Sacrococcygeal teratomas (SCT) are most commonly seen in infants and children but are rare in adults. Most adult SCT are benign and mature with a minority of tumors having immature components or overt malignancy. Here, we report a 65-year-old female with a SCT developing adenocarcinoma. The patient was diagnosed with benign sacrococcygeal cystic teratoma on her initial hospital visit and was treated with surgical resection. She was followed up postoperatively and was noted to have a markedly elevated CA 19-9 level 13 months after the surgery. Radiological and clinical examination revealed thickening of the perirectal soft tissues, located near the inferior portion of her previous incision site. Histological evaluation of the lesion showed invasive, moderately differentiated adenocarcinoma. Immunohistochemical staining results were suggestive, but not diagnostic, of anal gland adenocarcinoma. This case report expands the knowledge regarding an adenocarcinoma arising from a previously benign, adult SCT. Teratomas are germ cell tumors, composed of cells derived from one or more germ cell layers. Teratomas of the sacrococcygeal region are most commonly seen in neonates with an approximate prevalence of 1/27,000 live births –9. Most The patient is a 65-year-old female who initially presented to our hospital in 2019, complaining of pelvic pressure and anorectal pain for 2 years. She also had difficulty with urination and altered bowel movements. Abdominal and pelvic computed tomography (CT) scan showed a 17 cm cystic retrorectal mass. Pelvic magnetic resonance imaging (MRI) revealed a 17.3 × 11.4 × 7.7 cm retrorectal cystic mass with solid mural nodule . Her preTeratomas are composed of cells derived from one or more germ cell layers , and they are classified as a subgroup of germ cell tumors. Different theories exist regarding the origin of the SCT, including that they are derived from the totipotential cells in Hensen's node of the primitive knot . The majType I: the tumor is predominantly external with a very minimal intrapelvic component.Type II: the tumor is predominantly external but has a significant intrapelvic extension.Type III: the tumor is visible externally, but is predominantly located in the pelvic area with extension into the abdomen.Type IV: the tumor has no external presentation and is located in the pelvic bone.SCT are classified according to the Altman classification system /AmericanIn neonates and children, type I tumors have the lowest risk of malignancy and type IV tumors have the highest risk of malignancy .Most neonatal SCT are externally visible, while most adult SCT present as a pelvic mass and often cause compressive symptoms such as lower back pain, bowel/urinary dysfunctions, and venous engorgement of the lower limbs . SometimThe differential diagnosis of SCT in adults includes tailgut cyst, chordoma, meningocele, pilonidal cysts, rectal duplication or anal gland cysts, osteomyelitis of sacrum, giant cell tumor of sacrum, perirectal abscess, fistula, granuloma, and tuberculosis , 23. OurEarly and complete surgical excision of SCT with coccygectomy is the mainstay of treatment. The coccyx may contain the nidus of totipotential cells; the risk of tumor recurrence is reported to be 30-40% without concomitant excision of the coccyx . Unlike Adult SCT are rare and often recognized following symptoms caused by compressive symptoms, including lower back pain, bowel/urinary dysfunctions, and venous engorgement of the lower limbs. Most cases are mature and benign; however, rarely malignant transformation can occur. Atypical structures with a high Ki67 proliferative index, if present, may help to alert that tumor recurrence or transformation into adenocarcinoma is possible. The diagnosis relies on radiological imaging (CT or MRI), serum markers, and histopathologic analysis. Complete surgical excision with coccygectomy is the mainstay treatment for benign cases (both mature and immature SCT) and often leads to a favorable prognosis. Malignant SCT require surgical excision with adjuvant therapy including chemotherapy and/or radiotherapy. Postoperative follow-up of the patients is crucial to detect recurrence.


