Appendicoliths are calcified deposits located within the appendiceal lumen, usually measuring less than 1 cm in diameter. Appendicoliths greater than 2 cm in the largest diameter are uncommon and referred to as giant appendicoliths. Generally, patients with giant appendicoliths are asymptomatic, with these being detected incidentally on X-ray or computed tomography (CT). However, the presence of appendicoliths has been shown to be associated with an increased risk of developing appendicitis and is associated with more severe appendicitis. There is an increased incidence of appendicoliths in retrocecal appendices. This case report is of an adult male patient who presented with a three-day history of right iliac fossa pain, nausea, and decreased appetite. CT of the abdomen and pelvis showed acute appendicitis secondary to a calcified 3.1 cm giant appendicolith. Open appendicectomy was subsequently performed as the patient’s financial constraints hindered a laparoscopic approach. The clinical outcome was successful with no postoperative complications, and the patient was discharged the following day. The patient was reviewed six weeks post-laparotomy with no complaints and was discharged from the surgical outpatient clinic. Acute appendicitis can be caused by gastrointestinal infections or by appendicoliths, with the latter accounting for 10% of cases .A 48-year-old male patient with no known comorbidities and a history of conservative management of appendicitis in 2015 presented with a three-day history of right iliac fossa pain, with loss of appetite and nausea. On examination, Mc Burney’s sign was positive, and an ultrasound was performed which demonstrated a dilated appendix. Computed tomography (CT) of the abdomen and pelvis was performed, which showed a fluid-filled dilated appendix, periappendiceal fat stranding, and a 3.1 cm giant appendicolith, with no evidence of perforation or abscess formation  but can become larger than 2 cm, which are then termed giant appendicoliths. The largest reported giant appendicolith measured 3.5 cm in 2005 [There is wide discordance regarding the prevalence of appendicoliths in appendicitis. While historical data depict a prevalence of 33-44%, contemporary evidence estimates a prevalence of approximately 1.5-15% [Giant appendicoliths are generally asymptomatic unless there is luminal obstruction. Patients present with typical features of appendicitis, such as colicky right lower quadrant abdominal pain progressing to guarding and rebound tenderness . AppendiAppendicoliths are usually found incidentally on imaging studies and have a diagnostic sensitivity of 65% and specificity of 86% when detected on CT [Giant appendicoliths can be managed conservatively when patients are asymptomatic or when surgery may impose greater risk to the patient . LaparosGiant appendicoliths are rare and usually asymptomatic. However, when complicated, they are often associated with acute appendicitis with increased risk of perforation, for which urgent appendectomy is warranted.


Intratumoral hemorrhage/apoplexy in clival chordomas is extremely uncommon, with only 16 reported cases. The average age of patients was 46.2 years and slightly more than half were men. In cases published before 1990, all patients died from their disease without any intervention. Since then, 11 patients have undergone resection by a variety of approaches and there have been no deaths. The diagnosis of skull base chordomas relies on a combination of clinical presentation and radiographic features related to the location and invasion of the tumor. Chordomas presenting with sudden-onset symptoms should alarm the surgeon of a possible hemorrhage. As an illustration of this presentation, we describe a 58-year-old woman who presented with acute-onset headache and cranial nerve deficits. Computed tomography and magnetic resonance imaging demonstrated a hemorrhagic clival lesion with cavernous sinus extension. The patient underwent transsphenoidal resection of the lesion that resulted in the resolution of her symptoms. Histopathological evaluation of the lesion was consistent with chordoma with acute hemorrhagic components. Although intratumoral hemorrhage is rarely detected in chordomas, it should be considered a differential diagnosis of such lesions because prompt recognition and treatment are critical for patient survival. Skull base chordomas are aggressive recurrent intracranial lesions thought to originate from primitive notochord remnants and constitute <1% of all intracranial neoplasms . Clival A 58-year-old hypertensive woman with a remote history of breast cancer treated with mastectomy and chemotherapy presented to the emergency department with progressive left-sided acute-onset headache, nausea, vomiting, double vision, and left-sided partial ophthalmoplegia and ptosis of five-day duration. The neurological examination also demonstrated slight anisocoria consistent with partial left oculomotor nerve palsy. The left pupil was in the "down-and-out" position. Other neurologic findings were unremarkable. All pituitary hormones were within normal limits, except for a slightly elevated cortisol level  and a decreased adrenocorticotropic hormone value .Computed tomography (CT) of the head demonstrated a high-density lesion arising from the clivus, with extensive osseous erosion into the left greater wing of the sphenoid and left petrous apex and scattered hyperdensities within the tumor bed Figures -1C. MagnThe patient underwent a microscopic transsphenoidal approach with endoscopic assistance for excisional biopsy to establish a pathologic diagnosis and decompress the cavernous sinus. The left sphenoid sinus and clival recess were exposed. The tumor was removed from the left clival recess and followed into the left cavernous sinus, medial and lateral to the cavernous ICA. The cavernous component of the tumor was visualized and resected with 30° and 45° angled endoscopes. Once adequate decompression was achieved, hemostasis was obtained. Nasal packings were used for closure, and no cerebrospinal fluid leak was encountered.Histological examination revealed a moderately cellular tumor comprising plump cells with well-defined cytoplasmic borders Figure , foamy cThe patient tolerated the procedure well with no complications. Postoperatively, her ophthalmoplegia and ptosis improved dramatically, with no new neurologic deficits. She was discharged home on postoperative day 3 and referred for proton beam radiotherapy. On telephone follow-up seven months after her surgery, she reported complete resolution of her symptoms and no new neurological issues.Skull base chordomas are slow-growing, aggressive, recurrent tumors marked by extensive local invasion and destruction of surrounding structures . AlthougAcute hemorrhage is most commonly intratumoral 75%) % 5-15]  15] but   15] butThe diagnosis of skull base chordomas relies on a combination of clinical presentation, histopathologic, and radiographic features related to the location and invasion of the tumor. Chordomas presenting with sudden-onset symptoms should alarm the surgeon of a possible hemorrhage. Sudden headache 82.3%) ,8,15,17 2.3% ,8,1Skull base chordomas generally present as well-circumscribed, osteolytic, destructive lesions with associated cortical and lytic bone destruction and soft-tissue extension on non-contrast CT scans ,21, whicMacroscopically, chordomas are generally lobulated with a gelatinous matrix or chondroid solid features. Microscopically, chordomas take one of three forms. Chordoma not otherwise specified or classical chordoma is the most common form and is composed of lobules of epithelioid cells separated by septa of fibrous tissue in a myxoid matrix. The cells have vacuolated cytoplasm and are referred to as physaliphorous cells. Chondroid chordoma is the second histological form and comprises an extracellular matrix resembling hyaline cartilage. The last histological form is dedifferentiated chordoma, in which the tumor shows features of both classical chordoma type and undifferentiated spindle cell tumors . This foAlthough the cause of hemorrhage remains largely unknown, various mechanisms have been proposed. Some authors have reported that rapid tumor growth without adequate matched blood supply may cause these small friable vessels to rupture . VasculaDespite the importance of maximal surgical resection, adjuvant therapy remains an essential part of the armamentarium in the management of skull base chordoma, especially in cases where complete resection is not feasible . PostopeHemorrhagic clival chordoma arising from the cavernous sinus is a rare clinical and radiographic presentation. The presence of skull base lesions with mixed T2 hyperintensity and focal areas of hemorrhage and bone erosion should raise a high index of suspicion for hemorrhagic clival chordoma, which presents acutely with apoplexy and resulting neurological deficits, requiring prompt recognition and treatment by skull base neurosurgeons for patient survival.


