She was 31 weeks and four days pregnant after spontaneous conception. This was her second pregnancy. During this pregnancy, she had a 12 weeks screening and a 20 weeks scan. Both of these screenings were reassuring. Her first pregnancy was three years ago with normal delivery. The first baby was a girl who weighed 3210 grams.Initially, she had a cardiotocography (CTG), which was reassuring. The baseline was around 130 bpm with accelerations while no contractions were recorded. Afterwards, she had a scan, which was done by a consultant specialised in fetal medicine. During this scan, significant small bowel dilation was observed and the diagnosis of interstitial obstruction was made , duodenal atresia, malrotation (with or without midgut volvulus) or jejunal atresia. Low Interstitial obstruction is most commonly attributable to ileal atresia, meconium ileus, Hirschsprung’s disease, small left colon syndrome, colonic atresia or imperforate anus . HoweverBenign lymphoma, known as pseudolymphoma or benign lymphoid hyperplasia, is a condition that has symptoms similar to malignant lymphomas. Benign tumours are lumps or growths that may appear in several parts of the body. In the case of benign lymphoma, these sites are usually the skin, lungs, gastrointestinal tract, soft tissues and many other sites. The reasons for the development of a benign lymphoma are not completely understood but it is associated with genetics, radiation exposure, inflammation or infections. About one in every 2,000 people worldwide suffer from benign lymphomas, which are rarely life-threatening. Pseudo lymphoma in children is extremely rare, especially in neonates, since this is one of the few cases that have been described so far. In most cases, benign lymphoma is not severe. However, it may cause problems when the swollen lymph nodes start to press into surrounding tissues, or in even more severe cases, it could evolve into a malignant lymphoma .Although fetal interstitial obstruction is a relatively common diagnosis that most fetal medicine doctors will have to deal with, a tumour as a causative factor is not very common. Especially, pseudolymphoma tumours are extremely rare. Therefore, it should be included in the differential diagnosis, and it should be discussed with the neonatal team so that better treatment is planned for the newborn infant.


A 19-year-old male presented with a history of pulsatile tinnitus on the left side after he underwent drainage for a tonsillar swelling. He had noticed a painless neck swelling one month ago that was associated with difficulty in swallowing. His physician had made a diagnosis of peritonsillar abscess, and drainage had been performed. After the procedure, the swelling had progressed further, leading to drooling of saliva and disabling tinnitus. When he presented to the emergency, local examination revealed a pulsatile neck swelling with peritonsillar mass. CT angiography/digital subtraction angiography (DSA) of the neck and brain revealed a large aneurysm of the distal cervical segment of the left internal carotid artery (ICA). The patient successfully underwent an urgent open repair. This case highlights the importance of a thorough history and examination in identifying a rare cause of pulsatile tinnitus. A stepwise approach to determine the reason behind tinnitus revealed an underlying ICA aneurysm of the extracranial segment. Pulsatile tinnitus can have a vascular or non-vascular etiology. The most common vascular causes include a high jugular bulb, glomus tumor, cerebral aneurysm of the internal carotid and vertebral-basilar arteries, and dural arteriovenous fistula . The nonA 19-year-old man with no past medical and surgical history presented to the emergency department with a history of a ringing sensation in the ear for the last seven days. The patient had a history of painless swelling in the neck as well as oral swelling on the left side with difficulty in speech for one month. He had consulted a local practitioner, where he had been diagnosed as a case of peritonsillar abscess, and drainage had been done. Since then, he had developed a ringing sensation in the left ear, which was pulsatile, followed by hoarseness of voice and difficulty in turning the neck towards the left side. There was no preceding history of fever, headache, vomiting, trauma, or drug intake, which could be responsible for pulsatile tinnitus. On physical examination, the patient had a pulsatile swelling on the left side of the neck, no bruit on auscultation, and no local rise in temperature. Oral examination showed an erythematous swelling in the left