Fasciola hepatica and Fasciola gigantica. Human infestations are predominantly seen in developing countries where the disease is endemic, but, due to the increase in international travel rates, hepatic fascioliasis is also appearing in nonendemic areas including Europe and the USA. The clinical and laboratory findings are usually nonspecific. Cross-sectional imaging can be very helpful in the diagnosis of fascioliasis as well as to differentiate it from other liver diseases with a very similar clinical picture. The objectives of this case report are to discuss imaging findings of hepatic fascioliasis and to review the literature.Fascioliasis is a food-borne hepatobiliary zoonosis caused by We report the case of a 35-year-old Iranian patient who presented with right upper quadrant pain, low-grade fever, fatigue, and anorexia. The patient had a history of recent travel to the Gilan Province of Iran, almost a month before the onset of symptoms, which is an endemic area of fascioliasis in the country. Laboratory examinations revealed eosinophilia, elevated hepatic enzymes, and slightly raised C-reactive protein. Contrast-enhanced computed tomography of the patient shows clusters of focal ill-defined hypodense lesions with mild peripheral enhancement in the right liver lobe and subcapsular regions. Magnetic resonance imaging of the liver revealed multiple ill-defined lesions of low signal intensity on the T1-weighted image and high signal intensity on the T2-weighted image, extending from the liver capsule into deeper parenchyma toward periportal regions, which shows mild peripheral enhancement on post-contrast images. Imaging-based diagnosis of fascioliasis was made depending on the characteristic distribution of subcapsular tracts/lesions on the above-mentioned imaging, which was then confirmed by serologic tests using enzyme-linked immunosorbent assay. The patient was treated with triclabendazole, showing great clinical improvement, and was eventually discharged in good health condition.The imaging findings in this case report highlight the importance of cross-sectional imaging for further evaluation of suspected cases of fluke-induced liver disease. The hypothesis of hepatic fascioliasis should be always considered when consistent radiological findings are observed. Clusters of tortuous subcapsular lesions with peripheral contrast enhancement extending into deeper liver parenchyma are characteristic imaging findings that strongly suggest hepatic fascioliasis. Fasciola hepatica and Fasciola gigantica. Humans ingest infected watercress or contaminated water with encysted larvae (metacercariae), which excyst in the stomach/duodenum, migrate through the intestinal wall to the peritoneal cavity, and then penetrate the Glisson capsule to enter the liver and eventually the bile ducts [Fascioliasis is a food-borne hepatobiliary zoonosis caused by le ducts , 4. The le ducts –4, 8. ThA 35-year-old Iranian patient presented to our hospital with a 4 weeks history of right upper quadrant (RUQ) pain, low-grade fever, fatigue, and anorexia. At admission, her body temperature was 37.8 °C, and her vital signs were within normal limits . On physical examination, mild RUQ tenderness and normal liver span were noticed. No abnormal findings were present on neurological examinations. The patient was a nonsmoker and did not consume alcohol. She was a literate and married woman but had no official duties and was a housewife. She had no history of previous surgery, chronic medical illness, or family/psychosocial history of any health-related conditions including congenital malformation. The patient was receiving no medication before admission to the hospital. The patient had a history of recent travel to the Gilan Province of Iran, almost a month before the onset of symptoms, which is an endemic area of fascioliasis in the country.3 with eosinophilia , red blood cell count of 4.2 million/mm3, hemoglobin of 12.1 g/dL, and platelet count of 279,000/mm3. Laboratory examinations also revealed elevated hepatic enzymes , and slightly raised C-reactive protein 12 mg/L. Renal function tests and serum bilirubin were within normal limits. Abdomen/pelvis CT scan demonstrates clusters of focal ill-defined hypodense lesions in the right liver lobe and subcapsular regions. These clusters of hepatic lesions (abscess) show peripheral contrast enhancement on contrast-enhanced CT (CECT), forming a tunnel-like tract from the entry site at the Glisson capsule deep to the liver parenchyma (tunnels and caves sign)  was performed that revealed a total leukocyte count of 9300/mmIn the present study, we report a confirmed case of hepatic fascioliasis in a young woman, presented with nonspecific clinical findings and a recent history of travel to an endemic area of fascioliasis. This case report highlights the importance of cross-sectional imaging in the diagnosis of hepatic fascioliasis as well as to differentiate it from other liver diseases with a