The origin of the mesenteric vasculature is highly variable. One such variation is the common celiaco-mesenteric trunk (CMT). To our knowledge, this is the first reported case of subacute duodenal obstruction caused by common CMT. The awareness of this anomaly helps keep a high index of suspicion for varied presentations, prompts appropriate investigations, timely intervention, and avoids iatrogenic injury.A 15-year-old boy presented with a history of repeated attacks of colicky abdominal pain with bilious vomiting. Computed tomography of the abdomen with intravenous contrast revealed subacute duodenal obstruction caused by an acute angulation of common CMT with the abdominal aorta. To relieve the obstruction, the patient underwent a side-to-side duodenojejunostomy.A common CMT, where the coeliac artery (CA) and superior mesenteric artery (SMA) have a common origin from the aorta, accounts for less than 1% of all splanchnic artery anomalies. Most CMTs are incidental findings, but aneurysm or dissection of the common trunk commonly accompany this anatomical aberrancy. Intestinal obstruction due to CMT anomaly is a rare occurrence.There should be a high index of suspicion concerning vascular anomalies in patients, especially children presenting with repeated episodes of subacute intestinal obstruction. This knowledge of vascular aberrations prevents disastrous iatrogenic complications. •Rarity of the occurrence of common celiacomesenteric trunk•As per our knowledge this is the first reported case of CMT presenting with duodenal obstruction.•Its similarity to Wilkie's syndrome in terms of patient and management•Different presentations of CMT•Knowledge of this variations important in other surgeries and endovascular procedures to avoid dreadful complications. Several anatomical variations are described in literature. One such variation is the common celiaco-mesenteric trunk (CMT). These variations are due to aberrations in the embryological development of the four ventral splanchnic arteries arising from the primitive abdominal aorta, i.e., the left gastric artery (LGA), common hepatic artery (CHA), splenic artery (SA), and SMA . This anThis article reports a case of subacute intestinal obstruction caused by a common CMT anomaly, investigated and managed at our tertiary care teaching hospital. After a thorough literature review, there have been no reports on subacute duodenal obstruction caused by a common CMT anomaly to date. This work has been reported in line with SCARE criteria .22, the patient also reported an inability to gain adequate weight. The abdominal examination was unremarkable.A 15-year-old male patient presented to the emergency department with features of subacute intestinal obstruction. The patient complained of intermittent episodes of abdominal pain, distension and bilious vomiting since childhood which were relieved spontaneously or with over-the-counter medications. With a BMI of 16 kg/mErect X-ray and ultrasonography of the abdomen were unremarkable. Contrast-enhanced computed tomography (CECT) with angiography showed extrinsic obstruction of the third part of the duodenum with resultant dilatation of the stomach and duodenum (maximum diameter 4.3 cm). CA and SMA had a common origin from the abdominal aorta at the lower border of L1, forming a common CMT. CMT formed an angle of 19 degrees with the aorta compressing the third part of the duodenum. 3D reconstruction of the images confirmed the findings mentioned above while providing detailed anatomy of the CMT , Fig. 2.The patient was optimised nutritionally and taken up for exploratory laparotomy. Intra-operatively, the stomach, first and second parts of the duodenum were dilated and hypertrophied. There was an abrupt cut-off in the dilated duodenum where the CMT crossed it. These findings confirmed the diagnosis .Fig. 3ShA side-to-side duodenojejunostomy was performed to bypass the obstruction. The post-operative period was uneventful, and the patient obtained symptomatic relief .Fig. 4Sh2. He had no recurring symptoms and was pain-free thereafter.Follow-up at 3, 6 and 12 months showed an improved BMI of 17.8 kg/m3The primitive dorsal abdominal aorta gives origin to four ventral mesenteric roots- LGA, CHA, SA, and SMA, connected by a longitudinal anastomosis  3].Fig..Fig.3].FThe interruption in the longitudinal anastomosis between SA and SMA generates the normal configuration of CA and CMA. Any deviations in these lead to the formation of different types of common CMT .Common CMT, a rare anatomical variant, has a common origin of the CA and SMA from the aorta, with an estimated incidence of 0.25% ,5. In thThis anomaly is associated with clinical conditions like compression of CMT by abdominal aortic aneurysm , gastroiVon Rokitansky first described the compression of the anterior duodenal wall by the SMA as a cause of duodenal obstruction in 1861 on a post-mortem case. It was studied in detail by D. Wilke in 1912 . This vaThe length and attachment of Treitz's ligament, the level at which the duodenum crosses the vertebral column, influences the angle of SMA origin . The norThe primary symptoms of such vascular compressions are epigastric pain and vomiting, progressively increasing in severity, causing fluid and electrolyte disturbances with weight loss. Radiological imaging confirms the diagnosis . CECT abThe operative options include duodenojejunostomy, transection of the ligament of Treitz and relocation of the duodenojejunal junction (Strong's Procedure), gastrojejunostomy and anterior duodenal replacement . StarleyOwing to the anatomical similarities, a duodenojejunostomy can be performed in a common CMT causing duodenal obstruction as done in our case.4An understanding of the clinical relevance of CMT anomaly remains anecdotal, given its infrequency. Nevertheless, most incidentally diagnosed CMTs without intrinsic disease will remain asymptomatic. In patients presenting with repeated episodes of subacute intestinal obstruction, especially in the pediatric age group, there should be a high index of suspicion concerning vascular anomalies such as these. CECT abdomen with angiography remains the investigative modality of choice. The awareness of this anatomical variation helps plan therapeutic options and reduce the chance of surgical injuries.Our study is exempt from ethical approval.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.Dr. Priyanka Saha was the assistant operating surgeon, helped perform literature review and condense the manuscript to meet author guidelines.Dr. Keerthika Reddy helped draft the operative section of the manuscript and performed literature review.Dr. Rajeshwari Bhat B helped draft the manuscript discussion, edited images and is the corresponding author.Dr. Waqar Ansari was the lead surgeon on the case, helped draft the manuscript and reviewed it prior to submission.Dr. Asif Ansari was assistant operating surgeon and performed a literature review.Dr. Hridayanath Desai was the assistant operating surgeon and reviewed it prior to submission.Dr Priyanka Saha.Research registry unique identification number: researchregistry6675.A written informed consent was obtained from the patient and relatives for the publication if this case report and images. A copy of this available for review by the Editor-in-Chief of this journal.No conflicts of interest between the authors.


Headache is one of the most frequent complaints in the outpatient department. The types of headaches can be broadly classified into primary and secondary. The primary headaches have benign intrinsic causes and include tension, migraine, and cluster headaches. A detailed history and appropriate physical examination are essential in assessing patients with headaches. We present the case of a 33-year-old woman who presented to our primary care clinic with three days history of worsening frontal headache. She had been experiencing this headache daily for the last three months; however, the current episode is more severe. The headache episode was not associated with fever, neck stiffness, or loss of consciousness. She often became nauseated with the headache. There was no history of weakness, numbness, or visual disturbances with the headache. There was no family history of migraine headaches. On examination, no focal neurological deficit was noted. The head CT scan showed the presence of two highly hyperdense foci in the frontotemporal region, one of them was related to the dura. Such foci were not causing midline shift or brain edema. The preliminary diagnosis was calcified meningioma. Surgical excision of the lesions was planned. The patient underwent right craniotomy under general anesthesia. The two osseous lesions were observed and successfully resected. Histopathological examination of the lesions was consistent with osteoma. Intracranial osteoma is a very rare benign neoplasm of the mature bone tissue. The typical clinical manifestation of intracranial osteoma is a chronic headache. Head CT shows a well-defined, hyperdense structure. However, this is often mistaken as calcified meningioma. Headache is one of the most frequent complaints in the outpatient department. The types of headaches can be broadly classified into primary and secondary. The primary headaches have benign intrinsic causes and include tension, migraine, and cluster headaches . A detaiA 33-year-old woman presented to our primary care clinic with three days history of worsening frontal headache. She had been experiencing this headache daily for the last three months; however, the current episode was more severe. She described the headache as pressure across her head. The headache was bilateral and was not radiating. She reported not having any warning signs before the headache. The headache was not related to the menstrual cycle or her diet. However, the headache worsened when she was stressed. It was not awakening her from her sleep. She scored it as 6 out of 10 on the severity scale. The headache episode was not associated with fever, neck stiffness, or loss of consciousness. However, she often became nauseated with the headache. There was no history of weakness, numbness, or visual disturbances with the headache. She reported that the headache has significantly affected her quality of life. The past medical history was remarkable for diabetes mellitus that was well-controlled with metformin 1000 mg. She was not a smoker and never drank alcohol. There was no family history of migraine headaches. She worked as a school teacher and found her job very stressful.On examination, she was fully oriented to time, place, and person. No focal neurological deficit was noted. The tone and power were normal in both the upper and lower extremities. The coordination was intact, and the Babinski sign was negative. All examination findings were unremarkable. The patient was referred to undergo a head CT scan to rule out any space-occupying lesion, considering the chronicity of the pain. The scan showed the presence of two highly hyperdense foci in the frontotemporal region, one of them was related to the dura. Such foci were not causing midline shift or brain edema Figure . The preThe findings were discussed with the patient, and she was referred to the neurosurgery team for further management. Surgical excision of the lesions was planned. The patient underwent right craniotomy under general anesthesia. The two osseous lesions were observed. One lesion was attached to the dura mater, while the other had no dural attachment. Both lesions were successfully removed. Histopathological examination of the lesions consisted of mature bone Figure . The patWe reported the case of dural osteoma as a cause of chronic daily headaches. Osteoma is a benign tumor of mature bone tissue. In the head and neck region, osteoma more frequently involves the sinuses with attachment to the inner and outer tables of the skull bones . HoweverA limited number of cases have been reported about intracranial osteoma. It is reported to be more prevalent among women with a mean age of 42 years . It has Neuroimaging studies are essential to make the diagnosis. The presence of well-defined lesions with high attenuation on CT scans is the characteristic feature of osteoma. However, misdiagnosis of osteoma as calcified meningioma can occur, as in the present case. In fact, most of the reported intracranial osteoma in the literature were initially diagnosed as meningioma, and the correct diagnosis was made after the histopathological examination, which is the gold standard for diagnosis . HoweverIntracranial osteoma is a rare, benign neoplasm of the mature bone tissue. The typical clinical manifestation of intracranial osteoma is a chronic headache. Head CT shows a well-defined, hyperdense structure; however, this is often mistaken as calcified meningioma. Hence, radiologists should always remember intracranial osteoma as a differential diagnosis of calcified meningioma. A craniotomy is considered a safe and effective treatment for intracranial osteoma.