peritonsillar area. Central nervous system examination showed that uvula was deviated to the right side with absent gag reflex on the left side, weakness of the left sternocleidomastoid and trapezius muscle, and deviation of the tongue towards the left side . The cardiovascular examination did not reveal any murmur or added sound on auscultation. Normal blood pressure was noted and was equal in both upper limbs. Ear examination revealed normal external ears and a shiny tympanic membrane on both sides with no redness or pulsation. Rinne’s, Weber’s tests, and an audiogram were suggestive of sensorineural hearing loss on the left side; however, this was unnoticed by the patient.Hematological and biochemical investigations were within the normal range. Following discussion with the on-call neurology team, a CT angiography of the neck and brain was performed, which revealed aneurysmal dilatation of distal cervical segment of left ICA measuring 3.2 x 4.4 x 5.0 cm with the lateral part of it opacified by contrast (1.3 x 2.1 x 3.0 cm) and the rest of the non-enhancing region suggestive of thrombus. The aneurysm was causing partial effacement of the airway. A digital subtraction angiography (DSA) showed a large partially thrombosed wide-necked cervical ICA aneurysm . The patient was discharged on postoperative day 12.While assessing a patient with pulsatile tinnitus in the emergency department, thorough history and clinical examination can help to understand the likely pathology and narrow down the differential diagnosis. The medical history includes duration and severity of the tinnitus, history of trauma, associated auditory symptoms of hearing loss, vertigo, and neurologic symptoms of headache, visual disturbances, vomiting, and cranial nerve involvement. The clinical evaluation requires an otologic examination and a detailed neurological examination in addition to a local examination. Auscultation of the chest, neck, and skull should be performed to look for cardiac murmurs, cervical bruits, and objective tinnitus. Our patient had pulsatile tinnitus, lower cranial nerve deficit, a neck mass without bruit, and absence of ear mass on otologic examination. CT angiography revealed an aneurysm in the distal cervical segment of the ICA, which was partially non-enhancing due to the presence of the thrombus.The most common site of extracranial ICA aneurysm is common carotid artery bifurcation and proximal ICA. The causes of ICA aneurysm include atherosclerosis, fibromuscular dysplasia, trauma , iatrogenic lesions, infections, congenital defects, and irradiation arteritis ,4. In thIn our case, neck swelling was noticed about a month ago with the progressive increase in size. On further evaluation, we could not find any clue regarding traumatic or vasculitic etiology for this aneurysm. The patient may have been having a congenital swelling that was too small and had escaped his attention until recently.The clinical presentations of extracranial ICA aneurysms vary from being asymptomatic and incidentally diagnosed to pulsatile mass in the neck, transient ischemic attacks, stroke, and cranial nerve involvement. Major complications involve compression of neurovascular structures, embolization, rupture, or ischemia . The posAneurysms of the cerebral internal carotid or the vertebral arteries are a rare cause of pulsatile tinnitus. Pulsatile tinnitus as the sole manifestation of an ICA aneurysm in the paraclinoid portion has been reported . Still, In this index case, the peritonsillar swelling was not evaluated as an extension of neck swelling but was mistaken for peritonsillar abscess and drained. Iatrogenic trauma led to pseudoaneurysm formation with thrombus, causing a rapid increase in size, and the resulting pressure effects caused disabling tinnitus with gradual paralysis of multiple cranial nerves. There was no bruit over the swelling; however, an absence of bruit does not rule out vascular etiology as it can be thrombosed. These procedures can be life-threatening for some patients in the absence of prompt evaluation and treatment.An aneurysmal swelling mistaken for peritonsillar abscess can lead to iatrogenic trauma resulting in serious complications like neurological deficits and thrombus formation. A prompt diagnosis requires detailed history and examination. Among the plethora of neurological manifestations, tinnitus may be the presenting manifestation of extracranial ICA aneurysm in rare cases. Thus, pulsatile tinnitus may be a warning sign for suspecting a potentially serious underlying disease, and a high index of suspicion is required to avoid iatrogenic disasters.