very similar clinical and radiological picture. While travel history is most important, characteristic findings of tortuous subcapsular lesions with peripheral contrast enhancement, extending into deeper liver parenchyma on contrast-enhanced CT and MRI strongly suggest hepatic fascioliasis.In the presence of an appropriate clinical setting, direct parasitological tests, indirect immunological tests, and cross-sectional imaging techniques such as ultrasound, CT, and MRI are used for the diagnosis of hepatobiliary fascioliasis in humans . The diaIn a patient with fascioliasis, common symptoms are right upper quadrant pain, fever, weight loss, fatigue, pruritus, skin rashes, dyspepsia, nausea, and vomiting. Fascioliasis may mimic several hepatobiliary disorders, especially metastatic liver disease. Misdiagnosis or late diagnosis is still frequent and may lead to unnecessary surgical procedures such as cholecystectomy and hepatic segmentectomy –6. FurthThe best diagnostic clue of hepatic fascioliasis on imaging is clusters of patchy ill-defined hypoenhancing lesions mostly in subcapsular regions that may progress toward deeper parenchyma and periportal areas. Intrahepatic and extrahepatic biliary duct changes are best seen on sonography . RadioloEndoscopic retrograde cholangiopancreatography (ERCP) can be performed for both diagnosis and treatment of bile duct obstruction, which allows the detection and extraction of the motile fluke. ERCP findings include linear, elliptical, or rounded filling defects within a dilated biliary ducts or pancreatic duct –11.A summary of the literature regarding imaging findings of human hepatic fascioliasis is presented in Table Differential diagnosis includes cholangitis and hepatitis, liver abscess, brucellosis, cholecystitis, biliary tract stones, and primary/secondary liver malignancies. Misdiagnosis of fascioliasis is frequent and may lead to unnecessary surgery. The hypothesis of hepatic fascioliasis should be always considered in an appropriate clinical setting when consistent radiological findings are found on cross-sectional imaging , 4. A liClonorchis and Opisthorchis infections occur, and give them enough heat before consumption; (3) washing vegetables, especially in endemic regions, with acid detergents and a solution of potassium permanganate [The following ways can prevent getting infected with hepatic fascioliasis: (1) familiarity of the general public in the endemic areas or those who travel to these areas with this disease and its transmission methods; (2) avoiding consumption of raw wild aquatic plants, local vegetables, raw liver, freshwater fish, or shrimp from areas where anganate , 15, 16.Fasciola hepatica is particularly challenging in nonendemic areas, as clinical and laboratory findings are usually nonspecific. Cross-sectional imaging is usually performed to look for possible causes of these nonspecific findings. Clusters of tortuous subcapsular tracts/lesions with peripheral contrast enhancement extending into deeper parenchyma and periportal regions are characteristic findings on CT/MRI that strongly suggest hepatic fascioliasis. The hypothesis of hepatic fascioliasis should be always considered in an appropriate clinical setting when consistent radiological findings are observed.In general, the diagnosis of


Background: A prostatic utricle (PU) is an unusual pathology with most patients being asymptomatic. However, approximately 29% of patients may show lower urinary tract symptoms, recurrent urinary tract infections (UTI), postvoid dribbling, urethral discharge, epididymo-orchitis, stones, and secondary incontinence caused by urine trapping in the pouch and urinary retention. The standard treatment is through surgical resection, but it is only offered to patients with symptoms. Case summary: We report a case involving a six-year-old boy with congenital hypothyroidism and penoscrotal hypospadias who had previously undergone onlay urethroplasty for the proximal shaft, chordee release, orchidopexy for bilateral undescended testis, and laparoscopic herniorrhaphy for left inguinal hernia. However, the patient later evolved the repetition of UTI and right epididymo-orchitis. Cyclic voiding cystourethrography confirmed the presence of a cystic lesion communicating with the prostatic urethra from the utricle. The PU was then excised laparoscopically. The utricle was identified posterior to the bladder, and insertions of the vas deferens crossover into the utricle were detected by laparoscopy. The post-procedure course was uneventful. Conclusions: Laparoscopic resection of PUs offers a better exposure field, improved wound appearance, complete resection, and reduces the incidence of complications. During laparoscopy, the PU was clearly distinguished from the bladder or other pelvic organs. An incidental finding of vas deferens crossover has rarely been reported. A combined cystoscopy and laparoscopy for PU resection is executable, safe, and valid in this