While colonic leiomyomas are common, leiomyosarcomas of the GI tract are rare. Increased mitotic rate, as well as lymph node involvement, portend a worse prognosis in leiomyosarcomas. They can arise anywhere along the GI tract, but anal canal occurrence is extremely rare.We present the case of a 75-year-old male diagnosed eight years prior with leiomyoma of the anorectal junction. There was a recurrence after endoscopic resection. He was referred to colorectal surgery due to symptoms of bleeding, skin irritation, anal pruritus, and rectal pain. On exam, he had a palpable mass at the dentate line. Workup revealed a 3.5 cm mass at the anorectal junction with pathology showing a leiomyosarcoma with moderate atypia and a high mitotic rate. MRI revealed invasion into the prostate. Robotic pelvic exenteration, including cystoprostatectomy, abdominoperineal resection, and ileal conduit, was performed. Final pathology results showed a grade 2 leiomyosarcoma invading the prostate and skeletal muscle and incidentally found Gleason 3+4 prostate cancer, pT2. A very small percentage of anorectal leiomyosarcoma cases were located in the anal canal. Surgery remains the best curative option, as chemotherapy and radiation data are limited. This rare tumor, which previously has not been documented to have degenerated from a benign rectal leiomyoma, was diagnosed as a result of close monitoring after previous local resections and cured by local resection and radiation. Stromal neoplasms that arise in the GI tract are grouped into two major divisions. The first is GI stromal tumors (GISTs) which are common and predominantly arise in the stomach and proximal small intestine. The second group comprises several neoplasms and includes leiomyomas, leiomyosarcomas, desmoid tumors, schwannomas, and peripheral nerve sheath tumors .Overall, leiomyomas of the GI tract are uncommon and leiomyosarcomas are quite rare. Although leiomyosarcomas can resemble GISTs, they show features of smooth muscle differentiation and generally are an aggressive tumor with cellular atypia, abundant mitosis, and include areas of necrosis . LeiomyoColonic leiomyosarcomas are highly aggressive tumors irrespective of their tumor size or mitotic activity. On the other hand, rectal leiomyosarcomas are generally small and have prolonged patient survival with an overall more favorable prognosis. This is likely secondary to the polypoid nature of the tumors, which allows for easy palpation on rectal exams and sooner discovery by physicians . Only foWe present the case of a 75-year-old male who was referred to the colorectal surgery clinic for evaluation after noting hematochezia, anal skin irritation, and rectal pain. Eight years prior, he had been diagnosed with leiomyoma of the rectum. At that time, he had undergone a biopsy of the lesion and local resection was performed several times. After each resection and biopsy, the pathology proved to be a benign leiomyoma. Upon his latest presentation, eight years after initial local resection, a physical exam revealed a palpable mass at the anorectal junction. Biopsy of the mass was now consistent with leiomyosarcoma, including moderate atypia and a high mitotic rate (40 per 10 high power field), as compared to his previous diagnosis of leiomyoma (Figures He underwent staging workup with an MRI of the pelvis Figure . This prThe final pathology resulted in a grade 2 leiomyosarcoma that invaded the prostate and skeletal muscle. Zero of 26 lymph nodes were positive. Incidentally, he was found to have Gleason 3+4 prostate cancer that was staged as pT2. The postoperative course was uncomplicated and he was discharged home from the hospital.As an outpatient, he completed adjuvant radiation therapy to prevent local recurrence of his leiomyosarcoma. Complete surgical resection cured his prostate cancer, but urology will continue to monitor him for recurrence with interval PSA levels. As for monitoring of his leiomyosarcoma, he will continue to undergo regularly scheduled physical exams and imaging to assess the recurrence or development of metastatic disease. He is currently free of disease 18 months after surgery based on his most recent MRI scan and physical exam.Our case represents a rare disease process of anorectal leiomyosarcoma. Leiomyomas are more commonly found in uterine tissue, but can occur in any location with smooth muscle cells. Degeneration of leiomyomas has been described in uterine tissue, once again, but due to its rare nature, it is less well described in the anorectal region . In thisAccording to a 2019 systematic review of the literature for the last 20 years, there were only a total of 51 cases of anal or rectal leiomyosarcoma reported. This is estimated to be less than 0.1% of all cases of anorectal malignancies. Of those 51 reported cases of anal and rectal leiomyosarcoma, only four cases, or 8%, were located solely in the anal canal . This caSurgical resection with negative margins is the best curative option. Local resection or endoscopic treatment for benign leiomyomas is reasonable, as was performed previously, but the leiomyosarcoma needs to be addressed with radical resection. The disease progresses by local spread or invasion and does not tend to travel to lymph nodes. In cases of R1 or R2 resection, re-excision is indicated since positive margins are a predictor of local recurrence. Local recurrence after wide local excision was cited as having a 30% recurrence rate compared to radical resection which had a 20% rate. Radical resection and extensive surgical procedures such as en bloc resection of pelvic exenteration were reserved for tumors invading adjacent organs, as in our patient. Interestingly, distant metastasis was found to have a higher incidence in patients who underwent radical resection [Regardless of the treatment approach, metastasis of leiomyosarcomas were cited at an overall rate of 51.61%. Characteristics such as a mitotic rate greater than or equal to 10/10 high power field, high tumor grade, and/or a tumor size larger than 10 cm were associated with a higher risk of metastasis and worse survival. Lymphadenectomy is not an indicated procedure during resection as leiomyosarcomas do not generally metastasize to lymph nodes. However, if local regional lymph nodes are enlarged on preoperative imaging, then resection is indicated. Data on the use of chemotherapy and radiation is limited. Neoadjuvant radiation is thought to help with the R0 resection of the tumors compared to adjuvant radiation. However, patients with local invasion often undergo radiation therapy afterward, as seen in our patient, to prevent a recurrence. Additionally, those cases that received adjuvant chemotherapy had better rates of overall survival compared with those that did not. Although multiple trials have assessed different regimens, no regimen has yet to be proven to be the top choice for leiomyosarcomas of the abdomen. Doxorubicin-based regimens remain the standard first-line choice of chemotherapy regimen for metastatic or locally advanced soft tissue sarcomas .Leiomyomas are rare neoplasms of the GI tract and particularly of the anal canal. Their malignant counterpart, leiomyosarcomas, behaves aggressively and can spread locally to other organs. They are best worked up with MRI or endorectal ultrasound to determine the depth and possible invasion. They are best treated with radical surgery to resect any local disease. Discussion with a multidisciplinary tumor board should address the possibility of radiation and chemotherapy as an adjunct to the surgical plan. Overall survival is poor, but early detection and aggressive therapy lead to the best outcomes.


Ependymomas are primary glial tumors arising from cells related to the ependymal lining of the ventricular system. They are classified into at least nine different molecular subtypes according to molecular phenotype, histological morphology, and tumor location. Primary sellar ependymoma is an extremely rare malignancy of the central nervous system, with only 12 known cases reported in humans. We herein report a case of ependymoma located at the pituitary region in a 44-year-old female patient and discuss the molecular subtype, natural history, clinical presentation, radiological findings, histological features, immunohistochemical characteristics, ultrastructural examinations, treatment, and prognosis of sellar ependymoma. This case report may serve as a helpful reference for clinicians and radiologists in clinical practice. Ependymomas are relatively uncommon primary glial tumors, accounting for 2% to 6% of the central nervous system (CNS) tumors , that usA 44-year-old female was admitted to the Neurosurgery Department with a 7-month history of intermittent headaches, secondary amenorrhea, and visual blurring. The patient reported not having galactorrhea, hyponatremia, hyposexuality, weakness, or any other sensory or motor dysfunctions. A review of the family history indicated no history of tumor or cancer in close family members. Moreover, neuro-ophthalmologic examination disclosed bitemporal hemianopia, which was worse in the right eye, and no papilledema was found. A Snellen chart indicated a 0.4 value in the unaided right eye and 0.7 in the left eye. A preoperative laboratory examination showed a notable increase in the prolactin (PRL) level  and no evidence of pituitary dysfunction or hypoadrenocorticism.MRI scanning revealed a 1.94×1.91×2.16-cm  well-defined mass located in the sellar and suprasellar regions of the brain (Knosp II and Hardy-Wilson Grade III), with a noticeable expansion of the sella turcica. Furthermore, the tumor was isointense on the T1-weighted imaging  Grade II classification. Moreover, gross specimen sections showed a gray-brown, soft, and friable mass. When examining hematoxylin and eosin (H&E)-stained paraffin sections, we observed characteristic and well-formed perivascular anucleate zones (pseudorosettes) and (true) ependymal rosettes composed of bland cuboidal or columnar tumor cells  and ST-EPN-YAP1  . ST-EPN-YAP1 occurs primarily in infants and children and usually has an excellent outcome after surgery combined with radiotherapy  , 30. ST- fusion) . RELA-C1EPN-RELA . Moreoveotherapy , 27, 30.otherapy , 27. How1q amplification (15-20% PF-EPN) and the absence of H3K27me3 trimethylation are considered to be associated with a poor prognosis  was classified into two molecular subtypes of PF-EPN-A and PF-EPN-B, according to the methylator phenotype of the promoter CpG island . The forrognosis , 36, 37.rognosis . Some PFrognosis . The promutation . Howeverrognosis , 40, necrognosis .EPHB2 amplification and INK4A absence ] or cystic-solid  (%) 8, 13, with thThe mean outset age of SE has been reported to be approximately 46.9 years of age range: 31-81). In our analysis, we found two female patients and 11 male patients, suggesting a male inclination, consistent with previous reports, suggesting that males have a higher incidence of ependymoma occurrences than females -81. In o, with soThe rarity of SE has led to limited radiographic characteristics, and we have summarized imaging analysis of all 13 clinical cases discussed in this study in We summarized the pathological examination of all 13 cases, including our case in ), histological examination and immunohistochemical analysis of the patient were not straightforward. In difficult-to-diagnose cases, ultrastructural analysis by electron microscopy is imperative.Immunostaining analysis provides additional significant information for the correct diagnosis of ependymomas, with positive expression levels of TTF-1, GFAP, S-100, vimentin, EMA, and negative expression levels for secreted pituitary hormones and neural markers, such as synaptophysin , 18, 21.We found that electron microscopy examination was carried out in 3 cases  of SE , 10, 14.The different diagnoses obtained are quite extensive, including pituitary adenoma, craniopharyngioma, chordomas, Rathke’s cleft cyst, meningioma, glioma, and aneurysms, among others , 14, 53.Despite advances in many other tumor types in past decades, maximal safe tumor resection followed by postoperative adjuvant radiotherapy remains the most viable treatment modality for ependymomas , 54, 55.The use of radiotherapy modalities to treat ependymoma remains controversial, especially in children . WhippleChemotherapy has not been a routine treatment paradigm for ependymomas, and no patient with SE has been reported to undergo chemotherapy . Howeverin vitro and in vivo tests  for the publication of any potentially identifiable images or data included in this article.LZ and YJ made study design, data collection, data analysis and interpretation, and composed the manuscript and literature review. YL and YW were the surgeon that performed the surgery and did data collection, data analysis, and interpretation. LL and YB made English and grammar corrections, critical revisions, and approved final version. LZ made great effort in the modification of the article. LZ and YL had the acquisition, analysis or interpretation of data for the work, revising it critically for important intellectual content, final approval of the version to be published, and agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Retroperitoneal sarcomas are considered a rare type of malignant tumors, accounting for fewer than one-fifth of all cases. Among all malignancies, the incidence of soft tissue sarcomas is approximately 1%. It constitutes 38% of soft tissue sarcomas in liposarcomas. Putting it that way may better indicate that liposarcomas are a rare tumor. They are frequently asymptomatic until they become large enough to compress the adjacent organs. As a result, it must be validated using appropriate imaging modalities, for example, computed tomography (CT) abdomen or magnetic resonance imaging (MRI) abdomen with contrast. The preferred treatment is complete surgical resection.The patient presented with vague symptoms. She had a CT abdomen with contrast, as well as an MRI abdomen. Both images revealed the presence of a giant sarcoma displacing the pancreas and left kidney posteriorly and the transverse colon inferomedially. The patient underwent en bloc resection of the mass with distal splenopancreatectomy. The patient tolerated the procedure well and was discharged on day 6 postoperatively in a stable state.Giant liposarcoma is a rare and aggressive form of sarcoma. Because of the unique presentation, it is difficult to diagnose clinically. CT scans with MRIs are viable imaging modalities for determining tumor extent and ruling out any vascular invasion. Complete surgical resection of liposarcoma is a treatment of choice. En bloc resection of retroperitoneal sarcoma with distal splenopancreatectomy can be performed successfully and safely. Retroperitoneal sarcomas are considered a rare type of malignant tumors, accounting for less than one-fifth of cases. Liposarcoma is the most prevalent kind of sarcoma, accounting for 40% of all cases .They are frequently asymptomatic until they become large enough to compress the adjacent organs, thus producing symptoms . TherefoRetroperitoneal liposarcoma involving the spleen and tail of the pancreas has been reported in few studies -7. FurthThis is a case of a giant retroperitoneal liposarcoma including the spleen and tail of the pancreas that was effectively treated with en bloc resection.Case history and examinationA 36-year-old female presented to the clinic with a six-month history of abdominal distension. Examination revealed abdominal distension and ill-defined mass.A CT chest, abdomen, pelvis for staging was done Figures , 2: fat-Thus, an MRI abdomen and pelvis with contrast Figure  was doneThe case was discussed on the tumor board and agreed that the tumor was most likely G1 liposarcoma that needed oncological excision of retroperitoneal liposarcoma with distal splenopancreatectomy. The patient received presplenectomy immunization .Operation and postoperative periodThe patient was admitted for surgery. Preoperative lab tests  were normal. Antibiotic prophylaxis was given and an epidural catheter was inserted.OperationShe underwent en bloc resection of the retroperitoneal tumor with distal splenopancreatectomy. Medline laparotomy then exploration showed a retroperitoneal tumor that occupied 70% of the abdominal cavity with transposition of the viscera to the right side, beginning at the lower border of the tumor and dissection from the transverse colon, entry to the bursa omentalis, and identification of the inferior mesenteric vessels retroperitoneally, then further dissection from the caudal to cephalic identifying the left ovarian vein and ureter and both structures followed until the kidney hilum. The mass capsule was surrounding the hilum without invasion. Then, medial dissection up to the inferior pancreatic border was done, which was engulfed by the tumor and could not be easily separated. Then, lateral mobilization of the tumor en bloc with the spleen. This was followed by transection of the gastric greater curvature vessels and identification of the splenic artery and vein on the superior border of the pancreas, which was also engulfed by the mass and disappear in the mass completely.After transection of the pancreas tail, the splenic artery and vein were resected. After complete medial mobilization, lateral mobilization was completed by dissection of the mass from the left kidney, adrenal, and diaphragm. The mass was retrieved with an intact capsule and sent for histopathological examination Figure . Two J-VShe was placed postoperatively on analgesia, anti-emetic and anticoagulant prophylaxis.She was followed up daily. Blood sugar readings were normal during the hospital stay, and there were no attacks of hypoglycemia. In the postoperative phase, laboratory tests were unremarkable. Procalcitonin, amylase, and lipase levels were normal on days 3 and 5 postoperative.The patient felt attacks of dull abdominal pain upon eating in the left upper quadrant. As a result, an ultrasound of the abdomen and pelvis was performed and showed a left upper abdomen encysted turbid and multiseptated collection measuring 73 x 52 mm diameters and approximately 129 mL volume. The JVD was kept in place and was started on ondansetron regularly. On the sixth postoperative day, the patient was discharged on proton pump inhibitors, analgesia, and antibiotics. The patient was followed one week in the clinic after discharge and it was unremarkable.Histopathological examination revealed a neoplasm that consisted of adipose tissue with slightly variable in size fat cells and rare fibrous bands with spindle cells that exhibited no atypia. All margins were involved by neoplasm. There was no pathological alteration in the spleen. Mouse double minute 2 homolog (MDM2) was focally weak +ve, and cyclin-dependent kinase 4 (CKD4) was -ve. Diagnosis confirmed a low-grade liposarcoma.Retroperitoneal sarcomas are rare malignant tumors that account for 15% of all sarcomas. Among all malignancies, the incidence of soft tissue sarcomas is approximately 1%. It constitutes 38% of soft tissue sarcomas in liposarcomas . Our casLiposarcomas tend to grow to very large sizes before they produce symptoms due to large retroperitoneal space. When diagnosed, almost half of retroperitoneal sarcomas are larger than 20 cm in size ,10.The most often utilized imaging modality for sarcoma diagnosis, staging, and preoperative evaluation is CT ,12. ImagSeveral prognostic variables for survival and distant and local recurrence have been identified. Complete surgical resection with negative margins is the most powerful prognostic factor . SeveralFurthermore, histologic grade, which reflects the degree of differentiation, continues to be a crucial component in clinical course and prognosis . The fivGiant liposarcoma is a rare and aggressive form of liposarcoma. It is hard to diagnose clinically due to atypical presentation. CT scans with MRIs are viable imaging modalities for determining tumor extent and ruling out vascular invasion. The treatment of choice is complete surgical en bloc resection of retroperitoneal sarcoma with distal splenopancreatectomy, which can be performed successfully without complications. However, to detect any vascular invasion, a rigorous preoperative assessment is required.


