Computed tomography (CT) and Magnetic Resonance Imaging (MRI) scans showed the enhancing left temporal lesion and the right frontal lesion in the right superior frontal gyrus . In FebrAfter 1 year from the first operation, due to stable disease, a second surgery was performed in order to remove the right non-enhancing frontal lesion. The histological diagnosis was IDH1-mutant diffuse astrocytoma , macroscopically removed as the following CT and MRI scans documented. No chemotherapy or radiotherapy was administered after the second surgery.In July 2017, a new MRI scan documented a relapse of the lesion in the temporal lobe, and in August 2017 the surgical excision of the recurrent tumor was performed. A second-line chemotherapy with fotemustine was administered until January 2018 when a new relapse was documented by a brain MRI. On March 2018, a new surgical removal was performed, using 5-ALA fluorescence. The histological examination confirmed the diagnosis of recurrent GBM IDH wt. After surgery, a third-line treatment with regorafenib and subsequently, fourth-line therapy with metronomic temozolomide (TMZ) were administered. The residual component of the lesion was increased dimensionally on the last MRI scan, performed in June 2018, and the patient died in August 2018.4 Briefly, the lesion in the left temporal lobe was diagnosed as GBM IDH wt (grade IV WHO 2016) at the histological exam, whereas the concomitant lesion in the superior frontal gyrus was diagnosed as diffuse astrocytoma carrying IDH1 mutation.4 Only the GBM lesion, analyzed by fluorescence-in situ-hybridization, carried monosomy of chromosome 10 and homozygous deletion of 9p21 (cyclin-dependent kinase inhibitor 2A gene [CDKN2A]).4 Also, the expression of DNA mismatch repair (MMR) proteins was investigated, showing that both lesions were MMR proficient. Furthermore, microsatellite instability was detected in both lesions, that also carried a Telomerase Reverse Transcriptase (TERT) promoter mutation (228C>T).Both GBM and LGG lesions were characterized molecularly, as previously described.+/IL4Rα +) and MDSC-4 (CD14+/HLA-DRlow/−), polymorphonuclear (PMN)-MDSC-2 (CD15+/IL4Rα +), and early stage MDSC-3 (Lin−/HLA-DR−/CD33+/CD11b+).6Blood and the four clinical specimens were analyzed at each surgery as shown in + myeloid infiltrate, in which 96% of cells were MG , and a lower amount of MG cells , but both lesions did not change the composition of the immune infiltrate, which was constituted mainly by CD33 GBM-RII , panel B8 To evaluate their role in sustaining BMDM infiltration in low- and high-grade lesions, we sorted CD45− cells from GBM, GBM-RI, and LGG lesions and assessed by quantitative real-time reverse transcription-polymerase chain reaction (qRT-PCR) the expression levels of these cytokines. As shown in Among the factors driving myeloid cells infiltration, chemoattractant CCL2 and CSF1/M-CSF1 play a relevant role.3 So far, the simultaneous presence of low-grade and high-grade gliomas has never been explored from this point of view. Results from this unique case recapitulate the findings observed on a large number of cases, and indicate that when a simultaneous high- and low-grade lesions are present, each tumor microenvironment dictates the recruitment of the inflammatory cells. We found that CCL2, but not CSF-1 produced by tumor cells is highly expressed by high-grade lesions, thus highlighting the role of this chemokine in the recruitment of suppressive BMDMs. However, the integrity of the blood–brain barrier (BBB) may also play a role. In fact, the BBB is disrupted during GBM progression, and shows heterogeneous permeability, as evidenced by contrast-enhanced MRI, the gold standard method for assessing BBB dysfunction.10 The case that we present in this manuscript is in line with these observations, as GBM lesions show contrast enhancement, while the LGG lesion does not, after contrast medium injection.In our previous work, we observed that tumor grading in gliomas influences the recruitment of BMDM, since LGG has a significant lower amount of these suppressive macrophages.The compromised integrity of the BBB may facilitate the passage of the monocytes, and the consequent accumulation and differentiation into suppressive BMDMs. Thus, the presence of a large myeloid infiltrate in high-grade gliomas suggests an active passage of immune cells, driven by chemoattractant CCL2 and by a leaky barrier. In the simultaneous low-grade lesion, instead, the lack of contrast enhancement suggests the presence of an intact BBB, which, coupled to low or absent presence of CCL2, may explain the absence of suppressive BMDM in the tumor microenvironment.Our results indicate that the high-grade lesions  show the typical presence and proportion of macrophages with a prevalence of recruited BMDM. The same holds true for the low-grade lesion, in which macrophages are represented almost exclusively by MG cells, as observed in patients in which a single lesion is present, indicating that the local recruitment of myeloid cells in each lesion is not influenced by the presence of the other lesion.Results learned from this unique case stress the notion that blood-derived immune suppressive macrophages are a hallmark of high-grade gliomas, that their persistent accumulation in recurrent lesions, despite therapies, is an obstacle that must be overcome to find new efficient treatments, and finally that a rational therapeutic approach to GBM should include the targeting not only of BMDMs, but also of circulating myeloid cells, and of the soluble factors driving their accumulation.This work was supported by Italian Association for Cancer Research (AIRC) (IG2015-17400 to S.M.), TRANSCAN-2, ERA-NET to S.M. and Università degli Studi di Padova (CPDA144873/14 to S.M.).Conflict of interest statement. None declared.S.M. and A.D.P designed the study and supervised the work. S.M. wrote the manuscript and handled funding. L.P., E.M., M.V. performed experiments and analyzed the data. G.L. provided clinical information and discussed the data. A.D.P. performed neurosurgery. All authors have read and approved the final version of the manuscript.


Paraneoplastic cerebellar degeneration (PCD) is a rare neuroimmunological disease that may accompany tumors. In this article, we present a patient with progressive gait difficulty who was diagnosed with PCD and, in a rare comorbidity, with papillary thyroid carcinoma (PTC) following malignancy screening. A 46-year-old male patient reported having experienced poor balance for 2 years. A neurological examination revealed nystagmus, intention tremor, and ataxia, and anti-thyroid peroxidase and anti-thyroglobulin levels were found to be elevated. A brain MRI showed significant cerebellar atrophy in the superior vermis. Malignancy screening for PCD was performed, and thyroid ultrasonography revealed a nodule in the left lobe, while PET/CT detected elevated focal F-18 fluorodeoxyglucose uptake in the thyroid. Onconeuronal antibodies  were negative. Pathologic examination of the thyroid revealed PTC, for which the patient was treated with 0.4 g/kg intravenous immunoglobulin and referred to the medical oncology department. This case demonstrates that clinicians must be alert to the rare comorbidity of PCD and PTC. Paraneoplastic neurological syndromes (PNSs) are diseases associated with malignancy that involve the nervous system , which is affected by the immune system through different immunological pathways. PNSs are not triggered by direct tumor invasion, metastasis, or the side effects of treatment. Paraneoplastic cerebellar degeneration (PCD) is a very rare neuroimmunological PNS that may accompany tumors and is often associated with humoral factors that are secreted by tumor cells or with an immune response to tumor cells. It is mostly associated with gynecologic tumors, lung cancer, and Hodgkin’s lymphoma. Acute or subacute onset, moderate or severe truncal ataxia, nystagmus, vertigo, dysarthria, and diplopia may occur , and neuIn this article, we present a patient with progressive gait and balance difficulties who was diagnosed with PCD and a rare comorbid papillary thyroid carcinoma (PTC) following a malignancy screening.A 46-year-old male patient presented with a gait disorder and reported having experienced poor balance for 2 years. The patient had no known systemic diseases, was a smoker, and his family medical history included no similar abnormalities. Neurological examination revealed horizontal nystagmus, intention tremor, and ataxic gait, and his score on the Scale for the Assessment and Rating of Ataxia (SARA) was 24/40 . His routine hemogram and biochemical tests were normal, and, while thyroid function tests were normal, anti-thyroid peroxidase and anti-thyroglobulin levels were elevated. The patient’s cerebral MRI showed a significant bilateral cerebellar atrophy in the superior vermis . ElectroMalignancy screening  for PCD was performed; thyroid ultrasonography revealed a solitary hypoechoic nodule of 11 × 9 mm in the left lobe isthmus junction, and PET/CT detected elevated focal F-18 fluorodeoxyglucose uptake in the left lobe of the thyroid (standardized uptake volume (SUV) max: 7.22; late SUV max: 6.42) . CerebroThe patient was then treated with 0.4 g/kg intravenous immunoglobulin for 10 days, following which partial improvement of the ataxia was detected by a SARA score of 20/40 . The patient was then referred to the Department of Medical Oncology, and radioactive iodine treatment was started by nuclear medicine during follow-up. When permanent hypothyroidism occurred, thyroid replacement therapy was also started. The patient’s SARA score was still 20/40 after 6 months of follow-up.In cancer patients, tumor invasion, the effects of metastatic lesions on tissues, infections, and treatment-related side effects are well known. However, PNSs—which are not related to the tumor itself or to its metastasis, but which are dependent on the presence of the tumor—are becoming more frequently recognized. Several hypotheses regarding the pathogenesis and pathophysiology of PNSs have been proposed, but those based on hormones, peptides, and long-/short-acting signaling molecules being produced by tumor cells or through immune reactions with tumor-related antigens are most widely accepted . IncreasPCD is a rare PNS; while cerebellar degeneration is acute or subacute at onset due to diffuse Purkinje cell death, it progresses with a rapidly progressing PNS. There are two reported cases with PCD and PTC, which is a rare association in literature. One of the cases, featuring a 57-year-old female patient, was reported by Kroiss et al. . In our The symptoms of PCD can be variable. Moderate-to-severe truncal ataxia, nystagmus, vertigo, dysarthria, and diplopia may be observed ; in our MRI examination of PCD may be normal or reveal severe cerebellar atrophy—one study reported that 75% of patients with PCD had normal MRI findings —and radiPCD is often associated with gynecologic tumors, breast cancer, lung cancers, and Hodgkin’s lymphoma ,11,12. WAntineuronal antibodies associated with PCD are anti-Yo, anti-Tr, anti-Hu, anti-Ma2, and anti-Ri, and the most common and best described syndrome associated with anti-Yo is PCD. Neurological diseases have been reported to progress rapidly in rare antibody-negative PCD cases , and thePCD usually has a poor prognosis, but rapid and effective treatment can prevent the progression of symptoms. Tumor therapy has been reported to be more beneficial than immunotherapy in stopping neurological progression ; in a foAs in our case, patients with no known tumor diagnosis may present with the clinical symptoms and signs of PCD. The presence of a tumor can be shown through etiological investigation of PCD. Thus, the earlier a tumor is detected, the higher the likelihood of tumor and PCD treatment. Clinicians should be alert to patients with progressive gait disorder as a PCD symptom, which can be comorbid with PTC.