patient population. A prostatic utricle (PU), also called the Müllerian duct cyst, is a cyst that arises from remnants of the Müllerian duct. PU is an unusual pathology, and almost all patients are asymptomatic. However, approximately 29% of patients may show lower urinary tract symptoms, recurrent urinary tract infections (UTIs), postvoid dribbling, urethral discharge, epididymo-orchitis, stones, and secondary incontinence caused by urine trapping in the pouch and urinary retention. The main standard for management is through surgical resection, but it is only provided to patients with symptoms .A six-year-old boy with congenital hypothyroidism and penoscrotal hypospadias had previously undergone onlay urethroplasty for proximal shaft, chordee release, orchidopexy for bilateral undescended testis, and laparoscopic herniorrhaphy for left inguinal hernia.However, the patient later evolved repetition of UTI and right epididymo-orchitis. Finally, cyclic voiding cystourethrography confirmed the presence of a cystic lesion communicating with the prostatic urethra from the utricle . The PU First, urethrocystoscopy was performed, and a guidewire was inserted into the utricle for PU identification. A left-sided ejaculatory duct dilatation was noted .Afterward, laparoscopy was performed using a 5-mm cannula via the umbilicus and two 3-mm cannula trocars on the right and left lower quadrant of the abdomen. The prostatic utricle was carefully dissected free of surrounding tissue to the pelvic floor. The utricle was identified posterior to the bladder, and insertions of the vas deferens crossover into the utricle were observed. When the prostatic utricle was completely mobilized, the utricle and vas deferens were completely resected, and then the urethra was repaired with 6-0 PDS .Laparoscopic resection of the PU was successful in this patient. After the surgery, the patient recovered smoothly, with no complications and voiding difficulties, when the urethral catheter was removed on day two and then discharged. The patient visited our outpatient clinic in the first month after the operation and in the third month, then patients typically attend routine visits every 6 months at our outpatient clinic. No further UTIs or epididymo-orchitis occurred after ten months of follow-up consultation.A PU, also called the Müllerian duct cyst, is produced by the effect of either decreased hormonal secretion or shortage of hormone sensitivity in tissues at the stage when the urogenital plate, which the fused tips of the Müllerian duct originate from, comes into contact with the urogenital sinus, leading to PU formation . Some chThe PMDS is one of the DSDs categories. PMDS is a rare autosomal recessive disorder characterized by the persistence of Mȕllerian structures in a boy. In most cases, phallic development and testicular function are normal. The disorder is usually confirmed during surgery for inguinal hernia with or without cryptorchidism. Most cases are due to mutations in AMH (Anti-Müllerian hormone) or AMHR2 (Anti-Müllerian Hormone Receptor Type 2) . In the Several different surgical methods for PU resection have been described; however, the standard surgical method has not yet been established. Moreover, the problem of dissecting PUs deep in the pelvis and preventing infertility is another issue because these are anatomically close to the spermatic cord.Open surgical procedures are related to the hazard of insufficient PU resection, injury to important pelvic structures, and poor exposure ,8. TransDue to the disabilities associated with open surgical procedures, McDougall et al.  reportedA residual utricle stump was observed in three cases following the open excision of the PU, while no instances were observed following the laparoscopic approach . AdditioRecently, robot-assisted laparoscopy has increased all the benefits of common laparoscopy by raising the anatomic visual image and surgical precision when dissecting within the deep pelvis . HoweverA patient with a history of penoscrotal hypospadias underwent an onlay procedure, chordee release, and inguinal hernia repair presented with repeated UTIs. Differential diagnoses should include a utricle cyst. Furthermore, laparoscopic resection of the PU can be assumed as a viable option for open surgery because it supplies better visualization, simple dissection deep in the pelvis, and better wound healing.A guidewire was put into the PU under cystoscopy. A Foley catheter was also inserted into the bladder to deflate it and differentiate the junction where the cyst neck enters the prostatic urethra, which helped avoid complications of injuring the urethra. In addition, the surgeon could clearly distinguish between the PU and the bladder or other pelvic organs under laparoscopy. An incidental finding of crossover vas deferens is rarely reported. Thus, the combination of cystoscopy and laparoscopy for PU resection is executable, safe, and valid in this patient population.