Cyclopia with a proboscis, a rare congenital anomaly, and a severe form of holoprosencephaly occur as a result of incomplete separation of prosencephalon into two halves of hemispheres during organogenesis. A prenatal anomaly scan can help in the early detection of the condition and timely termination of the pregnancy. Cyclopia with a proboscis, a rare congenital anomaly, and a severe form of holoprosencephaly occur as a result of incomplete separation of prosencephalon into two halves of hemispheres during organogenesis. A prenatal anomaly scan can help in the early detection of the condition and timely termination of the pregnancy. There was a spontaneous expulsion of a single dead female fetus of 1.25 kg with a placenta weighing 150 grams with cyclopia with a proboscis. Also, the baby had a dysmorphic face with a single eye and the nose was absent in normal position; however, a nonfunctioning nose was present above the single eye , and other vitals stable. On abdominal examination, the uterus was of 32‐week size, relaxed; fetal parts were palpable but no fetal heart rate was detected. On vaginal examination, cervical os was 1 cm dilated, soft posterior, 10% effaced, and the amniotic membrane was intact. All her laboratory investigations were within normal limits. Ultrasonography showed a singleton pregnancy of approximately 293A cyclopia with proboscis is a rare form of HPE with few more cases reported worldwide.Alobar holoprosencephaly, as reported in this case, represents the most severe form and constitutes undifferentiated cerebral hemispheres with monoventricles, a fusion of thalamus, and other cranial midline abnormalities.A high prevalence of chromosomal abnormalities (mainly trisomy 13) and female predominance in HPE is found. The baby with this abnormality generally gets naturally aborted or is stillborn on delivery.Sonography is the most helpful in the prenatal diagnosis of cyclopia, and in most of the reported cases, the anomaly has been recognized early in the anomaly scan. This early diagnosis by fetal ultrasonography allows for timely termination of pregnancy and avoids maternal psychological trauma of giving birth to a deformed fetus.Holoprosencephaly is managed supportively along with other associated malformations treatment. Prognosis depends on the degree of fusion and malformation of the brain and other associated complications. Alobar and semilobar HPE have the worst prognosis and are often incompatible with life. However, with lobar HPE, a child can survive for some years with neurological and mental challenges.This report emphasizes the need for awareness and education about the importance of antenatal checkups and anomalous effects of alcohol to all women of reproductive age groups, particularly in developing countries like Nepal. Our patient was thoroughly counseled about the disease condition and encouraged to take necessary steps in cessation of the alcohol intake and avoid various risk factors.4Cyclopia is a rare congenital anomaly. The importance of an anomaly scan in the timely detection of anomalous babies and preventing mothers from the psychological trauma of carrying such fetus is to be stressed upon to decrease the suffering of parents and family members.None to declare.AK, PP, BMS, and SS drafted the manuscript. AK, PP, and SR were involved in editing and revising the manuscript. All the authors read and approved the final version of the manuscript.Written informed consent was obtained from the patient and her husband for publication of this case report and images of the baby. A copy of the written consent is available for review by the Editor‐in‐Chief of this journal.


Neurenteric cysts (NCs) are rare benign endodermal lesions of the central nervous system (CNS), most commonly found in the spinal cord. Intracranial lesions are rare, among which the posterior fossa appears to be the predominant location.We present a case of a 60-year-old man who presented with a suddenly decreased level of consciousness. After a series of radiological studies were done, a multilobulated cystic lesion in the right posterior fossa was observed. Surgical resection was performed and based on its histopathological characteristics, NC diagnosis was confirmed.Because of the wide list of differential diagnoses and low specificity of radiological features, surgical gross total resection remains the most effective treatment, followed by diagnosis confirmation through histopathological techniques. Neurenteric cysts (NCs) are rare benign endodermal lesions of the central nervous system (CNS) defined as "cysts lined by mucin-secreting epithelium resembling that of the gastrointestinal tract," according to the 1979 World Health Organization (WHO) Classification of Tumours of the Central Nervous System -6. Due tA 60-year-old man with a history of intermittent headaches presented to the emergency room due to a suddenly decreased level of consciousness, with a Glasgow Coma Scale (GCS) score of 12 . A computed tomography (CT) scan revealed a right posterior fossa hypodense mass with complete fourth ventricle obliteration, causing acute hydrocephalus (Evans' index of 0.4)  and braiThe patient was admitted to the operation room, where a ventriculoperitoneal shunt was installed uneventfully. Complete recovery of mental