Duodenal neuroendocrine tumor (d-NET) is a rare tumor originating in the neuroendocrine system. The clinical manifestations of d-NET are similar to those of other digestive tract tumors, resulting in a lack of specificity and complex clinical symptoms.A 55-year-old female patient was admitted to our hospital with a chief complaint of an abdominal mass that had been present for more than 4 months.The upper abdomen enhanced computed tomography scan showed an uneven density mass across the upper abdomen, and the tumor size was approximately 6.2 × 5.8 cm with obvious visible enhancement present in 1 area and a cystic nonenhanced area. The postoperative pathology showed the tumor cells to be positive for chromogranin, synaptophysin, cytokeratin, CD56 , negative for vimentin, CD117, DOG-1, CD34, S-100, SMA, desmin, and Ki-67 approximately 2%, which confirmed the diagnosis of d-NETs.We preferred laparoscopic surgical exploration, but the tumor started at the ascending part of the duodenum and involved the mesenteric artery. As the branches of the superior mesenteric artery were intertwined with the tumor, it was difficult to operate with the endoscope, so we converted to open laparotomy. The postoperative pathology revealed the presence of d-NET.The patient recovered uneventfully and was discharged after the operation. One-month and 3-month follow-up after surgery, showed no evidence of recurrence.Radiological imaging studies are insufficient for the differential diagnosis of abdominal mass from other diseases, whereas surgery is the only radical treatment method, and the preferred surgical method is still active radical resection of the tumor. NET is a rare disease, its less than 1% in all malignant tumors, but can be present in many organs and tissues. Gastrointestinal neuroendocrine tumor is a rare type of special low-grade malignant tumor, that can occur in any part of the digestive tract, such as the stomach and rectum, accounting for about 0.4% to 1.8% of all gastrointestinal malignancies.22.1The patient, a 55-year-old female, was admitted in the hospital on July 5, 2019, because of the discovery of an abdominal mass that had been present for more than 4 months. Approximately 4 months prior, the patient inadvertently found a hard upper abdomen mass but reported no associated pain. The patient self-reported the tumor to be about the size of an egg but reported no nausea, vomiting, abdominal pain, diarrhea, haematemesis, blood in the stool or other discomfort. A local hospital computed tomography (CT) enhancement scan revealed a solid cystic mass in the upper abdomen, and the initial segment of the jejunum was unclear, suggesting the possibility of a stromal tumor. The patient was physically healthy. The examination upon admission revealed a soft abdomen that allowed the abdominal mass to be palpated. The mass was hard and appeared to contact the upper left abdominal area, and the size was approximately 5 × 6 × 5 cm. The patient's level of activity was good, and there was no tenderness. After admission, the relevant examinations were completed, and no obvious abnormalities were found in the laboratory tests, which included routine blood tests, biochemistry tests, and tumor marker analyses.2.2The upper abdomen enhanced CT showed the following: an abnormally dense shadow was present across the upper abdomen, but the side was clear, and the tumor size was approximately 6.2 × 5.8 cm. There was an uneven density within the lesion, with obvious visible enhancement present in 1 area and a cystic nonenhanced area. The lesion was closely related to the intestinal tract of the small intestine (beginning approximately at the jejunum), and a small arterial branch was seen inside. There were no obviously enlarged lymph nodes beyond the retroperitoneum +, synaptophysin (Syn)+, cytokeratin +, vimentin–, CD56-, β-catenin membrane+, CD10–, PR–, CD117–, CD34–, CD7+, CD20–, CR–, and Ki-67 positive rate <1%  is a type of tumor that originates from stem cells and has neuroendocrine markers that produce bioactive amine or peptide hormones that cause different clinical syndromes, for example, carcinoid syndrome, has a wide range of malignant potential. The prognosis of this disease may be related to the pathological type, staging, endocrine components, postoperative adjuvant therapy, and other factors. Lymph node metastasis and distant metastasis are independent risk factors for poor prognosis of NETs. In short, d-NETs has an insidious onset, and clinical symptoms are not specific, especially in the early stage. In addition, there are no obvious symptoms, and the symptoms are not taken seriously. After progression, patients may have symptoms such as upper abdominal pain, fatigue, weight loss, digestive tract bleeding, abdominal mass, and anemia. At this time, the opportunity for radical surgery is often lost, so early diagnosis is particularly important. Thorough preoperative examination, detailed surgical evaluation, and early surgical treatment can avoid missed diagnoses and misdiagnoses of this disease. Postoperative adjuvant therapy based on pathological staging and immunohistochemistry can prolong the survival time of patients. At present, surgery is the only radical treatment method, and the preferred surgical method is still active radical resection of the tumor. After follow-up, the patient had a good prognosis and no tumor recurrence or metastasis. It has been reported that patients with d-NETs with tumors less than 1 cm without lymph node metastasis can undergo endoscopic local excision and have a better therapeutic effect.,15 At present, the pathogenesis of d-NETs is not completely clear, and further research is needed. Clinical treatment usually involves surgical resection , biological therapy, and targeted therapy in the early and middle stages. Regular physical examination, including gastroscopy, can improve the detection of d-NETs, diagnose the tumor stage early and predict prognosis. Ultimately, the appropriate treatment can improve the patient's 5-year survival rate.There are few reports on the diagnosis and treatment of d-NETs, and d-NETs generally have good differentiation, slow growth, and good prognosis. However, some tumors display the opposite characteristics.Funding acquisition: Yanbin Wu.Investigation: Xuefeng Cao.Supervision: Xingyuan Zhang.Writing – original draft: Xuewen Wang.Writing – review and editing: Yu Cheng, Lingqun Kong.


Schistosoma japonicum, with multiple pseudotumoral lesions presented with seizures and hemiparesis respectively, and a spinal cord conus medullaris schistosomiasis due to Schistosoma mansoni presented with conus medullaris syndrome.We describe three cases of neuroschistosomiasis, two cases with cerebral schistosomiasis due to In the two cases with cerebral schistosomiasis imaging with CT revealed multiple areas of brain edema, and gyriform calcifications in both cerebral hemispheres, which suggested cerebral parasitemia, chronic venous hypertension, multifocal cerebral vascular malformation, or a forme fruste Sturge Weber syndrome. Further MRI revealed corresponding blooming, T2W (weighted) -FLAIR (fluid attenuated inversion recovery) ibright signal intensity and enhancing lesions. In the third case with spinal cord involvement MRI revealed signal abnormality on T1W and T2W images with patchy and punctate post i.v. contrast enhancement of the conus medullaris.Excision biopsy and histopathological examination were undertaken for the first brain case and spinal cord case and showed multiple schistosomal granulomas in different evolution phases. In the second brain case, the diagnosis was suggested based on our experience with prior cases, positive laboratory tests, and urinary bladder wall biopsy.Neuroschistosomiasis must be considered in the differential diagnosis of multiple cerebral calcifications, and multiple nodular and linear like lesions with characteristic arborized enhancement pattern, especially for patients coming from endemic areas for Schistosomiasis. Schistosoma are capable of causing disease in humans: S. mansoni, S. japonicum, S. haematobium, S. intercalatum and S. mekongi.1 Schistosomiasis affects about 200 million individuals worldwide and are endemic in Brazil, Suriname, Venezuela, several of the Caribbean Islands, equatorial west and center-south of Africa, and Egypt.2 Yet, because of current easiness of locomotion and migration, schistosomal infection became more frequently found in non-endemic countries.3,4Schistosomiasis is a parasitic infection that uses aquatic clams as intermediate hosts, the male and other mammals as definitive hosts. Five species of S. mansoni, and cerebral schistosomiasis (CS) being most frequently affected by S. japonicum. Two forms of neuroschistosomiasis have been described, the tumoral  and diffuse pseudotumoral forms. The corresponding imaging findings of the pseudotumoral form include a single or multilobar cerebral involvement, with rare cerebellar, basal ganglia, and mid-brain involvement, the diameter of these lesions range from <1 cm to about 7 cm, with CT parenchymal calcification, and MRI long T1 and T2 signals, prominent post-contrast enahancement, and surrounding vasogenic edema and mass effect. The diffuse pseudotumoral form comprises multiple discrete 3 mm intensely enhancing nodules clustered together in a large mass.1,5–7Neuroschistosomiasis with involvement of the central nervous system (CNS) include the brain and spinal cord, with spinal cord being most frequentlyaffected by A 25-year-old male Filipino patient came to the A&E department complaining of seizures, his laboratory blood tests were normal. Non-contrast CT scan of the head showed multiple bilateral cerebral cortical and subcortical hyperdense linear and nodular lesions, consistent with parenchymal calcifications, surrounded by hypodense vasogenic edema, involving the left frontal, left occipital and bilateral parietal lobes .MRI was then undertaken for better characterization of the parenchymal abnormalities and showed multiple lesions with linear and nodular post i.v. contrast enhancement surrounded by areas of vasogenic edema .Schistosoma japonicum ova with absence of protruding spine.Excision brain biopsy was done, and pathological examination of the specimen showed granuloma formation around characteristic A 25-year-old Filipino lady, presented to the emergency department complaining of a 1 day history of left-sided lower limb weakness causing walking difficulty, associated with moderate headache radiating to the back of the neck, but otherwise no other associated symptoms.She was admitted to the hospital, where she had tonic clonic seizures followed by confusion, headache, and vomiting. Her past medical history was unremarkable.Plain CT head showed left frontal parasagittal cortical swelling with mild increased density and associated subcortical vasogenic edema . MRI scaPlain CT scan of the chest, abdomen, and pelvis (not shown) was also undertaken as a workup of possible malignancy with brain metastasis. And showed nodular urinary bladder wall and calcified capsular liver lesions suggestive of chronic schistosomiasis. The patient denied any urinary or gastrointestinal symptoms, but she gave history that she used to play and drink from local river back home in her childhood.Schistosoma antibody (Ab) titer was positive at 1:160. And urinary bladder wall biopsy was consistent with schistosomiasis. Given the above imaging and clinical background the diagnosis of neuroschistosomiasis of the brain was entertained, and treatment for schistosomiasis was started, and she received a single dose 40 mg/kg Praziquantel, and she was discharged on oral levetiracetam (Keppra) and prednisolone for seizure control.On 2 weeks clinical follow-up visit, the patient showed dramatic clinical improvement, and follow-up MRI showed significant resolution of the enhancing brain lesions, and decreased vasogenic edema .T2W image MRI, and thick nodular and linear enhancement on post i.v. contrast T1W image MRI  confined to cerebral areas, has no definite lobe predilection. With more clinically significant NS include involvement of the spinal cord and cerebellum..9The clinical syndrome of spinal NS is usually an acute or subacute myelopathy, with or without polyradiculitis, causing lumbosacral and lower limbs pain, paraparesis or paraplegia, bowel, and bladder sphincter dysfunction, and sensory, sexual and reflexic disturbances.9Whereas brain involvement may lead to headaches, nausea and vomiting, papilledema, seizures, or motor deficits according to the location of the granuloma.10CT in patients with brain schistosomiasis, show single or multiple variably enhancing hyperattenuated lesions with surrounding hypoattenuated oedema may be present.T1 W MRI reveals a central linear enhancement surrounded by multiple enhancing punctate and nodular lesions, forming a characteristic “arborized” appearance. Pathologically, this enhancement pattern has been correlated with a host granulomatous response to Schistosomal ova. This pattern of enhancement though characteristic of brain schistosomiasis, is not present in all cases, but when observed the diagnosis of CNS schistosomiasis should be strongly considered.11Contrast-enhanced T1W hyperintense signal of the globus pallidus and also, in substantia nigra in case of hepatic schistosomiasis japonicum even in the absence of liver dysfunction believed to be related to manganese deposition. NS findings in children and young adults with hepatosplenic schistosomiasis also include T2W cerebral white matter hyperintensity with rare corpus callosum involvementat.10Additional findings related to liver dysfunction and portosystemic shunt may be seen as bilateral symmetric In our two brain cases, CT showed multifocal and multilobar involvement, which was bilateral in the first case, and unilateral in the second case, cerebral cortical and subcortical hyperdense linear and nodular lesions, consistent with parenchymal calcifications, surrounded by hypodense vasogenic edema. MRI was then undertaken for better characterization of the parenchymal abnormalities and showed multiple lesions with linear and nodular enhancement surrounded by areas of vasogenic edema .6 described multiple brain lesions with a unique multinodular appearance in two cases of cerebral schistomiasis, which were almost similar to our first two cases. In the first case in this study, cranial CT showed multiple hyperdense lesions with surrounding hypodensity, and post-contrast enhancement in the left temporal lobe, thalamus, and pons, in the next case cranial CT showed a hypodense lesion surrounding a right parieto-occipital area of diffuse parenchymal calcifications, brain MRI showed the multinodular aspect of these lesions with surrounding vasogenic edema and mass effect and revealed an additional lesion in the left cerebellum in the next case.Thaigo et al,Júnior et al in another study9 described a similar multinodular tumefactive lesion in the left temporal lobe, which showed remarkable regression on follow-up MRI 3 months after treatment with praziquantel.Sanelli et al11 described two cases of cerebral schistosomiasis involving the right anterior parietal lobe in one and the left temporal lobe in another, both appeared as mass-like lesions, where the temporal lobe lesion showed typical (arborized) linear and nodular enhancement similar to our first two cases, which we suggest can be likened to palm tree and leaves .Neuroschistosomiasis has a characteristic appearance on CT and MRI which should in the proper clinical context lead to further laboratory confirmation and obviate the need for an invasive surgical biopsy.Early 2 weeks post anti-schistosomal treatment imaging assessment and subsequent imaging follow up at 3 months can further confirm the diagnosis by showing adequate treatment response.