Bilateral thalamic infarction is a rare entity (it occurs in 0.6% of ischemic strokes) and can be confused with other vascular etiologies such as basilar syndrome and deep cerebral venous thrombosis, as well as neoplasms, infections, or toxins. It is typically characterized by the triad of altered mental status, vertical gaze paralysis, and memory impairment, although their symptoms can be highly variable. We describe the case of a young man who came to the emergency department presenting diplopia, speech alteration, and decreased level of consciousness with frequent fluctuations. Baseline computerized tomography was normal, and because of the clinical findings, thrombolysis was performed. Subsequently made magnetic resonance showed a bilateral acute thalamic infarction caused by an obstruction of the Percheron artery. Although Percheron syndrome has been previously described in medical journals, our case is an unusual case in which we could perform an acute intravenous thrombolytic treatment. Besides, it is important for emergency physicians to enclose the Percheron syndrome in the differential diagnosis of coma in young people so that emergent treatments such as thrombolysis can be employed. Thalamic vascularization is complex and varies from person to person. The paramedian or Percheron artery is a rare variant where a single thalamoperforant artery supplies both thalamic nuclei, with variable midbrain irrigation. Bilateral thalamic infarcts are rare, occurring in 0.6% of ischemic strokes and between 4 and 18% of all thalamic infarcts. [This type of stroke is related to embolic phenomena, and cardioembolism should always be considered as part of its etiology, as well as the discovery of some congenital defects not known previously objectified. .It is characterized by decreased consciousness, neuropsychological and memory impairment, and vertical gaze palsy. Oculomotor disturbances, hemiplegia, cerebellar ataxia, and movement disorders have also been described. It can mimic other pathological processes including intracranial haemorrhage, infection, and inflammation. .Emergency physicians should be aware of this syndrome so that emergent treatments such as thrombolysis can be employed. However, diagnosis of Percheron artery infarction is often made in the late stage.A 43-year-old man with migraine as medical history for which he did not take any active pharmacological treatment begins two hours before his arrival in the emergency room with diplopia, speech alteration, and decreased level of consciousness with frequent fluctuations. He denied the consumption of any associated toxics.The physical examination revealed predominantly femoral right hemiparesis, binocular strabismus with limited convergence and vertical eye movements, nonreactive miotic pupils, and fluctuations in the level of consciousness with a score on the Glasgow Scale that varied between 8 and 10.Among the complementary tests that were requested, the analyses showed no alterations (including toxic and drug profiles) and the baseline computerized axial tomography (TC) showed no haemorrhage or incipient signs of ischemia .During his stay, the fluctuation in the level of consciousness persists and a right hemiparesis de novo is observed. Suspecting a posterior circulation stroke and in the absence of contraindications, intravenous thrombolysis was performed. The study was completed with the performance of computerized angiography that revealed a bilateral thalamic hypodensity predominantly in the posterior left thalamus  which was confirmed with the performance of magnetic resonance imaging Figures  and 3, wDue to the persistence of the alteration in the level of consciousness, the patient was admitted to the intensive care unit (ICU) for neurological control. During his stay in the ICU, the patient fully recovered his level of consciousness, persisting only at discharge a binocular diplopia of less intensity.The study was completed with a study of autoimmunity, vitamin B12 and folic acid, serologies for brucellosis, human immunodeficiency virus, and syphilis that did not show any alterations and the performance of a regulated echocardiogram which revealed a patent foramen ovale, without intracardiac thrombosis.The artery of Percheron (AOP) is a rare variant of the paramedian arterial supply in which a single dominant thalamoperforating artery nourishes both the paramedian thalamus and, in some cases, the rostral midbrain. .Bilateral thalamic infarcts are rare, occurring in 0.6% of ischemic strokes and between 4 and 18% of all thalamic infarcts. They are typically characterized by the triad of altered mental status, vertical gaze paralysis, and memory impairment, although their symptoms can be highly variable, including aphasia, amnesia, and hypersomnolence , motor pOcclusion of the AOP is not the only condition that can be found in thalamic lesions. We want to highlight the importance of framing a syndrome within the differential diagnosis of altered level of consciousness in young people, among which we find other vascular etiologies such as basilar syndrome and deep cerebral venous thrombosis; pathologies such as Wernicke's encephalopathy, neoplasms, infections, Wilson's disease, osmotic myelinolysis, toxic and metabolic processes, and Creutzfeldt–Jakob disease , 6; whicAOP infarction is an often delayed diagnosis due to various factors. First is a syndrome that is unknown to many emergency physicians due to its rarity, and next, the ultimate diagnosis is typically by magnetic resonance imaging in the hospital course. When we suspect an infarction in the territory of the posterior circulation, it is of vital importance performing a cerebral computerized axial tomography (CT) imaging to exclude hemorrhagic processes and enable adequate treatment with intravenous thrombolysis. Early identification of basilar artery occlusion may allow recanalisation and reperfusion via intravenous and/or intra-arterial thrombolysis. As far as we know, our case is the second case reported along with the one reported by Caruso et al. , in whicPatients have frequently missed the time window for acute management of ischemic stroke, most of the times because of the absence of alterations in the baseline imaging and authors reported a low sensitivity of angiographic study, being brain magnetic resonance the most specific imaging test for diagnosis, often based on clinical suspicion , 10.In conclusion, AOP is an uncommon entity of coma in young patients, being of vital importance for emergency physicians to keep it in the differential diagnosis of coma and altered mental status in young people, in order to exclude other vascular etiologies and metabolic diseases and to establish a treatment for a favorable neurologic outcome.