Progressive multifocal leukoencephalopathy (PML) can rarely occur in Multiple Sclerosis (MS) patients undergoing dimethyl fumarate (DMF) treatment. Our case stresses the limits of current diagnostic and stratification risk criteria, highlighting the potential role of Magnetic Resonance Imaging (MRI) in advising clinical choices.A 54 years old MS male patient treated with DMF, after 3 years of clinical stability developed a subacute clinical worsening. He had no severe lymphopenia but MRI signs suggestive of a coexistence of PML and MS activity. Although his viral title was negative, DMF was discontinued, with clinical and radiological improvement.This case highlights the challenges behind PML diagnosis, especially in patients not fulfilling the risk stratification criteria and that might present with concurrent disease activity, stressing the potential role of MRI in informing therapeutic decisions. Progressive multifocal leukoencephalopathy (PML), caused by the John Cunningham polyomavirus (JCV), represents a rare but known safety risk under dimethyl fumarate (DMF) treatment . So far,A 54 years old Relapsing-Remitting MS male patient presented to the Neurology Department in July 2019 for subacute clinical worsening. He had been diagnosed in September 2016,and treated with DMF since then. Along with DMF, he had started mirtazapine treatment for concurring depression. From the disease onset, the patient had no signs of disease activity, presenting minor stable clinical findings at the neurological examination , characterized by slight hands telekinetic tremor and mild lower limbs hypoesthesia. His absolute lymphocyte counts (ALC) under treatment always ranged between 623 and 678 cells/μL.His neurologic examination in July 2019 showed cerebellar ataxia, dysarthria and lower limbs weakness (EDSS = 3.5). Brain MR showed imaging features highly suggestive for PML overlapping with an enhancing MS lesion. In particular, compared to the previous MR exam, several new hyperintense and enlarging lesions were found on Fluid Attenuated Inversion Recovery (FLAIR) images, in absence of restricted diffusion. Among these, 4 confluent hyperintense lesions, with involvement of the U-fibers,were present, with 3 showing a punctate peripheral gadolinium enhancement along with a hypointense subcortical rim at the Spoiled Gradient Recalled (SPGR) sequence, while 1 lesion showed a well-defined area of contrast enhancement without SPGR hypointensity.Representative imaging findings are shown in Fig. ALC on admission was 620 cells/μL, while cerebrospinal fluid (CSF) proteins and cells were unremarkable. JCV-PCR (Polymerase Chain Reaction) in two CSF samples, obtained from the same spinal tap but analyzed in different laboratories, resulted negative. Extensive microbiological tests, including HIV, were unremarkable. Given the mild clinical course, no brain biopsy was performed. DMF therapy was discontinued, while mirtazapine administration was carried on.In January 2020 a brain MR demonstrated stable MS lesion load, with resolution of PML signs and no evidence of immune reconstitution inflammatory syndrome. ALC ranged between 790 and 950 cells/μL after treatment discontinuation. The patient showed a clinical resolution of gait ataxia and dysarthria, with a stability of the hand telekinetic tremor and mild lower limbs hypoesthesia. At this point, interferon-beta-1a treatment was started, with clinical and radiological stability.The concomitant occurrence of PML and disease activity in MS is a rare but possible event, and, in this case, prompt recognition is necessary to inform therapeutic decisions. Here we report the case of a “possible PML” overlapping with MRI MS activity in a patient under DMF without severe lymphopenia. Although in 2020 the European Medicines Agency recommended intensification of controls in patients with sustained lymphopenia, especially when concomitant risk factors for PML are present , in 2019 PML risk stratification under DMF treatment was still based on age and lymphopenia levels, with moderate lymphopenia  prompting a re-evaluation of the patients’ risk-benefit balance. In our case the risk of PML and the possibility to switch to another first-line therapy were discussed with the patient. Given the concomitant presence of hypertension, teriflunomide was not considered and the patient refused injectable drugs, which led to DMF continuation. When subacute neurological worsening occurred, in presence of moderate lymphopenia with brain MRI suggestive of PML, a spinal tap was performed.Routinely, PML diagnosis is based on the demonstration of JCV-DNA in the CSF, using PCR, in presence of suggestive features . Althoug6. Another new lesion presented completely different features, typical for an active MS lesion: ovoid shape, with well-defined contrast enhancement and margins. In our patient, despite the negativity of the spinal tap, in particular the identification of the highly specific punctuate pattern at MRI [In our case, although the PCR showed negative results, neuroimaging findings were highly suggestive of PML. Indeed, MRI showed 3 typical multifocal confluent subcortical FLAIR hyperintense lesions, different from the usual relapsing MS lesions. A subcortical U-fibers involvement was evident, with the gradient sequence showing a hypointense rim of the U-fibers adjacent to the white matter lesions. These features, coupled to a punctuate marginal contrast enhancement, are considered highly suggestive of PMLn at MRI  contribuGiven the relatively scarcity of reports about the development of DMF-associated PML in MS patients, no treatment or management guidelines are yet available. Nevertheless, growing importance has been recently given to MRI signs of PML in MS patients not only in the diagnosis, but also in the management of these patients. Indeed, a recent study suggested that the possibility to withdraw the treatment in case of radiologically suspected PML, even in those cases without a confirmation at the CSF analysis, and to perform a close imaging and clinical monitoring before starting a new MS therapy .2A-receptors, suggesting that 5HT2− antagonists as mirtazapine might improve PML course [Emerging studies reports clinical improvement attributable to an off-label use of mirtazapine in the treatment of PML, in HIV positive patients, autoimmune patients under immunosuppressive treatment and in natalizumab-associated PML , 12. In-L course . In thisIn conclusion, our case highlights how PML diagnosis remains challenging, especially in patients that do not fully meet the risk stratification or diagnostic criteria and that might present with concurrent disease activity. Although JCV demonstration is the main diagnostic criterion, JCV-PCR has proved to be not completely reliable and negative results cannot exclude PML diagnosis. In these cases, neuroimaging plays a central role in the diagnostic process and the presence of imaging features highly suggestive for PML should be thoroughly evaluated. In our case, an accurate evaluation of brain MRI scans was the key to the discrimination of MR MS activity from PML lesions, which in turn was crucial for treatment decisions. Imaging findings drove DMF discontinuation, notwithstanding the negative JCV-PCR, with clinical benefits.