To report an unusual case of oral hairy polyp (HP) and review the literature, providing epidemiological, clinical and histopathological information on this disease. A 12-year-old male patient was referred to a Stomatology department with a nodule in the posterior midline of the tongue. The patient did not know exactly when it arose or whether it had grown since then. Clinical exam revealed a bulky and mobile pedunculated mass lesion on the dorsum of the tongue, with a diameter of approximately 1 cm. The patient’s mother reported no previous health problem. An excisional biopsy was performed, the surgical specimen was sent for anatomopathological analysis, and the findings were compatible with the diagnosis of HP. Hairy polyp is a rare lesion, especially in the oral region. The literature search revealed only 10 case reports of oral HP published between January 1999 and January 2019, and they revealed a predominance of the disease in female newborns. Two uncommon facts were presented in this case: the patient was male and diagnosis was made at 12 years old. Congenital tumors of the oral cavity are rare manifestations, often diagnosed in the first years of life. One of these is hairy polyp (HP), a benign lesion composed of pedunculated masses of mesodermal and ectodermal origin, usually from the nasopharynx or oropharynx, and covered with skin with sebaceous glands and hair.,Clinical manifestations of HP depend on its location and size, but commonly cause respiratory symptoms and feeding difficulties.The present paper is a case report of a 12-year-old male patient with a HP in the oral cavity, whose diagnosis was confirmed by anatomopathological analysis of the surgical specimen. It also presents a literature review of the common characteristics of this lesion.The patient reported was escorted to the Stomatology Department, where clinical examination and biopsy were performed. The surgical material was sent to the pathology service where it was entirely and routinely processed for paraffin embedding and staining with hematoxylin and eosin (H&E). The case report was approved by the institutional review board.The 12-year-old male patient was referred to the Stomatology Department because he noticed a nodule in the posterior midline of the tongue during self-examination, although he did not know exactly when it arose or whether it had grown since then. Clinical exam revealed a bulky and mobile pedunculated mass lesion on the dorsum of the tongue, with a diameter of approximately 1 cm, displaced to the posterior part of the oral cavity, in close contact with the oropharyngeal region, with color and texture similar to the adjacent mucosa. The patient’s mother reported that he had never had any previous health problem.th, 2017 .The lesion was excised under local anesthesia without complications. The surgical specimen was sent for anatomopathological analysis supervised by an oral pathologist. Microscopic analysis revealed a polypoid lesion covered with keratinized stratified squamous epithelium, containing indentations along its entire length, which suggested the formation of skin adnexa and the presence of a variety of tissues, including seromucinous salivary glands, cartilage tissue, lymphoid hyperplasia, muscle and adipose tissue. All tissues were mature and had no degree of atypia . The hisA literature review was carried out in order to survey case reports published between January 1999 and January 2019 that focused only on the oral region. Thus, articles that presented case reports of HPs in the nasopharynx and oropharynx regions were not considered. Only cases of bigerminal lesions  that were similar to the classic description of HP/dermoid cyst presented in Arnold’s classificationThe search was performed in databases PubMed, Centro Latino-Americano e do Caribe de Informação em Ciências da Saúde (BIREME) and Scientific Electronic Library Online (SciELO) by pairing the descriptors “hairy polyp” and “dermoid cyst” with “mouth” and “oral”, using the “and” connector.From the total of 1,661 articles found in the initial search, two different authors independently selected the original publications in English that presented case reports of human HP in the oral cavity, excluding duplicates. In addition, the bibliographic references were checked in order that any reports not found in the initial search which fit the inclusion criteria could be added. Disagreements between evaluators were resolved by a third investigator during the selection process.A total of nine articles were included in this review. The selection process and search results are described in the flowchart in Two authors independently extracted and assessed the following information: surname of the first author, gender and age of the patient, location of the lesion, symptoms, histopathological and other findings and associated complications. When incompatible information was extracted by the two authors, a third author carried out the analysis to ensure data integrity.,,,,,,,Cases of HP are rare, and lesions are usually located in the nasopharynx or soft palate and identified in the first years of life.,,,,,,,The present study reports an unusual case of oral HP in a 12-year-old male patient, located on the posterior dorsum of the tongue, one of the rarest sites of occurrence of this type of lesion.,,,,It is worth noting that most lesions are single and unilateral,,,,,,,Although HPs are considered as rare lesions, especially in the oral cavity, they are the most common congenital benign tumors of the naso-oropharyngeal region.,No theory has explained the exact origin of HP, but some authors suggest that it is related to malformations of the first and second branchial arches, Eustachian tube and/or middle ear.,,,,The classification of HPs varies due to the difficulties to define the lesions. The differential diagnosis of HP includes benign teratoma, dermoid cyst, and choristoma.,,The histopathological analysis revealed mesodermal and ectodermal histologic structures that are usually found in HP lesions, corroborating literature reports:,,,HPs have no malignant potential and are treated by total excision of the lesion.Knowledge on HPs is relevant due to the fact that they are rare lesions, especially in the oral region. It is vital to exclude other diagnostic hypotheses, as well as to prevent respiratory distress and other complications.


Ciliated muconodular papillary tumor (CMPT) is a rare papillary nodule tumor with benign and malignant characteristics that occurs in the peripheral lung.A 70-year-old woman who underwent right hemicolectomy for colorectal cancer  2 years prior, presented with a sub-centimeter growing cavity shadow on chest computed tomography (CT), which was suspected to be a CRC metastasis. Because positron emission tomography CT suggested there was no other site suspicious of recurrence, thoracoscopic resection with preoperative pleural dye marking was planned to remove the small lesion, which seemed to be hardly palpable on CT. Immediately after pleural dye marking adjacent to the lesion using cone beam CT in the hybrid operating room, thoracoscopic wedge resection was performed and the tumor was finally diagnosed as CMPT, characterized by the papillary growth of mucus-producing cells in the alveoli.We resected the non-palpable small lung lesions following preoperative marking using cone-beam CT in the hybrid operating room. This case highlights a rare cavitary CT image of a CMPT mimicking a metastatic lung tumor from colorectal cancer. Ciliated muconodular papillary tumor (CMPT) is a rare peripheral lung tumor, characterized by the papillary growth of ciliated columnar, mucous, and basal cells. Our case was unusual because a growing cavitary lesion detected by computed tomography (CT) initially led us to suspect a colorectal cancer (CRC) metastasis.A 70-year-old woman with a 45-year smoking history, who had undergone colectomy for CRC . 2 years prior, presented with a CT nodule shadow in her right lower pulmonary lobe that had grown from 0.2 cm at her right hemicolectomy  by bronchoscopy was performed at a point around 5 mm cranial to the tumor. Preoperative marking took 6 min with two CT inspections Fig. a, b. Int mL by brSince CMPT was first reported in 2002 by Ishikawa et al., only 60 cases have been reported and it has not been categorized by the World Health Organization , 2 Tabl. CMPT reOur case presenting with a growing cavity shadow was radiographically suspected to be CRC metastasis, because necrotic components, known as dirty necrosis, which suggest a colorectal origin, were considered to be drained through the airway . HoweverRegarding optimal resection, in a thoracoscopic setting without any preoperative marking, accurate localization of the 6-mm tumor located apart from the pleura seemed to be difficult , 9. GeneCMPT can present as GGO, nodules, or (rarely) cavitary formation with irregular wall thickness mimicking a metastasis. Its pathology is not clearly defined, and it may have benign or malignant properties, depending on the molecular alterations. Although no recurrence or metastasis has been reported, CMPT should be resected with sufficient margins.