Desmoid tumors of the abdominal wall are rare fibroblastic proliferative tissue, included in the group of soft tissue tumors, not metastasizing but locally aggressive as an infiltrating tumor and a source of recurrence.This case report describes a rare case of desmoid tumor of the anterolateral abdominal wall presented with a large mass of the left flank and iliac fossa. The diagnostic was suspected radiologically following an abdominopelvic computed tomography (CT) and magnetic resonance imaging (MRI). An exploratory laparotomy found a large mass infiltrating the left rectus abdominis muscle, a part of the flat left abdominal muscles, and the left iliac crest. The patient underwent a total resection of the mass involving the left rectus muscle with autoplasty by a pedicled flap of the left LATA fascia with the placement of a bifacial mesh.Histologic analysis of the operatory specimen confirmed the diagnosis of a desmoid tumor of the abdominal wall.The patient has been discharged from the hospital on the fifth day post-operatory with an uneventful recovery; she was in good health after a one-year follow-up.Desmoid tumors of the abdomen are very rare. Although this tumor is histologically benign, it has the potential of invading vital structures and has a high rate of local recurrence.Histology staining confirms the diagnosis, surgery is the gold standard in the management of this pathology.We highlight the importance of radical surgical excision to avoid desmoid tumor complications and to minimize the recurrence risk. •Desmoid tumor are rare benign tumor with potential of invading adjacent structures.•The final diagnosis is made upon pathology study.•Complete en-bloc resection is the gold standard treatement.•The rate of recurrence is very high, and depends on the quality of the resection. Herein,This work has been reported following SCARE 2020 guidelines .2A 41-year-old woman consulted to our for a painful mass in her left flank and left iliac fossa that has evolved for four months without fever, vomiting, nausea, or any symptom of gastrointestinal obstruction.Her surgical past was remarkable by a Caesarean section one year ago.She was a non-smoker with no drugs use, and had no history of allergies. The history of her family is unremarkable, as there is no similar case reported in the family or proven genetic abnormalities. The physical examination finds a soft and tender abdomen with the presence of a Pfannenstiel scar. Moreover, a mass occupying her left flank and left iliac fossa was palpated. The mass was non-compressible, freely mobile, and non-pulsatile.To evaluate the abdominal mass detected by the physical examination. Abdominopelvic CT-scan was performed  showing She underwent a midline laparotomy, intraoperative exploration revealed a parietal mass infiltrating the sub umbilical left rectus muscle, the left iliac crest, and the left anterosuperior iliac spine with adhesions to the greater omentum and the sigmoid colon. We decided to realize a complete excision of the mass for curative resection.We proceeded to a total resection of the mass taking away the left rectus muscle and a part of the remaining muscle of the left abdomen wall with reconstruction by a pedicled flap of the left Fascia lata with the placement of a bifacial mesh with abdominal parietal drainage..The procedure was achieved by a chief of general surgery.Postoperatively, analgesia, antibiotics, and prophylaxis for thromboembolism has been administered. The patient adhered to and tolerated the advice provided such as heavy lifting, and the use of abdominal support belt.The histological examination found a fusocellular proliferation made of long and wide bundles that are inserted between the bundles of the striated muscle (in fingers of gloves). The tumor cells have elongated and sometimes undulating nuclei and eosinophilic cytoplasm with imprecise boundaries in favor of a desmoid tumor, the proliferation is based on an abundant keloid-like collagenous background which becomes focally lax.Figure 4The patient's post-operative recovery was without incident, The drain was removed on the fourth postoperative day and she was discharged on the fifth postoperative day.After a Follow up of one year after surgery, there was no evidence of recurrence.3Desmoid tumors are rare mesenchymal tumors, accounting for approximately 3% of soft tissue sarcomas and 0.03% of all cancers . The estThey occur sporadically, as in the case of our patient, or in the context of Familial adenomatous polyposis (FAP), which is part of the Gardner syndrome . The risDesmoid tumors can occur between the ages of 5 and 80 years with a peak between 28 and 30 years with a median age of 28.5 years .Clinically, fibromatous tumors are usually painless, often revealed by the palpation of a mass of 3–10 cm, poorly limited, with little or no sensitivity, of slow and progressive growth without ever crossing the midline [Desmoid tumors of the abdominal wall are particular in their location and in the circumstances in which they arise, they develop essentially