functions was observed after shunt placement.A complete neurological examination revealed normal mental status, cranial nerve function, strength, and sensation, followed by magnetic resonance imaging (MRI), which in the T1-weighted sequence showed an intra-axial, multilobulated, hypointense mass dorsal to the right pontomedullary junction. This mass did not enhance after gadolinium administration and appeared to be extending into the fourth ventricle, causing partial expansion of this region. Contrary to the typical hyperintense descriptions, the cyst was hypointense on the fluid-attenuated inversion recovery (FLAIR) sequence Figures -2D. WithHistological examination of the surgical specimen was compatible with the diagnosis of an NC, which, by light microscopy, resembled the gastrointestinal tract appearance with a single layer of pseudostratified cuboidal or columnar epithelium and intermingled goblet cells, exhibiting as well positive periodic acid-Schiff (PAS) staining. Immunostaining reported NC findings, such as cytokeratin 7 (ck7) and epithelial membrane antigen (EMA) positivity, and glial fibrillary acid protein (GFAP) negativity Figures -3D 9,10,109,10.Intracranial NCs represent 10-17.9% of all NCs and occur more often in the adult population. On the other hand, intraspinal NCs represent the most common location, which is more common in pediatrics . Due to In 1976, Wilkins et al. proposed the first classification, which is still in use, based on histological features . As intrThe imaging characteristics of NCs are variable. CT scan reveals a hypodense lesion with no contrast enhancement in most cases. Occasionally, iso and hyperdense lesions are observed, supposing a diagnostic challenge . Thus, MSurgical resection remains the mainstay of treatment in symptomatic patients. An adequate surgical exposure to achieve complete visualization of the interface between the tumor and the brainstem becomes the primary objective during surgery. For lesions located ventral to the brainstem, a far-lateral transcondylar approach has been used, with good postoperative outcomes ,18. We pIntracranial NCs are rare benign lesions that typically occur in young adults and should always be considered a differential diagnosis of cystic, intra-axial lesions of the CNS. An accurate preoperative diagnosis might not always be possible, as radiological features are not specific. When feasible, gross total resection is the most effective treatment method. Pathological examination and immunostaining must be performed to reach a correct diagnosis. Follow-up with MRI is advised for at least 10 years.


Patient was declined for surgery because of the high operative risk. Alternatively, treatment with a warfarin derivate was initiated. Follow-up echocardiography showed a gradual decrease of the echogenic structure size over time  requires urgent intervention. In general, surgery is the preferred treatment for symptomatic BPVT. However, fibrinolysis or oral anticoagulants should be considered in high–surgical risk patients.Video 1 Echogenic mass attached to mitral bioprosthesisVideo 2 Gradual decrease of echogenic mass under oral anticoagulant therapyVideo 3 Gradual decrease of echogenic mass under oral anticoagulant therapy


Appendectomy remains one of the most common emergency operations. Recent research supports the treatment of uncomplicated appendicitis with antibiotics alone. While nonoperative management of appendicitis may be safe in some patients, it may result in missed neoplasms. We present a case of acute appendicitis where the final pathology resulted in a diagnosis of a Burkitt-type lymphoma.An 18-year-old male presented to the emergency department with 24 h of right lower quadrant pain with associated urinary retention, anorexia, and malaise. Past medical history was significant for intermittent diarrhea and anal fissure. He exhibited focal right lower quadrant tenderness. Workup revealed leukocytosis and CT uncovered acute appendicitis with periappendiceal abscess and no appendicolith. Laparoscopic appendectomy was performed and found acute appendicitis with associated abscess abutting the rectum and bladder. Pathology of the resected appendix reported acute appendicitis with evidence of Burkitt-type lymphoma. A PET scan did not reveal any residual disease. Hematology/oncology was consulted and chemotherapy was initiated with an excellent response.Appendiceal lymphomas constitute less than 0.1% of gastrointestinal lymphomas. Primary appendix neoplasms are found in 0.5–1.0% of appendectomy specimens following acute appendicitis. In this case, appendectomy allowed for prompt identification and treatment of an aggressive, rapidly fatal lymphoma resulting in complete remission. While acute appendicitis is a common pathological entity, it is a surgical emergency. The lifetime risk of developing appendicitis ranges from 7 to 8%, with the median age of diagnosis between 10 and 11 years of age . While aEffective management of appendicitis is a topic of debate, with considerable options for operative and nonoperative approaches. Nonoperative management has become increasingly popular in recent years. A recent study demonstrated 4.9% of cases in 2014 were managed nonoperatively, which represents a 4.7% increase per year since 1998 .Our patient