Prompt and accurate diagnostic evaluation of the nontraumatic acute abdomen in the emergency department (ED) is crucial to lessen mortality burden. In patients with perforated viscus and pneumoperitoneum, point-of-care ultrasound (POCUS) can assist the emergency physician (EP) in the rapid bedside diagnosis. This report describes a case in which EP-performed POCUS led to the early detection and timely management of an atypical presentation of pneumoperitoneum. The critical management of a patient presenting with acute abdominal pain in the emergency department (ED) necessitates rapid diagnostic evaluation, especially given the substantial morbidity- and mortality-associated sequela of these clinical conditions ,2. GastrA 52-year-old male with no reported past medical or surgical history presented to the ED with three days of insidious, mild-severity generalized vague abdominal pain with associated nausea, nonbloody nonbilious vomiting, and decreased appetite. He denied fevers, chills, diarrhea, constipation, dark or bloody stools, flank pain, back pain, testicular pain or swelling, genital lesions or discharge, urinary complaints, or any other associated symptoms. Upon arrival, the patient was afebrile, with a blood pressure of 144/102 mmHg, heart rate 114 beats per minute, respiratory rate 16 breaths per minute, and oxygen saturation of 95% on room air. On physical examination, he was in mild distress and had mild diffuse abdominal tenderness to palpation without rebound, guarding, or peritoneal signs. Of note, examination was unremarkable for scleral icterus, flank or costovertebral angle tenderness, or clinical signs of significant dehydration. His clinical presentation was relatively nonspecific. However, there was concern for an acute abdominal pathology due to his clinical history and examination. CT imaging of the abdomen/pelvis, laboratory studies, pain medications, intravenous (IV) fluids, blood cultures and broad-spectrum IV antibiotics were ordered. POCUS was subsequently performed by an ultrasound fellowship-trained EP attending and emergency medicine resident physicians. POCUS assessment of the right hypochondrium with a curvilinear transducer in the sagittal plane demonstrated sonographic findings consistent with pneumoperitoneum; focal enhancement of the peritoneal stripe between the liver and abdominal wall and associated reverberation artifacts were appreciated Figure  Video 11.POCUS assessment of the bladder demonstrated sonographic evidence of air accumulation, with multiple hyperechoic foci in the anterior bladder wall and associated reverberation artifacts and/or dirty posterior shadowing Figure  Video 22. Of not3/μL and a venous lactate of 2.0 mmol/L. Portable chest radiography was then performed due to the abnormal POCUS findings, which confirmed the presence of intra-abdominal free air on the right consistent with bowel perforation Figure . GeneralCT of the abdomen/pelvis was obtained en route to the operating room, which ultimately demonstrated intraperitoneal free air, an air-fluid level in the bladder suggesting prior instrumentation or fistula, and sigmoid diverticulitis complicated by multiple abscess collections secondary to sigmoid colon perforation Figures -7. The pPrompt diagnosis can expedite the emergent management of pneumoperitoneum secondary to perforated viscus. EPs can harness the multiple advantages of POCUS in scenarios of acute abdominal pain, chiefly for the rapid primary evaluation and necessary re-assessments depending on the clinical presentation. The conventional practice of initially obtaining plain radiographs for pneumoperitoneum can be of limited diagnostic value in the ED, while multiple studies have highlighted the superior diagnostic sensitivity of ultrasonography compared to plain radiography -17. The EP training in advanced POCUS for gastrointestinal pathologies is of critical importance . In our Perforated viscus with associated pneumoperitoneum is a life-threatening etiology of acute abdominal pain in the ED. EP-performed POCUS provides an invaluable tool to rapidly diagnose pneumoperitoneum and expedite surgical consultation. Further studies are required to determine the most effective role(s) and timing of POCUS in ED evaluation of pneumoperitoneum in both atraumatic and traumatic clinical situations.


Mesenteric cystic lymphangiomas are rare benign lesions of the abdominal cavity characterized by lymphatic vessels malformation with an unknown etiology. Despite the silent clinical course of mesenteric cystic lymphangiomas, they are considered as clinically tricky lesions with an immense spectrum of presentations.We present a case of abdominal mesenteric cystic lymphangioma in a 1-year 9-month-old female patient, who complained of fever and abdominal pain for 10 days duration. Laboratory investigations, abdominal X-ray, ultrasonography, computed tomography and histopathological examination were all used to establish the diagnosis.A trial of true-cut biopsies performed by an interventional radiologist was not informative, so a multidisciplinary team decision was made to excise the mass. Intraoperative findings include multiloculated fused cystic lesion (8.0 × 5.0 × 4.0 cm) on the descending mesocolon. Histopathological examination revealed the diagnosis of a mesenteric cystic lymphangioma. The postoperative period was not complicated.Mesenteric cystic lymphangiomas are mostly asymptomatic in nature, yet predisposed to life threating events. Surgical excision is the modality of treatment characterized by low recurrence rate and a non-complicated postoperative period. Around 2A 1-year 9-month-old female patient with non-significant past medical or surgical history, a full-term product of a non-complicated vaginal delivery with no neonatal intensive care unit admissions was referred to our center for further investigations regarding the diagnosis of an intraabdominal mass versus an abdominal abscess. The patient complained of abdominal pain and fever for 10 days duration. The pain was located in the epigastric area radiating to left upper quadrant, and associated with nausea, vomiting and anorexia. The pain was severe enough to awaken the patient from her sleep. The fever was intermittent in nature with maximum recorded value of 39 °C orally, responded well to paracetamol. Upon physical examination, an epigastric tenderness was observed associated with left upper quadrant guarding to palpation. Otherwise, the abdomen was soft and lax. The suspected mass could not be palpated.Laboratory investigations , abdominB and C). No evidence of malignancy was observed.A Multidisciplinary team decision was made to excise the mass by the pediatric surgery team. Intraoperative findings include : multiloFig. 4Postoperatively, a nasogastric (NG) tube was inserted, and the patient was on the following medication: vancomycin , piperacillin/tazobactam , diclofenac suppositories  and paracetamol (pro re nata). Medications were stopped at day 5 and the NG tube was removed at day 3 postoperatively. The patient was doing well, vitally stable, passed flatus on day 2, passed stool at day 4 and discharged after 5 days postoperatively. The patient was followed 1 week after the surgery in an outpatient clinic, the wound was healthy, not infected with normal bowel motion and no fever spikes, abdominal pain or abdominal distention.3Mesenteric cystic lymphangiomas (MCLs) are clinically challenging abdominal lesions with a wide spectrum of clinical presentations ranges from an asymptomatic incidentally discovered finding to the case of acute abdomen. MCLs have been previously reported to mimic appendicitis with right lower quadrant pain associated with fever and leukocytosis . In addiThe differential diagnosis of MCL include mesenteric cysts, tuberculosis, tumor metastasis, hydatid disease, bowel adenocarcinomas, and other rare mesenteric malignancies . The mosThe benign asymptomatic nature of MCLs are predisposed to bleeding, torsion and even rupture, leading to life-threatening emergencies which requires an early surgical excision to avoid such complications . The vag4We yet report another case of MCL in a 1-year 9-mounth old female patient complained of fever and abdominal pain for 10 days duration. Although, mesenteric cystic lymphangiomas are rare but should be included in the differential diagnosis of abdominal pain especially in pediatric age group. Early surgical resection is highly endorsed even if it is asymptomatic to avoid many serious complications.The authors report no declarations of interest.This case report was not funded.The presented case was reported according to the ongoing regulations of case reports and case series in the King Abdullah University Hospital (KAUH) as stated by the institutional review board committee. Case reports are exempt from ethical approvals in our center.Written informed consent was obtained from the patient’s parents 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.Acquisition of data: Abdulraheem AK, Al Sharie AH, Shalakhti MH, Alayoub SY.Analysis and interpretation of data: Abdulraheem AK, Al Sharie AH, Shalakhti MH, Alayoub SY, Al-Domaidat HM, El-Qawasmeh AE.Drafting of manuscript: Abdulraheem AK, Al Sharie AH, Shalakhti MH, Alayoub SY, Al-Domaidat HM, El-Qawasmeh AE.Critical revision: Abdulraheem AK, Al Sharie AH, Shalakhti MH, Alayoub SY, Al-Domaidat HM, El-Qawasmeh AE.Not applicable.Ahmad K. Abdulraheem and Saleh Y. Alayoub.Not commissioned, externally peer reviewed.