in the rectus and oblique muscles. More rarely they may develop from the pelvic wall .Large size free abdominal endometrioma can exibit the same clinical presentation with potential compression of the adjacent structure, it should be considered as a differential diagnosis of desmoid tumor .Imaging relies mainly on CT and MRI which are useful for assessing the size and extension of desmoid tumors, studying surrounding structures, and evaluating tumor recurrence .Ultrasound, the first-line examination, typically shows a hypoechoic mass, relatively well limited, smooth in its parietal location, and irregular and infiltrating in the other forms. The CT scan shows a more or less well-limited mass of tissue density, which is iso or discreetly hypodense comparing to the surrounding muscle, homogeneously enhancing, sometimes heterogeneous in large tumors with areas of necrosis, microhemorrhages, and metaplasia. MRI allows a better characterization of the tissue, a better study of the relationships with adjacent structures , and the differentiation of postoperative changes or after medical treatment of a tumor recurrence . It is oIn our Case, CT and MRI scans were used to determine the exact location and relationship of the tumor which was the rectus abdominis and the flat muscles of the abdomen.Curative surgery, when possible, should remain the treatment of choice despite the high risk of recurrence, the surgical excision must be large, passing through the healthy zone with a margin of a safety margin of 2–3 cm beyond the palpable tumor. However, complete resection cannot be judged macroscopically because the tumor is not encapsulated and the muscular extension is impractical .The deliberately mutilating nature of the surgical resection and the parietal defects constitute a difficulty for the surgeon. Several procedures are used: directed cicatrization based on repetitive steps; fascial grafts known as auto genetic grafts, most often using the tensor fascia lata muscle; flaps using vascularized and pediculated structures.This technique can use the dorsal major muscle, the fascia lata muscle, the rectus abdominis, the fascia lata, and the aponeurotic or epiploic flap.The insertion of synthetic material must comply with the rules of rigorous asepsis, using resorbable or non-absorbable meshes whose insertion site may be subperitoneal, preperitoneal, or intraperitoneal .In the case of non-resectability, medical treatment is based on hormonal therapy and non-steroidal anti-inflammatory drugs allowing stabilization or, more rarely, regression of the tumor size. Chemotherapy or external radiotherapy may be proposed in aggressive forms, resistant to medical treatment, or deemed untreatable as palliative or neo-adjuvant treatment .In our case, the patient underwent a large excision with a parietal reconstruction using a fascia lata flap over a bifacial mesh.The postoperative course was simple and no adjuvant treatment was used.The histological study shows a monoclonal proliferation of spindle-shaped fibroblastic cells surrounded and separated from each other by collagen fibers arranged in bundles . In our Our patient underwent clinical and CT surveillance at 3 and 6 months with a 1-year follow-up and no locoregional recurrence was observed.4Desmoid tumors of the abdominal wall are rare, benign tumors with a high risk of local recurrence. The clinical manifestation of these tumors is characterized by masses that are often large. Complementary radiological examinations, in particular ultrasound, MRI and CT, allow the diagnosis to be suspected, but confirmation is only provided by anatomopathological examination, which reveals a proliferation of fibroblasts of benign appearance.Treatment is ideally based on wide surgical which consists of carcinological removal of the tumor with sufficient safety margins followed by parietal reconstruction using prosthetic reinforcement if necessary. The combination of radiotherapy and hormonal therapy is a good alternative that provides objective answers in inoperable desmoid tumors and reduces the recurrence rate.No ethical approval necessary.The author(s) received no financial support for the research, authorship and/or publication of this article.Dr Mabrouk Mohamed Yassine: Have written the article, have consulted the patient, prescribed all of the tests and prepared the patient for surgery and participated in the surgery.Dr Bouzayan Laila: have helped writing the article, data collection.Dr Malki Samia: Interpretation of histological data.Pr Benani Amal(anatomopathology professor): confirm the histological diagnosis.Pr Jabi Rachid: supervised the writing of manuscript.Pr Bouziane Mohammed (oncology surgery professor): have supervised the writing of the paper, and has been the leader surgeon of the case.Our paper is a case report; no registration was done for it.Mabrouk Mohamed Yassine.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.The authors declared no potential conflicts of interests with respect to research, authorship and/or publication of the article.