The mediastinal malignant germ cells tumor represents less than 0.5% of thoracic tumors, although the mediastinum is one of the main extragonadic locations of these tumors. In the majority of cases, young people are those most affected. The prognosis of mediastinal malignant germ cells tumors is poor, especially non-seminomatous germ tumors. In this article, we report a rare case of a young 19-years-old patient treated for a mediastinal germ cell tumor of yolk sac. The patient presented a chest pain; the chest computed tomography (CT) showed a right paramedian mediastinal mass with a pleural effusion associated with supraclavicular and cervical lymph nodes. Biopsy revealed a non-seminomatousgerm cell tumor of yolk sac. The exams showed elevated alpha-fetoprotein (AFP), without any meaningful elevation of other serictumor markers. The patient received 4 cycles of chemotherapy based on etoposide, ifosfamide and platinum salts then a complete excision of the mass. These tumors produce significantly alpha-fetoprotein (AFP) [Germ cell tumors (GCT) are rare tumors that arise from primordial germ cells. They are classified into two main categories: seminomas and non-seminomas and are usually located in gonadal sites (testis in men) . The yolin (AFP) , 2 and aWe report the case of a 19 years old patient who presented at the Hassan II University Hospital with a 3 months history of chest pain and dyspnea. The initial clinical examination revealed a slight reduction in vesicular murmurs in the right lung field, and a left cervical lymph node. The X-ray thoracic radiography revealed a non-compressive tracheo-bronchial opacity with para-hilar calcification. Chest tomography (CT) scan revealed a voluminous right paramedian mass measuring 170 mm of diameter, with significant central calcification and infiltration of the pulmonary aorta and left pulmonary parenchyme; the left subclavicular lymphe nodes measuring 25 mm and a medium pericardial effusion were also found .The biop2 days 1 to 5), Ifosfamide (1500 mg/m2 days 1 to 5), Uromitexan (1500mg/m2 days 1 to 5) and Cisplatin (20mg/m2 days 1 to 5) with 480 micrograms subcutaneous daily injection of filgrastim during 5 consecutive days. Biological assessment revealed an AFP decrease. Radiological evaluation objective a partial response according to the Response Evaluation Criteria in Solid Tumours (RECIST) version 1.1. A residual mass measuring 100mm has been shown. The patient underwent a surgical intervention, based on complete excision of all residual anterior mediastinal tumors with resection of the phrenic nerve. The anatomopathological examination revealed a total and massive necrosis of the tumor with contingent of mature teratomas with no viable cells. The patient is in complete biological and radiological remission without any evidence of recurrence after 42 months of follow up.The patient received 4 cycles of chemotherapy based (VIP): Etoposide (75mg/mGerm cell tumors (GCTs) are classified as extragonadal if there is no evidence of a primary tumor in the testes. These tumors include seminomas, non-seminomatous GCTs, mature teratomas, and immature teratomas based upon histology . About 2Histologically, the histogenesis of extragonadal YST is not well defined, several proposed hypotheses can explain the existence of germ cell tumors in extragonadal sites , 8, in oClinically, mediastinal germ tumors usually manifest as a dyspnea (25% of cases) with chest pain (23% of cases). Other described symptoms include, according to an international study reporting 381 mediastinal GCTs, fever (13%), night sweats or weight loss (11%). Fatigue, hemoptysis and superior vena cava syndrome were seen in less than 10% of patients with mediastinal GCT . SimilarOn the biological level, high rates of AFP are usually objective in the YSTs at the moment of diagnosis and they significantly decrease after treatment. Thus, it is considered as a predictive marker for therapeutic response. Similarly to extragonadic non seminomatous GCTs, mediastinal YSTs carry a poor prognosis with 40-50% overall survival and puts this entity in the poor prognosis group according to the International Germ Cell Cancer Collaborative Group (IGCCCG). Systemic treatment of YSTs is based on the same chemotherapy regimens that are used for patients with advanced, intermediate- or poor-risk gonadic GCTs. However, VIP is preferred, rather than BEP regimen for the mediastinal sites.Actually mediastinal GCTs are typically candidates to thoracotomy to excise the residual disease. This may require prolonged exposure to high partial pressures of oxygen during surgery and lead to bleomycin-related pneumonitis. Indeed, in a retrospective study reporting 221 patients who underwent thoracic surgery, 13% of patients treated with BEP developed acute respiratory distress syndrome versus 0% with VIP. There were 6.6% of post operative death with BEP mainly related to pulmonary failure, versus no deaths in those treated with VIP . After cThe case presented in this paper shows the importance of multidisciplinary approach which offers the best chance of survival to these patients; in our case the combination of chemotherapy and surgery with complete exercise resulted in complete remission of the disease, our patient achieved a 42-month recurrence-free survival.


Gastrointestinal stromal tumors rarely occur in children, but when they do, their biological behavior and histopathological patterns differ from those of adults.A 13-year-old boy with a gastrointestinal stromal tumor was characterized by a rare genetic mutation. The patient complained of “fatigue with intermittent abdominal pain for 1 month”. According to the preoperative imaging examination, gastroscopy, and gastroscopic biopsy, the patient was diagnosed with a gastric stromal tumor. Postoperative pathology showed that the tumor cells were fusiform and ovoid, and mitotic figures were easily seen. Immunohistochemistry revealed that the tumor was S-100(+), SOX10(−), CD34(+), SMA, DOG-1(+), CD117(+), KI-67 (positive for 20% + of the subjects and 40% + of the hotspots), and SDHB(−). Genetic tests showed missense mutations in ALK and TSC1. With surgical treatment, the tumor was completely removed. The patient recovered well and was discharged on the ninth day after the operation. He is currently under follow-up.In this case involving a patient with a gastrointestinal stromal tumor, immunohistochemistry indicated that the tumor was an "SDH-deficient type", and gene detection showed no KIT or PDGFRA mutation but rare ALK and TSC1 mutations, which adds to the knowledge of the types of gene mutations in children with gastrointestinal stromal tumors.The online version contains supplementary material available at 10.1186/s12893-021-01208-0. A gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor in the gastrointestinal tract . GISTs aA 13-year-old boy patient was admitted for "fatigue with intermittent abdominal pain for 1 month". In the prior month, the patient presented with fatigue symptoms without obvious incentives. However, intermittent paroxysmal abdominal pain and dizziness occurred after exercise, without abdominal distension, nausea, vomiting, acid reflux, and belching. The symptoms had no obvious periodicity or regularity. Before admission to our hospital, a routine blood examination in another hospital revealed a hemoglobin of 63 g/L, and abdominal enhanced CT showed gastric antrum surrounding a mass, thought to be either a stromal tumor or a lymphoma. The patient was physically fit, without any history of disease and surgery. No family history of illness. Physical examination on admission revealed the appearance of anemia, a flat abdomen, no varicose abdominal wall, no peristaltic waves, and no masses; the abdominal wall was soft, with no tenderness, rebound pain, or muscle tension. The liver, spleen, and subcostal areas were not palpable; no abnormal masses were palpated in the abdomen. Anemic tremors and water sounds were observed. There was no percussive pain in the liver and kidney areas. The number of bowel sounds is 4 per minute.2, leading to consideration of a mesenchymal tumor, either (1) neuroendocrine, (2) glomus, (3) stromal, or (4) other; and 2. a small amount of pelvic fluid  of high risk. No tumor was observed in "duodenal incision margin, fracture end of the suture, and gastric fracture end on the opposite side of suture". Immunohistochemical results (both positive and negative controls were set): SDHB (−)  Fig. , and tumGenerally, GISTs are dominated by KIT and PDGFRA mutations and mainly occur in adults . GISTs wIn this case, the patient’s GIST was SDHB(−), and missense mutations of ALK and TSC1 were observed, which have not been reported in previous studies. At the 2-month follow-up, when we wrote this report, the patient was healthy, without recurrence or metastasis. ALK tyrosine kinase inhibitors may be recommended if recurrence and metastasis are found during subsequent follow-up.Additional file 1: e-Fig. 1 Immunostaining reveals that the mass was positive for S-100(× 200)Additional file 2 : e-Fig. 2 Immunostaining reveals that the mass was negative for SOX10(× 200)Additional file 3: e-Fig. 3 Immunostaining reveals that the mass was positive for CD34(× 200)Additional file 4: e-Fig. 4 Immunostaining reveals that the mass was partially positive forSMA (× 200)Additional file 5: e-Fig. 5 Immunostaining reveals that the mass was positive for DOG-1(× 200)Additional file 6: e-Fig. 6 Immunostaining reveals that the mass was positive for CD117(× 200)Additional file 7: e-Fig. 7 Immunostaining reveals that the mass was positive for KI-67(× 200) in 20% of the subjects and approximately 40% of the hotspots