Presence of two primary malignancies is rare and occurs in 3-5% of the cancer patients. As per our extensive internet research, this is the only reported case of a synchronous sino-nasal embryonal rhabdomyosarcoma with squamous cell carcinoma-tongue. The case report is important because of the rare diagnosis and the challenge we faced in the diagnosis and treatment of the patient because of the paucity of literature available on management adult rhabdomyosarcoma.We present a very rare case of an adult male with a sino-nasal mass diagnosed to be an embryonal type rhabdomyosarcoma. The patient also had a moderately differentiated squamous cell carcinoma-tongue for the past 8 months. Radiological investigations were done to see the extent of the sino-nasal mass and the extent of tongue lesion, which was seen to be involving the base of the tongue. The patient was referred for chemoradiotherapy but succumbed to the disease after 2 weeks of treatment.Occurrence of rhabdomyosarcoma in synchronous malignancies is extremely rare as the most common first as well as second primary malignancy in a diagnosed case of head and neck cancer is squamous cell carcinoma. A multidisciplinary approach to the treatment of adult rhabdomyosarcoma has been recommended. The combined use of chemoradiotherapy and surgery has improved treatment in the recent past but RMS in adults is still a rare head and neck tumour that carries a poor prognosis despite aggressive therapy. Presence of two primary malignancies is rare and occurs in 3-5 % of the cancer patients. In a case of head and neck cancer, the second primary tumour mostly develops in the head and neck region . In headA 34-year-old male presented with a complaint of left nasal cavity mass, nasal obstruction for the past 6 months . The patient also had a tongue ulcer on the left lateral aspect of the tongue for the past 8 months. The patient was already a diagnosed case of carcinoma tongue at the previous hospital 6 months back. The histopathology was moderately differentiated squamous cell carcinoma. The patient neglected tongue ulcer and did not take any treatment. There was no history of any substance abuse .On examination, the oral cavity showed an ulcerative lesion over the left lateral border of the tongue (anterior two-thirds) of the size 3x1 cm. On palpation, ulcer base was indurated with induration extending posteriorly to involve left side tongue base. On nasal examination, widening of the nasal bridge along with left-sided periorbital swelling was present, more so in the region of the left medial canthus.Reddish polypoidal mass was seen protruding from the left nostril which was covered with necrotic slough. The septum was pushed towards the opposite side. Contrast-enhanced computed tomography (CECT) of the nose and paranasal sinus showed heterogenous mass with mild enhancement completely filling the left nasal cavity with opacification of the left maxillary sinus and anteriorly extending out of the nasal cavity. Medially, the mass was causing bowing of the nasal septum towards the right. Postero-superiorly, it was extending to the sphenoid sinus and causing widening of the on the left spheno-ethmoidal recess. Erosion of lamina papyracea was also seen on the left side Magnetic resonance imaging (MRI) face showed an irregular heterogeneously hypointense lobulated soft tissue mass (95 mm x31mm x48mm) in the left nasal cavity extending up to the left ethmoid sinuses superiorly, medial canthus of the left orbit antero-superiorly and into the left half of nasopharynx through left choana with associated mucosal thickening of the left maxillary and bilateral frontal sinuses. An enhancing lesion involving the base of tongue was also seen. .Biopsy from nasal cavity mass showed elongated spindle-shaped cells with features of embryonal rhabdomyosarcoma . The biopsy sample was also subjected to immunohistochemistry with myogenin marker and the diagnosis was confirmed.Owing to the pathology and the extent of the sino-nasal tumour, the case was discussed with the Department of Radiotherapy and a multimodality treatment was planned. The patient was referred for chemoradiotherapy (CRT) initially with surgical excision of the nasal mass later on kept as an option if required post-chemoradiotherapy. Unfortunately, the patient succumbed to the disease after 2 weeks of chemoradiotherapy due to the advanced stage of the disease and poor health.Second primary malignancy (SPM) represents the leading cause of mortality in head and neck squamous cell carcinomas (HNSCC), responsible for approximately one-third of HNSCC deaths that is three times the deaths due to distant metastasis . Hong et al in 1990, based on the original criteria given by Warren and Gates in 1932, proposed the criteria for the definition of the second primary malignancy (SPM): the tumours have to be histologically certified as malignant; if they are of identical histological type, it must be an interval of at least three years between the two malignancies and/or presence of a distance of at least 2 cm of mucosa unchanged between the index tumour and the second primary tumour; it has to exclude the possibility that the second tumour to be the metastasis of the index tumor  which was unresectable made CRT a better option. Also, the paucity of literature available on the management of synchronous malignancies, one of which is a rhabdomyosarcoma posed difficulty in deciding the management protocol. The combined use of chemoradiotherapy and surgery has improved treatment in the recent past but RMS in adults is still a rare head and neck tumour that carries a poor prognosis despite aggressive therapy.


Teaching Point: Intestinal lymphangectasia should be evoked in the rare context of protein-losing enteropathy with low-attenuation thickening of the bowel wall. A 54-year-old female was hospitalized for severe hyponatremia and electrolytic troubles. She had increasing fatigue, loss of appetite and lower limbs edema. Abdominal contrast-enhanced computed tomography (CT) revealed circumferential parietal thickening of the proximal duodenum, with low attenuation of the submucosal layer Figure , arrows.There was no fat infiltration nor vessel hypertrophy suggestive of inflammatory bowel disease. Endoscopic exploration showed mucosal ulcerative lesion and the biopsy revealed focal lymphangiectasias in the submucosal layer. Clinical presentation associated with biological and histological findings suggested the diagnosis of protein-losing enteropathy (PLE). Human serum albumin scintigraphy (Tc99m) confirmed the diagnosis and showed loss of tracker in the duodenum.Eight years later, a repeat CT showed extension of the low-attenuation thickening to the whole duodenum Figure , arrows,The radiological features and its localization highly suggested the diagnosis of cystic lymphangioma. Final diagnosis was primary intestinal lymphangiectasia, also known as Waldmann’s disease.Waldmann’s disease or primary intestinal lymphangiectasia is usually diagnosed in children, but few cases have been reported in adults. It is characterized by lymphatic dilatation and results in excessive loss of proteins into the intestinal lumen leading to lower limbs edemas, diarrhea, and ascites. Findings of lymphangiectasia on CT include bowel wall thickening with hypoattenuating submucosa, corresponding to dilated lymphatic channels . DevelopLymphangiectasia is a difficult diagnosis on imaging but should be evoked in presence of other lymphatic abnormalities, especially in the context of protein-losing enteropathy.


Neuraxial anesthesia is preferred over general anesthesia for cesarean delivery when appropriate because the latter is associated with a higher incidence of airway complications and an increased need for neonatal resuscitation. Common complications of neuraxial anesthesia include backache and headache, whereas feared but rare complications include paraplegia, intraspinal hemorrhage, cauda equina syndrome, nerve injury, and epidural hematoma. This case report presents a 40-year-old female with undiagnosed and asymptomatic ependymoma who presented with concerning neurological symptoms after receiving spinal anesthesia for elective cesarean delivery. Stat lumbar and thoracic spine magnetic resonance imaging (MRI) were performed on postoperative day 13 and were suggestive of a large hypervascular mass with areas of hemorrhage, concerning for ependymoma. An emergent laminectomy and mass resection were performed, resulting in an improvement in the patient’s neurological symptoms. Neuraxial anesthesia is commonly performed for obstetric indications such as vaginal or cesarean delivery, tubal ligation, and cervical cerclage placement. Neuraxial anesthesia is generally safe, with common potential complications that include backache, post-dural puncture headache, and hypotension resulting in dizziness, nausea, and vomiting . Rare coA 40-year-old G6P5 female with a history of a torn left meniscus presented for a scheduled primary cesarean delivery and bilateral tubal ligation due to breech presentation. She had delivered vaginally five times before, each uneventfully with a labor epidural. Spinal anesthesia was performed at the L3-L4 level with a 25g Whitacre needle  in one attempt without difficulty. 12mg of 0.75% hyperbaric bupivacaine, 15mcg of fentanyl, and 100mcg of morphine were administered intrathecally. The cesarean delivery was uneventful and the patient was discharged home on postoperative day three. The patient presented on postoperative day six with new-onset low back pain with radiation to the lumbar paraspinals and posterior right thigh. The patient reported a history of spontaneously resolving sciatica symptoms in the postpartum period after receiving epidurals in prior pregnancies. The patient denied incontinence of urine or bowel and her neurologic exam showed no new weakness. She reported mild weakness in her left lower extremity that had been present prior to neuraxial anesthesia and was thought to be from her torn left meniscus. The pain was alleviated by hydrocodone and ibuprofen. The clinical picture was not consistent with a neuraxial abscess or hematoma and our differential diagnosis at the time included musculoskeletal pain and discogenic impairment of her lumbar spine, given her sciatica after prior deliveries. Given the benign neurological history and physical exam, a space-occupying lesion was low in the differential diagnosis. The patient was discharged home with a recommended trial of gabapentin for pain management.The patient subsequently returned on postoperative day 13 for new-onset urinary retention and difficulty ambulating. Her neurologic examination was remarkable for numbness in the bilateral lower extremities, 3/5 strength on hip flexion, and 4/5 strength on plantar and dorsiflexion bilaterally. Stat lumbar and thoracic spine magnetic resonance imaging (MRI) exams were obtained and revealed a large hypervascular mass with areas of hemorrhage occupying and expanding the spinal canal from T11 through L4-L5 junction with a small amount of subarachnoid hemorrhage in the sacral canal (Figure Neurosurgery performed an emergent T11-L5 laminectomy for mass resection. Surgical pathology confirmed the diagnosis of myxopapillary ependymoma Figure .She was discharged to inpatient rehabilitation on postoperative day four with residual but improved lower extremity weakness and neurogenic bowel and bladder. While in inpatient rehabilitation, the patient’s neurogenic bowel and neurogenic bladder improved, with urology recommendation to continue intermittent straight catheterization daily to twice daily. She continued to have lower extremity weakness, although this improved during her rehabilitation admission. She was subsequently discharged home with outpatient physical therapy and occupational therapy.Spinal cord ependymomas are rare and generally slow-growing tumors with complete surgical resection being the first-line treatment. Patients with spinal cord ependymomas usually present in their 30s-40s with nonspecific symptoms that may progress over years prior to diagnosis. However, intratumoral hemorrhage can provoke acute deterioration. Neuraxial anesthesia may be a factor in unmasking symptoms due to local trauma and intratumoral hemorrhage. Depending on the location, ependymomas may cause upper or lower extremity symptoms if present in the cervical area, or incontinence, radicular back and leg pain, and asymmetric weakness if present in the lumbar area .Five prior cases were described in the literature of women who underwent neuraxial anesthesia for delivery, followed by neurologic symptoms and subsequent discovery of spinal ependymoma Table . CampbelOur patient likely had this large preexisting asymptomatic ependymoma that was discovered when spinal anesthesia caused localized trauma, hematoma, and progressive neurological symptoms. Localized trauma and hemorrhage following neuraxial anesthesia may be a common factor in acute deterioration within a week in these otherwise healthy young patients as tumors may be more vascular and likely to bleed than the usual CNS contents. As undiagnosed asymptomatic CNS tumors are rare and it is not feasible to screen all parturients prior to neuraxial anesthesia, this case and the previously reported cases highlight the importance of prompt evaluation of worsening neurologic symptoms after neuraxial anesthesia. Following this incident, to improve the quality of care and patient education, we have revised our discharge instructions to include a special section on concerning symptoms following neuraxial anesthesia that patients should return to be evaluated for. We hope that this will allow patients to receive timely evaluation and treatment to potentially avoid permanent neurological sequelae.