was an 18-year-old male who presented to the emergency department with a complaint of approximately 24 h of pelvic pain. The symptoms were worsening and associated with urinary retention, nausea, and anorexia. The pain was exacerbated by movement. He reported similar symptoms approximately 1 month prior, with a negative workup by his primary care physician at that time. Past medical history was significant for intermittent loose stools, anal fissures, and a prior cyst in his neck. Surgical history included a colonoscopy as part of a workup for hematochezia several years earlier, which diagnosed an anal fissure. Excision of a reportedly benign neck cyst was also completed previously; however, no pathology was available.On presentation, he was a well-appearing young adult male in mild distress. He was afebrile, and his vital signs were within normal limits, without evidence of tachycardia, tachypnea, or hypotension. His abdomen was soft but tender to palpation in the right lower quadrant, without evidence of generalized peritonitis. Lab values were insignificant except for leukocytosis WBC 17.4 k) and mild hyperglycemia (blood glucose level 124 mg/dl). Computed tomography imaging obtained prior to the surgical consult demonstrated dilation of the appendix up to 11 mm with periappendiceal fluid  was initiated and the patient brought to the operating room and placed under general anesthesia for the procedure. Operative findings included purulent fluid throughout the peritoneal cavity with the appendix laying down in the pelvis. Appendiceal inflammation with obvious perforation to the appendiceal tip and adjacent abscess was noted. The patient’s postoperative course was significant for persistent nausea for 12 h after the procedure, which was treated with antiemetics and resolved. He was discharged to home on postoperative day #1 with a plan to complete a 10-day course of oral amoxicillin/clavulanic acid (Augmentin).Several hours after his discharge, an urgent call was received from the pathologist reviewing the appendix specimen. Surprisingly, the appendix demonstrated a high grade transmural lymphoid malignancy with extra-appendiceal deposits Figs.  and 4. TGiven the Burkitt-type features and the potentially rapid progression of a Burkitt lymphoma, the decision was made to treat as a confirmed Burkitt lymphoma. The patient promptly underwent PET scanning, lumbar puncture, and port placement. PET imaging revealed no evidence of distant disease. He also had sperm banking performed to preserve the potential for future fertility. After sperm banking, the patient underwent a total of four cycles of cyclophosphamide, vincristine, doxorubicin, high-dose methotrexate, ifosfamide, etoposide, high-dose cytarabine (CODOX-M/IVAC) chemotherapy with an excellent response. Now more than 30 months out from treatment, he remains in complete remission.The gastrointestinal tract is the most common extranodal sites of lymphoma metastases, accounting for 5–20% of all cases . PrimaryInterestingly, patients with Burkitt lymphoma of the appendix presented at an earlier age (33 years) compared with follicular lymphoma (59 years) and diffuse large B-cell lymphoma (53 years), which is consistent with our case involving a young white male patient. The mortality rate of primary appendiceal lymphoma is promising, as the 5-year survival rate was found to be 67% in the cohort studied. Similarly, the 5-year survivor rate of Burkitt lymphoma/leukemia is 87% in ages 0–19 and 60% in those 20–39 . The cliCT Imaging is a vital tool for diagnosis of appendiceal neoplasms by demonstrating the disproportionately increased diameter of the appendix shown on CT-scans (> 3 cm). In contrast, non-tumoral appendicitis does not have as large a diameter. CT Imaging is also critical in assessing the extent of tumor burden throughout the body . However. Skendelas et al. found a 0.53% incidence of appendiceal malignancies in NSQIP database patients who had undergone appendectomy for appendicitis from 2010 to 2018, and 1.7% incidence in their local patients who had surgery at two hospitals in The Bronx, New York City [Timely appendectomy, either open or laparoscopic, has been the recommended treatment of choice for acute appendicitis due to recent advances in perioperative management, which has lowered rates of wound infection, shortened hospital stays, and significantly decreased morbidity . Howeverork City . Finallyork City .While new research has provided evidence for antibiotics alone as treatment for acute appendicitis, we believe it will lead to missed or delayed diagnoses of primary appendix neoplasms. Our patient discussed above presented with acute appendicitis, he was treated with appendectomy and was diagnosed with Burkitt-type lymphoma of the appendix on pathology. Due to the early diagnosis, this patient was able to be rapidly and effectively treated resulting in a complete remission and a significant improvement in his prognosis. Had he been treated with antibiotics alone, even if it was successful in the control of his appendicitis, the chances of a poor outcome from a later diagnosis of Burkitt lymphoma is very high. This potential risk needs to be considered when examining the risks and benefits of operative versus nonoperative management of acute appendicitis.