Superior mesenteric artery (SMA) pseudoaneurysm is a very rare condition, typically associated with trauma, inflammation, and infection, and as a post-operative complication. If left untreated it can lead to serious consequences such as rupture and fatal haemorrhage.A 17 year old male presented to the emergency department with a history of intermittent progressive epigastric pain with no preceding significant symptoms of a possible cause. He was initially treated conservatively until the intensity of pain was so severe an abdominal computed tomography (CT) scan was justified. A pseudoaneurysm of the SMA was found. Full inflammatory and immunological workup was unremarkable. Repeat CT scan showed the SMA pseudoaneurysm was larger, mandating surgical intervention; the vascular surgeon suggested an exploratory laparotomy. Intra-operatively, unexpectedly, a wooden foreign body measuring 5.0 × 0.3 × 0.5 cm was seen once the aneurysm sac was opened. The pseudoaneurysm was repaired and the abdomen closed after ascertaining that all other organs were intact. The patient had a simple recovery with no complications and was discharged home. The follow up CT scans were unremarkable.Pseudoaneurysm of the SMA in the paediatric age group is an extremely rare and life threatening phenomenon. The clinical presentation may be subtle, leading to delayed diagnosis. Early surgical intervention may be lifesaving and prevent further complications. •Pseudoaneurysm due to swallowed foreign body is an extremely rare pathology.•Visceral artery pseudoaneurysm are usually silent but potentially fatal.•Surgical intervention is mainstay treatment of visceral artery pseudoaneurysm. Visceral artery aneurysms and pseudoaneurysms (VAPA) are rare pathological conditions with an estimated prevalence of 0.1%–2%,The present study is the case report of an adolescent who presented with a superior mesenteric artery (SMA) pseudoaneurysm induced by an ingested foreign body, which was managed surgically.A 17 year old male with no significant past medical history presented to the emergency department (ED) with a sudden onset of severe abdominal pain located mainly in the epigastric region radiating to the back, which was associated with nausea and vomiting. During examination, there was generalised tenderness on deep palpation of the abdomen. There was no guarding or rigidity, and McBurney's sign was negative. Blood investigations and abdominal ultrasound were unremarkable. Therefore, the patient was sent home once his pain was alleviated by analgesics.One week later, the patient re-presented to the ED with a similar presentation. On questioning, he denied being pain free since his previous visit to the hospital. Blood tests found a slightly elevated C reactive protein , the results of other tests were normal, including neutrophil count (3.4 g/L). An abdominal computed tomography (CT) scan with intravenous (IV) and oral contrast was performed, which revealed abnormal fat infiltration at the root of the mesentery with sub-centimetre lymphadenopathy with non-specific features. The SMA had an irregular demarcation, and radiological features were suggestive of a possible SMA pseudoaneurysm  and therefore broad spectrum antibiotic therapy (ceftriaxone 2 g IV once daily) was initiated. A full septic workup was unremarkable except for an elevated CRP (83 mg/L). Serial blood workup found a progressive decrease in haemoglobin from 11.2 g/dL to 8.7 g/dL within 24 hours; thus, an abdominal CT scan was repeated on day 2 post-admission, which found a significant SMA pseudoaneurysm, the greatest diameter of which was 7 mm on axial section and 15 × 4 mm on coronal section, associated with abnormal infiltration of the mesentery around the aneurysm sac , which were all negative.The abdominal CT angiogram was repeated on day 7 of admission and showed an increase in the size of the SMA pseudoaneurysm to 8 mm on axial section and 20 × 8 mm on coronal section .Histopathology examination reported a foreign body of vegetable origin (a piece of wood) measuring 5.0 × 0.3 × 0.5 cm . After pStreptococcus anginosus, Aggregatibacter aphrophilus, and Enterobacter cloacae). The antibiotic regimen was modified according to the bacterial sensitivities to ceftriaxone 2 g IV once daily and levofloxacin 500 mg orally twice daily, which was continued for one month. The patient made a good recovery. A follow up abdominal CT scan done five days post-operatively found no signs of intraperitoneal collection or abscess formation, and with good SMA patency. He was discharged home on day 8 post-operatively in a stable condition. The patient was seen on a follow up visit after five months, with the CT angiogram showing an intact SMA arterial wall with no aneurysm recurrence. The patient was pain free.Bacterial culture of the aneurysm sac isolated three species of bacteria (,Pseudoaneurysms due to swallowed foreign bodies are rare. The aortic arch has been found to be the most commonly affected site.SMA aneurysms and pseudoaneurysms typically occur on the proximal 5 cm of the SMA.Most patients are asymptomatic, but some may present with abdominal pain, nausea, and vomiting. SMA aneurysms are usually found incidentally when performing abdominal imaging for other reasons.In most cases, surgeons opt to repair pseudoaneurysms because of the high mortality rate if left untreated. Complications associated with VAPAs include rupture, acute thrombosis, or embolisation to small bowel vessels.In this case, the patient presented ambiguously with recurrent abdominal pain of increasing intensity and high grade fever. Serial abdominal CT scans revealed an enlarging SMA pseudoaneurysm, which justified exploratory laparotomy with pseudoaneurysm repair. On opening the aneurysm sac, a wooden foreign body was found, which explained the clinical manifestation. The hypothesis is that the patient accidentally ingested the foreign body, which then descended the gastrointestinal tract to the stomach/small bowel traversing its wall towards the SMA, eventually settling within the arterial wall, and, over a period of time, evolving into a localised infection leading to pseudoaneurysm formation. However, it cannot be excluded that chemical or mechanical arterial damage caused by the foreign body may also have contributed to the pathological condition.None.None.


Langerhan's cell histiocytosis is an uncontrolled proliferation of dendritic cells. The involvement of skull base is rare. Variable clinical presentation and multi organ involvement often warrant a multidisciplinary approach for a successful diagnosis. We are reporting a case of 16-year-old male with sphenoid sinus Langerhan's cell histiocytosis which presented as a sudden and painless loss of vision. It is a rare entity in the diagnosis of blindness. Delayed diagnosis and treatment can result in serious complications. The radiological features and management options are discussed with a review of the pertinent literature. Non ocular lesions also cause monocular vision loss when it arises from close structures. Non ocular lesions are easily missed due to its non specific symptoms as well as complex anatomical location. Sphenoid sinus lesions can cause vision loss due to its close relationship with optic nerve. Optic neuropathy from sphenoid sinus may arise from the spread of sinus inflammation and infection, compression by an expansible lesion, neoplasm or ischemia.5The clinical presentation of sphenoid sinus lesions involves vague headaches and may be associated with purulent rhinorrhoea, retropharyngeal drip, nasal obstruction, abnormal vision and nerve deficit. Inflammatory conditions appear to be the major cause of sphenoid sinus lesions accounting for 65-72% of cases, followed by neoplasm  accounting for 16 to 17.5%.6 As our case had a rare tumor in an uncommon location with a vague presentation and normal nasal endoscopy, the diagnosis was made in a dilemma.Langerhan's cell histiocytosis (LCH) is a rare neoplasm and occurrence in the sphenoid sinus is very rare. Isolated sphenoid sinus LCH lesions most commonly present with headache, followed by ophthalmological and nasal symptoms. Delayed diagnosis may occur due to its nonspecific symptoms. Ophthalmologist plays a vital role in suspecting non ocular causes of vision loss when they find normal ophthalmic examination. High index of suspicion is required as endoscopic examination can be normal despite sphenoid sinus lesion.A 16-year-old male presented with complaints of headache which was more on the right side and decreased vision in the right eye for one month. There was no history of trauma, sinusitis or any eye complaints in the past. Ear, nose and throat examination was unremarkable. Documented vision on right eye was 2/60 and relative afferent pupillary defect (RAPD) with normal left eye. Fundus/posterior segment examination were normal on both the sides. Extraocular movements were restricted on medial aspect on right eye grossly and not able to perform the visual field charting due to vision loss. Other Neurological examinations were normal and no signs of meningitis.CT showed ill-defined soft tissue with hyper dense foci seen in right sphenoid sinus with erosion of lateral wall and involving orbital apex .In MRI, lesion appears hypointense in T2/FLAIR and intense enhancement in post gadolinium contrast study .We had differential diagnosis of invasive fungal sinusitis, lymphoma and malignant neoplasm. We were in a dilemma whether to treat him with an empirical anti-fungal therapy before histological and microbiological confirmation in view of rapid vision deterioration. We performed an emergency endoscopic debridement of the lesion under general anaesthesia and sent specimens for fungal KOH, culture and histopathological examination. KOH smear was negative for fungus and histological features were suggestive of Langerhan's cell histiocytosis. Diagnosis of LCH was confirmed by H&E stain showing highly cellular, polygonal histiocytes with round nuclei and CD1a antigenpositivity  and 4.We referred him to a haematologist for further management. They performed a bone marrow biopsy which showed normal cellular marrow with no abnormal cells. Thus, the diagnosis of a solitary type LCH (SSLCH) was made. Unfortunately, he lost to follow up without taking treatment at our centre.8LCH is an uncontrolled proliferation of dendritic cells with an undetermined aetiology. The clinical presentation of LCH is heterogeneous, ranging from mild disease with self-healing, solitary lesions (SS-LCH) to multisystem disease with fatal dissemination (MS-LCH) . Solitary lesion is the most common and 75% of cases occur before 20 years of age. The involvement of skull base is very rare and the temporal bone is the most common site. The delayed diagnosis may be rarity of lesion in skull base and vague symptoms. Variable clinical presentation and multi organ involvement often warrant a multidisciplinary approach for successful diagnosis.6In our patient, the solitary lesion extended to the sphenoid sinus, orbital apex, parasellar, sellar and the right cavernous sinus. In this region, the other radiological differential diagnosis that must be considered includes chordoma, chondroma, chondrosarcoma, meningioma, fibrous dysplasia, tubercular or fungalosteomyelitis, metastatic disease, primary Ewing's sarcoma, plasmacytoma, hemangioblastoma, giant cell tumor of the sphenoid.3 This may be the one of the reason for late diagnosis of retro orbital lesions. Whenever ophthalmologist encounters a vision loss patient with normal or mild fundus changes which is not correlating with vision loss, he/she should be sent to ENT / Neurosurgeon for evaluation of possible retro orbital causes.Vision loss is one of the serious complications of sphenoid sinus lesions. Most patients visit ophthalmologist for their vision loss, the examinations could be normal without any oblivious fundus changes and might look like primary optic neuritis.9Imaging in the form of CT / MRI with contrast should be done despite normal endoscopic nasal examination. Sphenoid sinus soft tissue in CT /MRI with vision loss should be biopsied to rule out possible fungal or neoplastic aetiology.Our patient had a MRI scan with contrast before visiting our hospital; this helped us to go ahead with an early endoscopic surgery. Intra operative frozen section and KOH smear study helped us in differentiating the fungal lesion from a neoplastic lesion as well as to avoid treating them with empirical anti-fungal therapy. In view of vision loss, most of the surgery was done in an emergency basis to prevent further deterioration of vision. The timing of surgery and the availability of these facility will improve the diagnostic approach as well as avoid empirical therapies.10 Surgical excision is the treatment for most of SS LCH but this may be not applicable in head and neck region due to functional and cosmetic aspects. In skull base, complete surgical excision may not be possible in sphenoid sinus lesions due to involvement of cavernous part of internal carotid artery, cavernous sinus and optic nerve. In our case, debridement of the lesion was done as much as possible and mainly on the optic nerve part for possible compressive effect. Other treatment modalities like steroid therapy, chemotherapy and radiation therapy can be offered for residual lesions. The prognosis is better in SS LDH compared with MS LDH and has been improved because of newer chemotherapeutic agents which are much tolerable with fewer side effects.11Histopathological examination shows large foamy, multinucleated histiocytes with deep cleft with reactive infiltration. Diagnosis is confirmed if stains are positive for CD1a antigen or Birbeck granules seen with electron microscopy.The limitations of this report are lack of outcome of the disease in this patient and follow up. Being a tertiary care centre, most of the patients travel from various places and not able to stay for long time due their financial issues as well as poor family support.None.JNMA Case Report Consent Formwas signed by the patient and the original is attached with the patient's chart.