We report the first case of a Hispanic infant with a congenital hyperpigmented macule of the tongue at Woodhull Medical Center in Brooklyn, NY. Congenital melanocytic macule of the tongue (CMMT) is a rare entity with a benign course usually reported in dark-skinned and Caucasian patients . Our litThis case report would aim to increase awareness about this benign skin condition among the general pediatricians practising in nursery and neonatal intensive care unit (NICU) setting. Because there are technical difficulties associated with invasive procedures, such as surgery or biopsy, that are performed on a child, close follow-up of this type of lesions should be performed. The lesions would likely increase in size with a child’s growth . We repoA full-term male newborn (39 weeks) of Hispanic origin presented with a pigmented lesion on the tongue, which was noted at birth. A physical exam showed an active newborn, skin phototype IV, and a single 5x15 mm, irregular, dark-brown, homogeneous pigmented lesion on the right dorsum of the tongue . Additionally, there is an increase in the basal epidermal layer’s melanin pigmentation and hyperkeratosis associated with abundant subepidermal pigment-laden macrophages .LimitationsThis report’s limitations include the paucity of data regarding CMMT in the literature, which led to a brief review of the treatment algorithm. We suspect that the reason for this scarcity could be the low rate of reports of existing cases.Congenital melanocytic macule of the tongue is a rare but benign entity with an underestimated prevalence. General pediatricians usually have low exposure to this condition resulting in failure to recognize and underdiagnosis. In most cases, frequent follow-ups are conducted to ensure that the lesion is not presenting with acute changes in shape or color that might suggest a different diagnosis. To date, there is no specific treatment for this condition. In this case, the patient received no medications, and at seven months of age, remained asymptomatic. However, even though the patient was monitored every three months, a biopsy was indicated.


Teaching point: Ovarian teratoma rupture can manifest clinically as an acute or chronic syndrome, associated with specific imaging features, both characterized by intra-abdominal fatty fluid. A 55-year-old female was admitted with complaints of diffuse abdominal pain and fever for three days. A computed tomography (CT) of the abdomen Figure  demonstrA second CT was performed three months later for persistent abdominal tenderness. It showed more organized fat zones along the mesenteric vessels, surrounding the intestine and mostly prevailing in the upper part of the abdominal cavity, under the diaphragm Figure , arrows.Mature cystic teratomas are one of the most common ovarian benign masses. They can contain different components as mature skin, hair follicles, nails, or teeth. Fat is present in almost all lesions, although a small proportion shows minimal or no fat which leads to challenging imaging diagnoses.Most frequent complications of teratomas are torsion and rupture, which occurs in 1–4% of the cases. Teratoma rupture is usually secondary to torsion, infection, direct trauma, or delivery. Spontaneous rupture is less frequent because of the thick capsule that typically surrounds the tumour. It induces intra-peritoneal leakage of the sebaceous content of the tumour, causing peritoneal inflammation. Two different clinical syndromes are described: acute rupture is associated with abdominal pain or heaviness with nausea and vomiting, while chronic rupture is a more indolent syndrome characterized by diffuse abdominal tenderness or discomfort secondary to chronic granulomatous peritonitis [CT is the most sensitive technique to assess teratoma rupture, showing patchy hypoattenuating fatty fluid collection in the abdominal cavity, characteristically below the right hemi-diaphragm. In chronic rupture, signs of chronic peritonitis are typically found like diffuse intra-abdominal fat stranding and peritoneal thickening. These findings should not be mistaken for tuberculous or carcinomatous peritonitis.


Chordoid gliomas arise near the third ventricle and commonly present around 40 years of age. These rare tumors are non-invasive and often present with headaches and visual disturbances. Contrast enhancement on MRI is typical for these tumors and immunohistochemical (IHC) staining is positive for glial fibrillary acidic protein (GFAP). Surgical resection is the treatment of choice. We present this case of chordoid glioma because of its unique characteristics. The tumor lacked contrast enhancement on MRI and demonstrated juxtanuclear dot-like immunoreactivity for synaptophysin which is a feature not previously reported in the literature. It is important for pathologists and radiologists to be on the lookout for atypical presentations of these rare tumors. Chordoid gliomas are rare, non-invasive tumors that usually arise within or near the third ventricle. These tumors were established as pathologic entities in 1998 and are classified as grade II by the 2016 World Health Organization (WHO) [The initial presentation of these tumors can vary from an incidental finding to an invasive at the time of diagnostic imaging . There iA 48-year-old man with a past medical history of diabetes mellitus type II presented with a several-week history of acutely worsening ataxia, forgetfulness, nausea, intermittent vomiting, headache, and blurred vision. MRI showed a non-enhancing, cystic-appearing intraventricular mass centered on the third ventricle near the right foramen of Monro (arrow), with obstructive hydrocephalus and no restricted diffusion Figures  and 2. ADifferential diagnoses, in this case, include chordoma which was ruled out with positive IHC staining for GFAP. Additional differentials considered including meningioma which was ruled out with negative IHC staining for epithelial membrane antigen (EMA).Chordoid gliomas typically demonstrate contrast enhancement by MRI , althougChordoid gliomas are considered grade II by the 2016 WHO revised brain tumor classification, as tumors may recur following subtotal resection. Gross total resection, as achieved in this case, maybe curative. As such, surgical resection is the treatment of choice. At present, no evidence base exists for the role of adjuvant radiotherapy [We present a rare case of chordoid glioma of the third ventricle. Our case differed from those previously reported in that it lacked contrast enhancement on MRI and showed distinct, juxtanuclear dot-like immunoreactivity for synaptophysin. It will be interesting to see if subsequent cases are reported with these phenotypic features and whether these features correlate with a favorable outcome.


A 41-year-old woman was referred because of abdominal pain, reporting on an appendectomy 24 years ago. At that time, she developed a postoperative intra-abdominal abscess and was told that the tip of the appendix had been left in the abdomen. Seventeen years later, a CT-scan showed a 6 cm cystic tumor at the caecum base . The treA 59-year-old man presented with abdominal pain nine years after a laparoscopic appendectomy. He reported on an intra-abdominal abscess eight months postoperatively, requiring radiological drainage. A low-grade PMP (PCI 12) was diagnosed, and an appendix tip remnant identified .A PMP can arise from an appendiceal tip remnant. A history of appendectomy does not exclude an appendiceal origin of PMP.


A 55-year-old Japanese woman, who had been diagnosed with ulcerative colitis at 18 years of age, underwent screening endoscopy examinations. Esophagogastroduodenoscopy revealed an extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) of the stomach. Colonoscopy showed a slightly elevated reddish lesion with dilated microvessels but no erosions or ulcers. Although MALT lymphoma in the cecum was endoscopically suspected, flow cytometry and pathological analyses led to the diagnosis of appendiceal orifice inflammation in ulcerative colitis. This case highlights the diversity of the endoscopic appearance of appendiceal orifice inflammation in ulcerative colitis. Ulcerative colitis typically causes continuous inflammation in the colorectal mucosa extending from the rectum to the more proximal colon. However, the peri-appendiceal mucosa in the cecum is sometimes involved in a skip lesion –5. This We, herein, report a case involving a patient who had ulcerative colitis concomitant with extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) in the stomach. Of note, although the patient's cecal lesion was initially considered to be involved with the MALT lymphoma, flow cytometry and pathological analyses led to the diagnosis of appendiceal orifice inflammation in ulcerative colitis. Typical endoscopic features of colorectal MALT lymphoma and appendiceal orifice inflammation in ulcerative colitis are discussed later in this report.α antibody use, her ulcerative colitis was treated with a mesalazine and Clostridium butyricum preparation. She had also been taking fexofenadine, tranexamic acid, and brotizolam for sleep disturbance and prevention of drug allergy. She had a history of Helicobacter pylori eradication. Esophagogastroduodenoscopy revealed multiple white irregularly-shaped depressed lesions with partial redness in the gastric body  was diagnosed.A 55-year-old Japanese woman underwent screening esophagogastroduodenoscopy. The patient had been diagnosed with ulcerative colitis at the age of 18 years. Since palmoplantar pustulosis and arthritis of the sternoclavicular and foot joints appeared after anti-tumor necrosis factor- Figures . Narrow- Figures , arrows, Figures , which w Figures  and nega Figures . Immunos Figures . BIRC3-Mκ and Ig λ predominance was not observed on staining, which was consistent with the results of the flow cytometry assay. Thus, we diagnosed the cecal lesion as appendiceal orifice inflammation in ulcerative colitis, rather than being involved with the lymphoma. After discussing with the patient, we decided to manage her gastric MALT lymphoma with a watch-and-wait approach because the lesion was localized in the stomach, and she showed no symptoms, bulky adenopathy, or organ compromise.Colonoscopy revealed coarse reddish mucosa with yellowish-white pus-like deposits in the splenic flexure , which aSkip inflammation of the peri-appendiceal region in patients with ulcerative colitis was first described in 1958 by Lumb and Protheroe . AppendiMALT lymphoma generally develops in the stomach, and colorectal involvement in MALT lymphoma is rare. Jeon et al. investigated the endoscopic features of 51 patients with colorectal MALT lymphoma and classified the lesions as subepithelial tumors (51%), polyps (20%), epithelial masses (14%), and ileitis (16%). MALT lymphoma was most frequently observed in the rectum 39%), followed by the ileocecal area (30%) . Won et %, followSeo et al. reported a patient with a MALT lymphoma in the transverse colon, which showed a flat elevated lesion with diffuse granularity of the mucosa and loss of vascularity. Narrow-band imaging revealed an abnormal branch-like capillary pattern in patches . These mκ or λ chain. Thus, light chain restriction is the hallmark of B-cell lymphomas. For the present patient, the flow cytometry results were obtained on the day of colonoscopy, resulting in the timely achievement of the nonlymphoma diagnosis.For the present patient, flow cytometry analysis performed using an endoscopic biopsy specimen aided in establishing the prompt nonlymphoma diagnosis. Flow cytometric immunophenotyping is a sensitive and accurate method for the diagnosis of lymphomas that enables the detection and characterization of neoplastic lymphocytes. Since B-cell lymphomas generally develop due to the unrestricted growth of a particular clone of B cells, neoplastic cells express only one class of immunoglobulin light chain, which is either a In Japan, flow cytometry has not typically been used to diagnose gastrointestinal lymphoma, as an appropriate protocol has not yet been established for lymphocyte isolation from endoscopic biopsy specimens. We recently established lymphocyte isolation techniques and used them to confirm that light chain expression analysis can be performed on such specimens , 18. HowIn conclusion, this case highlights the diversity of the endoscopic appearance of appendiceal orifice inflammation in ulcerative colitis. Although pathological analysis is essential for the differentiation of nonlymphoma lesions from lymphomas, flow cytometry analysis has the advantage of delivering results rapidly.


Alveolar soft part sarcoma (ASPS) is a clinically and morphologically distinct malignant soft tissue tumour. It occurs mostly in the lower extremities in adults. The purpose of our study was to describe the related clinicopathologic factors, treatment and prognosis of recurrent orbital ASPS in children.Three children aged from 1 to 12 years presented with unilateral proptosis, restricted ocular motility and impaired visual acuity of the affected eye. Periodic acid Schiff (PAS) -positive diastase-resistant crystalline granules were found in all cases. Immunostaining of TFE-3, INL1 and Ki67 was positive in the tumour cells of two patients. The time until local recurrence of primary tumor in patient 2 and patient 3, who only underwent tumour resection, was shorter than that of patient 1, who underwent tumour excision and postoperative radiotherapy. The recurrent masses were successfully treated with complete tumour excision followed by adjuvant radiotherapy. Patient 1 presented metastasis at 11 years after radiotherapy.Orbital ASPS in children is easily misdiagnosed due to its rare occurrence and atypical clinical findings. Early diagnosis with multidisciplinary, complete surgical resection combined with adjuvant radiotherapy is essential for achieving long-term disease-free survival in orbital ASPS patients. Alveolar soft part sarcoma (ASPS) is an extremely rare soft tissue sarcoma that accounts for 0.5–1% of all sarcomas . It usuaAfter review of the diagnoses of cases accessioned in the database of the Ophthalmology Department and Pathology Department of West China Hospital of Sichuan University, three cases of orbital alveolar soft part sarcoma (ASPS) diagnosed between 1993 and 2019 were identified. The clinical histories, manifestations and pathologic findings of the tumours were reviewed. A literature review was performed using MEDLINE for studies published between 1950 and the present, and EMBASE was searched for studies published between 1980 and the present.A 9-year-old Chinese girl sought treatment for a five-month history of prominence of the left eye. On examination, the corrected visual acuity was 20/20 OD and 20/25 OS. The left eye had a nonpulsatile and nonreducible proptosis of 8 mm, as revealed by a Hertel exophthalmometer. A dilated epibulbar vessel on the temporal aspect of the left eye and dilated retinal veins of the left fundi were noted by indirect ophthalmoscopy. CT imaging indicated a 3.5 × 2 × 2.5 cm well-defined soft tissue mass occupying the posteroinferior part of left orbit in close relation to the optical nerve that did not invade the orbital wall see Fig. . a. LeftA 12-year-old girl presented left upper eyelid swelling and prominence of the left eye for one month. Her corrected vision was 20/20 OD and 20/40 OS. The left eye showed a proptosis of 3 mm with limited motility in all directions. The remainder of the ophthalmic examination results were normal. CT imaging revealed a hyperdense, well-defined intraconal mass of 2.4 × 2.0 × 1.5 cm that compressed the posterior of the eyeball and the superior part of the optic nerve. Although the mass was resected by left orbitotomy and pathologically confirmed to be ASPS, the patient returned with painless prominence of the left eye two months later, indicating local recurrence. Examination of the left eye showed marked eyelid swelling, chemosis and a proptosis of 4 mm with limited motility. A CT scan revealed a tumour of 4.2 × 4.0 × 2.8 cm. After adequate surgical removal tumour . The tumour cells had a distinctly epithelioid appearance with well-defined cell borders, abundant eosinophilic cytoplasm and large vesicular nuclei with prominent nucleoli . Periodic acid Schiff (PAS) staining revealed characteristic diastase-resistant crystalline structures that varied in size and shape within the cytoplasm . Nuclear atypia and mitotic figures were seen in the tumour cells of the recurrent ASPS in patient 2. Muscle markers  and non-muscle markers  were used for immunohistochemical staining in our study . The tumours from patient 1 were positive for S100 and myoglobin. TFE-3, INL1 and Ki67 stained positive in patient 2. The tumours of patient 3 were positive for TFE-3, INL-1, CD34, Desmin, and Ki67(ranging from 10 to 20% positivity), indicating the proliferative potential of ASPS.Gross specimens of the tumour appeared yellow to brown in patient 1 and red brown in both patient 2 and patient 3. The specimens were all irregularly shaped soft to firm masses. Microscopic examination of the primary and recurrent tumours from three cases showed that the large oval or polygonal tumour cells were arranged in an organoid or alveolar pattern separated by delicate septa of connective tissue containing capillary-sized vascular channels ASPS is a malignant soft tissue sarcoma that rarely occurs in the orbit. A review of the literature indicates 60 reported cases in the orbit to date –29. OrbiASPS usually presents as a slow-growing, painless tumour mass. Orbital lesions have no typical characteristics in terms of clinical and imaging features. ASPS commonly presents proptosis, lid swelling, and dilation of conjunctival vessels. Some patients show compression of the optic nerve, as was seen in our cases, which might lead to impaired vision. PAS staining reveals the characteristic diastase-resistant crystalline structures in ASPS, and D-PAS staining was also used to demonstrate that the PAS-positive structures were not glycogen. Although orbital ASPS has characteristic histopathology, its immunohistochemical staining pattern has been controversial, and the histogenesis of ASPS remains uncertain. Some muscle markers  and non-muscle markers (S-100) have been reported to be expressed at variable frequencies in ASPS. Our report showed that TFE-3 and INL1 were positive in both the primary and recurrent tumours of two patients. The current concepts demonstrate that ASPS is characterized by the translocation t p , which results in the chimeric ASPSCR1-TFE3 transcription factor, which drives tumorigenesis and provides an important clue for the diagnosis of ASPS , 31.ASPS may be treated as a vasogenic tumour due to its high vascularization on CT imaging, including haemangioma or lymphangioma. The pathologic differential diagnoses of ASPS include paraganglioma, malignant melanoma, alveolar rhabdomyosarcoma, granular cell tumours and metastatic renal cell carcinoma (RCC), which also need to be considered . ASPS usDue to the rare occurrence of orbital ASPS, the optimal management remains controversial. Although complete surgical excision of the small tumour with functional preservation remains the treatment of choice , it is dThe prognosis of ASPS is largely dependent on the initial presentation , tumour size, and age of onset (patients less than 20 years old may survive significantly longer than those over 20 years old) , 38. Orb


Uterine sarcomas are a rare malignancy, often retrospectively diagnosed after myomectomy or hysterectomy. Undifferentiated uterine sarcomas (UUS) are a particularly aggressive variant of this condition. Little evidence exists regarding the postoperative management of undifferentiated sarcomas diagnosed after hysterectomy performed for presumed benign conditions. We describe the case of a 33-year-old woman who presented with heavy bleeding and subsequently underwent hysterectomy on an emergency basis after failed medical management. Cut-section of the uterus revealed a grossly benign-looking sub-mucosal fibroid. However, the final histopathology report revealed undifferentiated uterine sarcoma. We worked up the patient postoperatively with MRI to rule out metastasis and performed bilateral salpingo-oophorectomy based on hormone receptivity status. We followed this with single-agent chemotherapy with adriamycin, which was followed by continuous therapy with oral letrozole (aromatase inhibitor). The patient was found doing well at the two-year follow-up, with no evidence of relapse. Postoperative diagnosis of UUS should include imaging to rule out metastasis, consideration for completion of surgery based on hormone receptivity of tumour, and lymphadenectomy based on the subtype of tumour. Uterine sarcomas are a rare malignancy and often a post-operative diagnosis. High-grade undifferentiated uterine sarcomas (UUS) are an aggressive subtype of uterine sarcomas and are often associated with poor prognosis . The preOur patient was a 33-year-old homemaker who presented in the emergency with the chief complaint of heavy vaginal bleeding for the past 14 days. Since her menarche at the age of 11 years, she had had regular menstrual periods with average flow. However, for the past one year, she had been suffering from heavy, prolonged menses, lasting 15-20 days and producing large clots. She also complained of the descent of a vaginal mass, which she felt was more evident during intercourse and attributed it to the post-coital bleeding she had been experiencing for the past few months. There was no increase in the size of the mass after prolonged standing, straining during defecation, or micturition. She had no history of abdominal pain, abdominal mass, urinary or bowel complaints, easy bruising, excess fatigue, or weight loss. She had been advised tranexamic acid orally and had been taking 3 g per day. She had been married at the age of 18 years, had had four term vaginal deliveries, and had never used any form of contraception in the past. For the past 14 days, her bleeding had been more profuse than usual, with episodes of flooding; and tranexamic acid had provided minimal or no relief. She had been then referred to us, a tertiary centre, for subsequent management.We found her to be extremely pale, with a pulse rate of 102/minute, and a blood pressure of 92/64 mm. General physical examination was otherwise insignificant. Her abdomen was soft with no organomegaly. On per speculum examination, the vagina was found to be entirely filled with a large, irregular 8x8-cm mass coming out of the cervix. It bled on touch, but there were no areas of necrosis or ulceration. There was no foul odour. On per vaginal examination, the mass was firm, occupying the entire vagina, and non-tender. Her uterus could not be felt separately, and fornices could not be reached. Her diagnostic workup showed severe anemia and fibroid uterus  . We atteOn histopathological examination, a gross evaluation showed a total hysterectomy specimen with a 9x6-cm polypoidal growth from the posterior isthmus, extending into the cervix, which was fleshy, white, and congested, but with no necrosis or haemorrhage. The uterine fundus was free of tumour. Microscopy showed spindle-shaped cells with eosinophilic cytoplasm, prominent nucleoli, marked nuclear pleomorphism, brisk mitosis, and giant tumour cells. Immunohistochemistry was positive for CD10, cyclin D1, desmin, ER and PR and negative for pan-cytokeratin, epithelial membrane antigen, smooth muscle actin, and myogenin. The expert pathological review was confirmatory of high-grade UUS, stage IB. 2 IV, followed by continuous therapy with the aromatase inhibitor letrozole 2.5 mg/day. At the two-year follow-up, the patient was found doing well, with no evidence of relapse.To guide further management, we obtained a pelvic and abdominal MRI with gadolinium contrast, which showed no evidence of residual tumour or lymph-node involvement. We performed laparoscopic bilateral salpingo-oophorectomy three weeks after the first surgery, in light of ER and PR positivity. We then administered three cycles of single-agent chemotherapy with adriamycin (doxorubicin) at the dose of 75 mg/mUterine sarcomas are rare mesenchymal tumours accounting for 3% of endometrial cancers. The most common are leiomyosarcomas (LMS), which account for 60%, followed by endometrial stromal sarcomas . Our patient had an undifferentiated or poorly differentiated uterine sarcoma, the least common of uterine sarcomas . SarcomaOne of the questions pertinent to this case is whether we could have diagnosed or suspected uterine sarcoma preoperatively. This is often a difficult task, even with a prudent combination of history and examination, imaging, biopsy, and tumour markers. In a retrospective analysis of 1,332 women undergoing hysterectomy for fibroid, 0.27% of the 371 patients who had a history of rapid growth had a final diagnosis of sarcoma; whereas 0.15% of 961 who did not have any such history was diagnosed with sarcoma. This refuted an earlier theory that rapid uterine growth (6-weeks' size) within one year suggested sarcoma . MRI mayThe subsequent dilemmas which arise post-diagnosis include uncertainty about performing salpingo-oophorectomy, lymphadenectomy, radiotherapy, and other adjuvant therapy. There is extremely limited literature specific to UUS; hence we extrapolated findings from other types of uterine sarcomas. Adjuvant radiation therapy has been retrospectively found to be associated with poor survival in uterine sarcomas, leading us to rule it out it for our patient . RegardiThe evidence on the management of UUS is very limited and is often based on the recommendations on other types of sarcomas with shared characteristics. Uterine sarcomas can occur at any age and require a very high degree of suspicion and multiple diagnostic modalities to predict preoperatively. MRI is considered the most useful modality currently. Bilateral salpingo-oophorectomy should be done if the ER is positive. Hormone suppression therapy in the form of aromatase inhibitors should be strongly considered for ER-positive sarcomas, irrespective of the subtype. There is inadequate evidence for or against lymphadenectomy for UUS.High-grade UUS is a rare and often unexpected postoperative diagnosis. This case demonstrates how careful application and extrapolation of evidence can lead to successful patient outcomes.