Anomalies of the biliary system are frequently encountered. Agenesis of the gall bladder is a rare. The exact incidence is not known as most cases are asymptomatic and diagnosed incidentally during surgeries for unrelated conditions or at autopsy. This anomaly may occur alone or in association with other anomalies such as other biliary anomalies, portal vein anomalies and other vascular anomalies, or in some cases hepatic, gastrointestinal or anomalies in other body systems.An elderly man diagnoses with segment VI liver tumor and planned for surgical resection. During surgery we accidentally discovered gall bladder agenesis with slightly dilated common bile duct. The tumor resected successfully and the final diagnosis was well differentiated with hepatocellular carcinoma.Careful search for the gall bladder must be done before diagnosing agenesis of the gall bladder. No conversion is required provided adequate visualization and search for the gall bladder is done during laparoscopy. Postoperative MCRP will define the biliary anatomy and to help the surgeons to have postoperative accurate decision. Agenesis of the gall bladder is a rare anatomical anomaly of the biliary system, in which there a complete absence of the gall bladder and or cystic duct. The exact incidence is not known as the majority of cases are asymptomatic and many cases are diagnosed incidentally during surgeries for unrelated conditions or at autopsy. It is reported in some literature that the incidence range between 0.01 % and 0.075 % .This anomaly may occur alone or in association with other anomalies such as other biliary anomalies, portal vein anomalies and other vascular anomalies, or in some cases hepatic, gastrointestinal or anomalies in other body systems .Most cases are asymptomatic provided it is not associated with other anomalies, patients may complain from biliary symptoms for long time before being diagnosed either by radiology or more commonly during surgery. Right upper quadrant abdominal pain is the commonest presentation, other symptoms may include nausea and vomiting, fatty food intolerance, dyspepsia and attacks of jaundice .Most cases are missed during ultrasound examination which require high index of suspicion and great experience for the diagnosis. Hepato-biliary scintigraphy, cholangiography, CT-scan of the abdomen, endoscopic retrograde cholangio-pancreatography (ERCP), or magnetic resonance cholangio-pancreatography (MRCP) are greatly helpful and diagnostic, they show details of the anatomical structures and possibly show some of the associated congenital anomalies when present .If the diagnosis is made during surgery, it is important to confirm the condition and exclude the possibility of ectopic gall bladder, most patients reported improvement after surgery which have been explained to be attributed to release of adhesions in this region and reduction of the pain .The work of this report case has been reported in line with the SCARE 2018 criteria .2A 77-year-old man presented with dull aching right hypochondrial pain for 1 month duration. The pain was poorly localized and radiated to the back. The patient had no history of jaundice, fever, nausea, or vomiting. There was no history of reported weight loss.The patient had no history of chronic medical conditions. The past surgical history was negative.2.1Ultrasound of the liver revealed an evidence of 55*50 mm well defined iso-echoic mass with increased peripheral vascularity within the right lobe of the liver.CT-scan of the abdomen showed hypo dense soft tissue mass within segment VI of the liver. After intravenous contrast injection the mass showed vivid enhancement with rapid washout, hypo-attenuation was noted in the portal phase with visualization of a feeding vessel to the tumor, the CT-scan findings were suggestive for hepatocellular carcinoma. 9/L), and the platelets were normal (225*10*9/L). The blood sugar and the renal function test were normal. The serum levels of alpha-feto protein was normal (3.4 ng/mL), CA 19.9 was normal (20 U/mL), carcino-embryonic antigen was normal (4.62 ng/mL) and the CA 125 level was also normal (5.52 U/mL). The bleeding time and the coagulation profiles were normal.The hemoglobin level was normal (13.8 g/dl), the WBC were normal (10,500*10*2.2An extended right subcostal incision was done and exploration of the peritoneal cavity was done, there was evidence of 5*5 cm mass related to liver segment VI. During surgery we accidentally discovered gall bladder agenesis with slightly dilated common bile duct. The tumor resected using radiofrequency device and sent for histopathological study.The histopathological examination showed well differentiated proliferating hepatocytes, with formation of thick trabeculae and low grade atypia. The cells were negative for B-catenin, Glypecan-3, and CD10. The proliferative index was 35 %. The diagnosis was consistent with well differentiated with hepatocellular carcinoma. 2.3The patient was admitted to the hospital for 5 days and discharged with no post-operative complications.3Isolated gall bladder anomaly without agenesis of the extra-hepatic biliary system is usually diagnoses in adulthood because it is an asymptomatic condition, while when there agenesis of the extra-hepatic biliary system it is evident in the very early neonatal life. The condition is very rare and less than 500 cases are reported in literature .The exact etiology is not known till now, most cases are often sporadic. Some case series are reported to affect many member of the same family suggesting that a genetic predisposition may play a role .The main concern is that when gall bladder agenesis is discovered during surgery, in such condition the operating surgeon should prove that is a true agenesis rather than an ectopic embedded one by thorough examination of the retro-hepatic space, the falciform ligament, the lesser omentum, and the left side for possibility of left sided one ,6,7.The complain of the patients may be due to stones in the common bile duct, attacks of cholangitis, or dysfunction of the sphincter of Oddi. When there is no improvement after surgery, search for other possible causes must be done such as peptic ulcer disease, musculo-skeletal pain, or pain from other pathological sources ,8,9.There is a great debate about whether to convert the surgery from laparoscopic cholecystectomy to the conventional open technique when there is failure of gall bladder visualization. Most authors agree that no conversion is required provided adequate visualization and search for the gall bladder was done. Conversion to open surgery unduly increase the operation time and may result in increased morbidity with unnecessary prolongation of the hospital stay, in such situation postoperative MCRP is mandatory to define the biliary anatomy and to help the surgeons to have a more accurate decision. In cases of isolated gall bladder agenesis with no cystic duct agenesis, intra-operative cholangiography through the cystic duct may be done which will appropriately define the anatomy ,11.The author has no conflicts of interest to declare.None.Ethical approval has been exempted by my institution for reporting this case.An informed written consent was taken from the family for reporting the case and the accompanying images.Dr Ayad Ahmad Mohammed and Dr Sardar Hassan Arif contributed to the concept of reporting the case and the patient data recording.Drafting the work, design, and revision done by Dr Ayad Ahmad Mohammed.Final approval of the work to be published was done by Dr Ayad Ahmad Mohammed and Dr Sardar Hassan Arif.Not applicable.Dr Ayad Ahmad Mohammed is guarantor for the work.Not commissioned, externally peer-reviewed.I was really concerned about the liver tumor, as this was an accidental finding and I was symptomless until this age, I don’t think it will cause great problems.


Non-traumatic mesenteric hematomas are usually well controlled, with no resulting symptoms. Herein, we report a case in which collapse of a large mesenteric hematoma, after rupture of a right colic artery aneurysm, caused small bowel obstruction and rapid absorption of the hematoma contributed to cholestasis.A-44-year-old man presented with a sudden onset of severe right lower abdominal pain. Computed tomography (CT) revealed rupture of a right colic artery aneurysm and intra-abdominal bleeding. After embolization of the right colic artery aneurysm, a large mesenteric hematoma remained. As the patient had no symptoms, we elected to pursue conservative treatment. However, on day 16 post-onset, he developed right lower abdominal pain. On CT imaging, partial collapse of the wall of the residual mesenteric hematoma was observed, with visible leakage from the hematoma into the abdominal cavity, resulting in small bowel obstruction and cholestasis. Symptoms did not improve with conservative treatment, and we proceeded to surgical treatment on day 32 after onset. Intra-operatively, adhesions between the small bowel and the abdominal wall were identified and caused the small bowel obstruction. We proceeded with removing these adhesions and as much of the hematoma as possible. Although the small bowel obstruction improved after surgery, cholecystitis developed, and percutaneous transhepatic gallbladder aspiration was performed on day 45. The patient was discharged on day 70.Collapse of a mesenteric hematoma can cause small bowel obstruction. Rapid absorption of the hematoma due to the collapse might contribute to cholestasis. A large abdominal hematoma might be a risk factor for failure of conservative treatment, and surgery might be required due to abdominal complications. Non-traumatic mesenteric hematoma can result from rupture of an abdominal aneurysm or as a complication of anticoagulation therapy, among other causes , 2. In sA 44-year-old man was admitted to our hospital due to a sudden onset of lower right abdominal pain. The patient had a history of hypertension and hyperlipidemia. Computed tomography (CT) revealed a spindle-shaped and dilated right colic artery, intra-abdominal bleeding, and a large mesenteric hematoma Fig. . A diagnWe performed a laparotomy using a midline incision. Adhesions between the small bowel and the abdominal wall were observed, which were likely the cause of the small bowel obstruction. The release of these adhesions removed the cause of the small bowel obstruction. We also observed a collapse of the caudal wall of the hematoma in the ascending colic mesentery, with a pooling of the leaked blood in the pelvis Fig. . We remoThe small bowel obstruction improved after surgery and the patient was started on a diet. However, on day 45, the patient developed epigastric pain and fever. CT and ultrasound imaging revealed dilation of the gallbladder, with surrounding inflammation. From blood tests, the white blood cell count and levels of biliary enzymes and total bilirubin were elevated. The diagnosis of cholecystitis was made, and we proceeded with percutaneous transhepatic gallbladder aspiration (PTGBA). After PTGBA, the patient’s symptoms improved, with a decrease in white blood cell count and biliary enzymes and total bilirubin; the postoperative course of these levels is shown in Fig. Our case demonstrates the possibility of small bowel obstruction caused by the collapse of a mesenteric hematoma. This collapse caused leakage from the hematoma into the intra-abdominal cavity that allowed adhesions to form between the small intestine and the abdominal wall. The full diagnosis required diagnostic imaging, the patient’s clinical course, and intra-operative findings.We identified five previous cases in the literature that reported complications due to a non-traumatic mesenteric hematoma without re-hemorrhage. The characteristics of these cases are summarized in Table The intra-abdominal adhesions observed intra-operatively caused the small bowel obstruction after leakage of the hematoma into the intra-abdominal cavity. Previous studies have reported on small bowel obstruction , 7 or duAfter the collapse of the mesenteric hematoma, our patient developed pooling of bile sludge in the gall bladder, and elevation of serum bilirubin and biliary enzymes before surgical treatment, followed by cholecystitis after surgery. We considered that bile sludge and the elevation of serum bilirubin and biliary enzymes indicated cholestasis. Figure When the patient was discharged for the first time, we opted for conservative management as he was asymptomatic. This is consistent with the recommendations by Corzo et al.  for non-Collapse of a large mesenteric hematoma after rupture of a right colic artery aneurysm resulted in small bowel obstruction. Furthermore, the hematoma might contribute to cholestasis through the rapid absorption of the hematoma. Therefore, we considered that a large abdominal hematoma might be a risk factor for failure of conservative treatment, and surgical treatment might be required due to complications.