Synovial sarcomas are rare malignant tumors that originate from primitive pluripotent mesenchymal stem cells that look similar to the developing synovium, but are histologically unrelated to it. Sarcomas commonly metastasize to the lungs and surrounding pleura, with a documented incidence as high as 85% for pleural-based metastases. The incidence of spontaneous pneumothorax in patients with sarcomas is only 1.9%, with synovial sarcoma being the third most common type of sarcoma associated with pneumothorax. While surgical resection is usually the treatment for localized primary synovial cell sarcoma, metastatic disease requires systemic therapy, mainly chemotherapy. Failure of chemotherapy calls for the use of targeted therapeutic agents such as pazopanib. Pazopanib has been linked to the incidence of spontaneous pneumothorax in previous case studies. However, primary research fails to establish a statistically significant causal association. Research shows that pneumothorax can result from lung metastases independent of therapeutic side effects. We report a case of synovial sarcoma of trapezius origin with secondary lung metastases, and development of pneumothorax after pazopanib treatment. We discuss the incidence of pneumothorax as a medication side effect versus independent effect of natural disease progression, and how this plays role in deciding when to continue using a medication in the face of complications. Synovial cell sarcomas are rare malignant tumors that originate from primitive pluripotent mesenchymal stem cells that microscopically resemble the developing synovium, but are unrelated to the synovial tissue ,2. They No optimal treatment for synovial sarcomas has been defined so far . CompletA 28-year-old female was diagnosed in Tunisia with stage IV synovial sarcoma originating from the soft tissue of the left trapezius with lung metastases, after complaining of shoulder swelling and pain back in July 2018. Written reports of the CT imaging obtained in Tunisia revealed that she had a 4 x 2 cm left paravertebral pleural formation, 1 cm left pleural nodule and 10 x 9 x 3.5 cm left posterior dorsal wall mass of muscular origin presumed to be the trapezius, at the time of diagnosis. Outside reports of the biopsy of the intramuscular mass revealed that she was diagnosed with a grade 2 monophasic synovial cell sarcoma. She had no family history of malignancy. She received 6 cycles of adriamycin and ifosfamide with mesna. The size of tumor decreased after the third cycle and continued to be stable afterwards. The plan was to start radiation after completion of chemotherapy, but she relocated to the United States before starting radiation therapy.She established care in our institution in February 2019. A repeat CT revealed a left pleural nodule measuring 3.9 cm, left lower lobe nodule measuring 1.5 cm, and a right upper lobe nodule measuring 3.5 cm  in sarcoma is only 1.9% and bilateral involvement is common . The mosIn our case, the drug that was thought to be responsible for triggering the SSP was pazopanib. There are individual case reports in the literature that report the incidence of SSP in sarcoma patients who had lung metastases, after beginning treatment with pazopanib ,12. HoweOverall, the prognosis of sarcoma-associated SSP is poor, and the one-year survival rate is generally around 20% . To our Through this case, we highlight the dilemma of attributing an unexpected complication like pneumothorax to therapeutic side effects versus an independent result of malignant disease progression. Pneumothorax in the setting of cancer with lung metastases worsens respiratory status and further decreases the chance of survival. Therefore, the clinical decision to continue a specific therapeutic agent in spite of complications that ensue, must be made by carefully weighing the beneficial effect of the drug in reducing further disease progression against validated side effects and the natural course a disease would take in the absence of treatment.


Soft tissue dedifferentiated leiomyosarcoma with heterologous osteosarcomatous component is an extremely rare entity described in only few cases in the literature.We report the case of a 65-year-old male patient who, after initial inadequate surgery of a tumor of the left forearm, developed local recurrence that was treated with neoadjuvant chemotherapy, surgery and postoperative radiation therapy. Histologically the tumor showed an abrupt separation of two different patterns. One component consisted of interlacing fascicles of spindle cells with cigar-shaped nuclei strongly positive for smooth muscle actin, desmin and H-caldesmon. The other component consisted of a high-grade pleomorphic sarcoma with osteoid and chondroid matrix production, which positive for SATB2. Thus, a final diagnosis of dedifferentiated leiomyosarcoma was rendered. Fifteen months after treatment, the patient presented further local and distant relapse with pulmonary metastases and died 23 months after the first presentation.Dedifferentiated leiomyosarcoma is a highly malignant neoplasm with a poor outcome. Extensive sampling of soft tissue leiomyosarcomas is recommended to detect possible dedifferentiated areas as they represent a crucial prognostic parameter. Leiomyosarcoma (LMS) is an aggressive soft tissue sarcoma that accounts for about 7 to 10% of all soft tissue sarcomas and usually occurs in adult and elderly patients , but it The term ‘dedifferentiated leiomyosarcoma’ was first used by Schmookler and Lauer in 1983  and refeA 65-year-old man came to our observation with a solid-elastic mass of about 4.5 cm maximum diameter on the proximal region of the left forearm, ulnar side, that appeared hypomobile on the underline planes. He reported being affected by this “bump” for at least 20 years, but it had recently shown a rapid growth. Furthermore, he reported having already undergone another excision in local anesthesia after an ultrasound examination about 1 year before at another hospital. An X-ray of the forearm showed a soft tissue mass with irregular calcifications Fig. . StagingAfter preoperative needle biopsy, surgical excision of the tumor was performed, including skin (with the scar of the previous surgery), fascia, muscles and a portion of the ulna. Reconstruction was performed with plate, screws and cement and coverage with a microsurgical rotation flap.Gross examination of the surgical specimen revealed a solid white neoplasm with a maximum diameter of 6 cm in the soft tissues. Histologically, the tumor showed two different, abruptly separated components Fig. . One conImmunohistochemically, areas with leiomyosarcomatous appearance were strongly positive for alpha smooth muscle actin Fig. a and desThe final diagnosis was leiomyosarcoma grade 2, according to the French Federation of Comprehensive Cancer Centers (FNCLCC) grading system  with areAfter the surgical treatment, the patient received three cycles of high-dose adjuvant chemotherapy with adriamycin and iphosphamide and local sequential radiotherapy (66 Gy). After an interval of 15 months, MRI showed multiple local recurrences at the site of previous excisions and a PET CT total body scan highlighted bilateral pulmonary nodules, for which he underwent a new cycle of chemotherapy with gentamicin. The patient died after 23 months from presentation due to the progression of the disease.Even if there is no consensus regarding the exact definition of dedifferentiated leiomyosarcoma, it may be described as a well-differentiated tumor showing typical features of a smooth muscle neoplasm with a high-grade undifferentiated pleomorphic sarcoma component lacking expression of muscle markers.Dedifferentiation is a well-known occurrence in liposarcoma and chondrosarcoma, but this phenomenon has been only rarely reported in LMS. In most cases, dedifferentiated LMS presents discrete transition from well-differentiated smooth muscle morphology to high-grade pleomorphic morphology with loss of smooth muscle differentiation . Only exThe first case was a 71-year-old man who underwent resection of an intramuscular lesion of the leg, which had rapidly enlarged in the preceding 8 weeks . The tumGrabellus et al.  studied The large series of 18 dedifferentiated LMSs reported by Chen et al. [Feeley et al.  describeThe main clinical and histological differential diagnosis is with myositis ossificans. This benign lesion presents a characteristic zonal growth pattern, with a central area that consists of a nodular fasciitis-like proliferation of bland spindle cells, and progressive deposition of osteoid matrix in the periphery. Malignant bone forming tumors of the soft tissues, including the present case, lack this orderly growth pattern and usually present marked cytologic atypia. Another entity that can be considered in the differential diagnosis is dedifferentiated liposarcoma with heterologous smooth muscle and osteo-chondroblastic differentiation. This possibility was excluded because there was no evidence of a well-differentiated liposarcoma component in both the first specimen and in the recurrence, and in addition the tumor was negative for MDM2 and CDK4. The possibility that the lesion represents a soft tissue osteosarcoma in which spindle cell areas show features of leiomyosarcoma or myofibrosarcoma was also evaluated, but it seemed unlikely because the two components were always sharply separated, a feature more consistent with the concept of dedifferentiation.Considering all the cases reported, the average age is 60 years, with a clear male predominance (4:1). It is very important to notice that all the 5 patients had local recurrences and developed distant metastases despite aggressive treatments. In 3 patients the outcome was fatal  due to the progression of the disease, while no clinical follow-up was detailed for the remaining 2 patients. These data confirm that dedifferentiated LMS of the soft parts is a highly aggressive tumor. According to the literature, these neoplasms present 50% to 65.2% of mortality and 89% of incidence of metastasis , 13. It