Teaching Point: This case highlights the extrapulmonary lymphatic abnormalities that may be associated with pulmonary lymphangioleiomyomatosis. A 37-year-old woman consulted in our hospital for an increase in the size of her left thigh and abdominal discomfort. Ultrasound excluded venous thrombosis the lower limb.Figure 1), suggestive of lymphatic malformations.Contrast-enhanced body computed tomography (CT) was performed to exclude obstruction on the venous return. It showed two large retroperitoneal well-defined multi-locular cystic masses with homogeneous fluid content and enhancement of the cyst walls and septa . The left extraperitoneal pelvic mass  pressed against the bladder and extended in the inguinal canal, likely causing the left thigh enlargement.The proximal mass surrounded the aorta and the inferior vena cava, spreading along the common iliac axes  and thin-walled cysts of uniform distribution and variable sizes in both lungs  compatible with pulmonary lymphangioleiomyomatosis (LAM).There was a similar low-attenuating mass in the posterior mediastinum  and pelvic (C) masses.We further investigated the patient history. An MRI was actually performed previously in another hospital for abdominal pain which we had no knowledge of at the time of the CT. It showed the same retroperitoneal (After our diagnosis, our patient was treated with Sirolimus, stabilizing her lymphangioleiomyomas. However, they increased after a following pregnancy and sclerotherapy was recently proposed for the symptomatic pelvic lymphangioleiomyoma.LAM is a rare multisystem disorder that usually affects adult women, associated with cystic destruction of the lungs, abdominal neoplasm, and lymphatic thoraco-abdominal involvement. It can be associated with tuberous sclerosis complex.Lymphatic involvement in LAM includes lymphangioleiomyomas and adenopathy, chylous ascites, and chylothorax. The lymphangioleiomyomas are oestrogen-dependant benign cystic masses, sometimes causing incomfort. Since LAM is a rare disease usually first presenting with dyspnea, there are no established guidelines concerning first lymphatic abdominal presentation. However, LAM diagnosis and accurate management is crucial since its prognosis is variable and can lead to pulmonary transplant . Abdomin


Spontaneous aortic thrombosis is rare, and prompt diagnosis is needed whenever encountering a case of unexplained abdominal pain. The cause of the thrombosis needs to be evaluated thoroughly to rule out any underlying thrombophilias. Spontaneous aortic thrombosis is rare, and prompt diagnosis is needed whenever encountering a case of unexplained abdominal pain. The cause of the thrombosis needs to be evaluated thoroughly to rule out any underlying thrombophilias. Symptoms vary widely, ranging from being asymptomatic to a more catastrophic presentation with organ ischemia. We report the case of a 40‐year‐old lady who presented with 1‐week history of abdominal pain and was found to have spontaneous abdominal aorta thrombosis. A 40‐year‐old lady was presented to the hospital with 1‐week history of generalized abdominal pain, moderate to severe in intensity, associated with nausea and four episodes of vomiting. An initial CT scan of abdomen with contrast showed features of abdominal aorta thrombosis with ischemic colitis. Screening for an autoimmune etiology or hereditary thrombophilia was negative. The patient was treated with anticoagulation and she showed evidence of improvement in clinical symptoms and partial resolution of thrombus on follow‐up radiology. Spontaneous aortic thrombosis is rare in occurrence. Evaluation includes abdominal imaging with either CT scan or MRI, and the cause of the thrombosis needs to be evaluated thoroughly. Treatment includes anticoagulation and if needed re‐vascularization by endovascular methods.Spontaneous abdominal aorta thrombosis is extremely rare with an unclear incidence due to its infrequent occurrence. To this date, there are very limited data on spontaneous abdominal aorta thrombosis in literature.2A 40‐year‐old lady was presented to the hospital with 1‐week history of generalized abdominal pain, moderate to severe in intensity, associated with nausea and four episodes of vomiting, with no aggravating or relieving factors. There was no history of fever, hematemesis, bleeding per rectum, chest pain, or palpitations. The patient was a known case of hypothyroidism, vitiligo, and type 1 diabetes mellitus. She was a non‐smoker. The patient had no personal or family history of thrombophilia and there was no history of recurrent abortions. Physical examination revealed an afebrile patient, with a heart rate of 92 beats per minute, blood pressure 124/76 and respiratory rate 17 breaths per minute. Abdominal examination showed diffuse tenderness, no organomegaly, normal percussion note with normal bowel sounds. Cardiac, respiratory, and nervous system examination were normal.Labs investigations showed mild neutrophilic leukocytosis, high C‐reactive proteins, microcytic anemia, normal urea, creatinine, electrolytes, and liver function tests Table . CoagulaA computed tomography (CT) scan of the abdomen with contrast showed diffuse thickening of the splenic flexure, descending colon, and sigmoid colon with surrounding mesenteric edema and fat stranding as well as a thrombus in the abdominal aorta at the level of L3 vertebra Figures  and 2. HThe patient was managed as a case of ischemic colitis due to spontaneous abdominal aorta thrombosis. She was started on anticoagulation with warfarin bridged by therapeutic dose enoxaparin with a target INR of 2–3. A follow‐up CT scan of the abdomen that was done 2 months afterward showed resolution of thrombus and improvement of the inflammatory changes in the bowel (Figure 3Spontaneous arterial thrombosis is rare in occurrence, but it has a significant clinical importance as it leads to several complications secondary to blood flow impediment.Spontaneous abdominal aorta thrombosis is rare as the blood flowing in the aorta has a high velocity which, along with a high caliber of the artery, makes it less prone to thrombosis and it is almost always secondary to an underlying hypercoagulable status.4Spontaneous aortic thrombosis is rare in occurrence, and prompt diagnosis is needed whenever encountering a case of unexplained abdominal pain or embolic phenomena. Evaluation includes abdominal imaging with either CT scan or MRI, and the cause of the thrombosis needs to be evaluated thoroughly to rule out any secondary hypercoagulable states as well as evaluating for primary hypercoagulability. Treatment includes anticoagulation and if needed re‐vascularization by endovascular methods.None declared.Ahmad S Matarneh: History and physical examination, literature review, and manuscript writing. Amna Sadiq: Radiology and clinical images. Adeel Ahmad Khan: Literature review and manuscript writing. Asma Eltahir and Mazen Alansari: Clinical care. AbdulMoqeeth Mohammed: Clinical care and manuscript writing.The case report was approved by Hamad medical corporation, MRC number MRC‐04‐21‐387.Published with written consent of the patient.


The salivary gland tumors are rare entities and the majority of these are benign. However, there are some entities such as prior neck radiation, certain infections, and systemic diseases which should raise the clinical suspicion for a malignant lesion. Patients with Sjogren syndrome are at increased risk for a salivary gland neoplasm, specifically non-Hodgkin lymphoma. While clinical findings play an important role in the initial workup, imaging plays a critical role in the diagnosis and management. This case describes a patient with Sjogren syndrome who presented with a left face mass where imaging was able to confidently diagnose her with a suspicious parotid neoplasm with lymphoma as the favored diagnosis. After histological evaluation, she was diagnosed with primary parotid mucosa-associated lymphoid tissue (MALT) non-Hodgkin lymphoma after which she went on to non-operative management. The salivary gland tumors are rare, accounting for only 6-8% of all head and neck tumors annually in the United States . The risA 57-year-old female was presented to the ED with swelling on the left face for eight months as well as unintentional 10 lb weight loss over the same time. Additional symptoms included frequent bouts of acute nervousness and tinnitus. She denied fevers and night sweats and had no pain or erythema associated with the swelling. Initial laboratory results demonstrated normal white blood cell and platelet count. The patient's kidney function and red blood cell count were normal. CT of the neck with intravenous contrast performed in the emergency department showed asymmetric enlargement of the left parotid gland  should all be elicited from the patient. Physical examination, detailing size, mobility, fixation of the mass to adjacent anatomic structures, local or distant lymphadenopathy, and findings suggestive of facial nerve paralysis can also provide important clues to help the clinician develop a potential diagnosis ,10. AlthImaging studies to include ultrasound (US), CT, or MRI can be performed to determine the size and location of the neoplasm. Often, the extent of tumor involvement is much more accurately appreciated on either CT or MRI than is suggested by physical examination ,12. The The management of most parotid gland neoplasms involves surgical resection. Benign or low-grade malignancies can often be treated with surgery alone, while higher-grade malignancies require surgery with adjuvant chemotherapy and/or radiation therapy. The surgical approach is largely dictated by pre-surgical imaging, with efforts taken to remove all affected tissue, while attempting to spare the facial nerve . While sThe primary MALT non-Hodgkin lymphoma of the parotid gland is a rare neoplasm though it is most commonly seen in the setting of Sjogren syndrome. When patients present with symptoms concerning a salivary gland neoplasm, imaging plays a critical role in the diagnosis. While US can be a good initial screening tool, CT and MRI are the primary modalities for characterization and to help drive further management. Certain findings can be reliable indicators for benign tumors which need no further workup or malignant entities which would need a biopsy to determine further treatment. This case shows a rare tumor with classic imaging features which led to appropriate diagnosis and subsequent management.