While overwhelming majority of laparoscopic cholecystectomy specimens performed for gallstones or cholecystitis show rather typical findings, sometimes polypoid structures are also removed. These can be related to cholesterolosis or conventional adenomas, but occasionally extraordinary findings do emerge. In our case, a 67-year old lady with typical complaints of cholecystitis underwent routine laparoscopic cholecystectomy. Preoperative ultrasound revealed a polypoid mass with inflammation and without suspicion for malignancy. Microscopic examination showed partly conventional, low-grade dysplastic crypts forming a villous and rather complex structure. Ectopic crypt foci, slit-like serration pattern and serrated dysplasia with eosinophylic cytoplasm and centrally located nuclei were seen throughout the lesion, thus a traditional serrated adenoma (TSA) of the gallbladder was diagnosed. TSA represents the rarest subtype of serrated lesions in the colon and extracolonic manifestations are sporadically reported. Until now only a single case of a serrated adenoma was reported from the gallbladder. Here we describe the detailed clinical, pathological and molecular findings of our case and discuss these in the light of current literature data regarding this field. Gallbladder specimens removed due to cholecystitis represent a large segment of surgical specimens with few remarkable changes of which carcinoma has the highest importance . AccordiAfter hyperplastic polyps (HP) and sessile serrated lesions (SSL), traditional serrated adenoma (TSA) is the least frequent type of serrated lesions with a heterogenous molecular background. TSAs have three main characteristical features: 1) ectopic crypt formation/focus (ECF), 2) slit-like epithelial serration of the lumen, 3) tall columnar epithelial cells with eosinophylic cytoplasm. If the nuclei of these cells are centrally located and penicilliform it is called serrated senescence, but if nuclei become atypical, vesicular, crowded, it is regarded as serrated dysplasia. Of these three features, two should be present—with having greater than 50% proportion—for diagnosing a TSA , 9. The Our patient was a 67-year old female with paroxysmal atrial fibrillation, with good cardial function according to her regular cardiological follow up. Her medical history also included osteoporosis, cysts in her liver and kidneys and smoking. No malignant disease or clinically important hereditary condition was noted. She had been suffering from abdominal complaints suggestive for chronic cholecystitis for 18 months and came to the surgical ward of Bajcsy-Zsilinszky Hospital for an elective surgical intervention. Physical exmination, ultrasound and laboratory results suggested a polypoid lesion related to chronic inflammation and a gallstone in the cholecyst. The lesion was not suspicious for malignancy or an infiltrative process, thus laparoscopic cholecystectomy was performed.Macroscopically, a polypoid lesion with a diameter of 15 mm and with velvety surface emerged from the mucosa of the removed gallbladder. The polyp showed neoplastic appearance, however, did not seem to be infiltrative or malignant . The musBRAF was of wild type, but a KRAS G12D mutation, a frequent (∼50%) finding in TSAs, was identified [Immunohistochemical reactions with the four mismatch repair proteins (MMR)  showed retained nuclear staining, claiming for MMR-proficiency. entified .As bile discharge was noted postoperatively through the surgical drains, the urgently performed ERCP found a stenotic main biliary duct and bile leakage from the bed of the gallbladder. This situation was resolved by the implantation of an endobiliary stent. The patient became asymptomatic and was discharged home soon. The 5-years follow up found the patient well, without any complications related to her TSA.Our case was an unexpected finding in a routine cholecystectomy performed for chronic cholecystitis. Preoperative examinations suggested some polypoid structure growing in the gallbladder on the background of chronic inflammation, but no suspicion for malignancy. Macroscopic picture suggested also a benign polyp. In fact, the most important differential diagnostic question was to rule out a possible malignancy in the gallbladder, since this have a dismal prognosis. Gallbladder polypoid changes usually represent cholesterolosis, but in case of bigger lesions, an adenoma is more probable. Non-epithelial neoplastic lesions can also present as polypoid structures, but our case was clearly of epithelial origin.Firstly, the tubulovillous structures and conventional dysplasia were suggestive of a tubulovillous adenoma (TVA). TVAs are rare in gallbladders, but do occur and follow the adenoma-carcinoma sequence known from colorectal carcinogenesis . FurtherErsöz et al. compared the imunohistochemical characteristics of the TVAs and the TSAs, finding that CK20 and CK7 were mainly expressed in ECFs, which also showed elevated ki-67 index. MUC5AC and cytokeratin seven coexpression was more frequent in TSAs. P16 stained more TSAs, while p53 highlighted the dysplastic compartments in both groups. But the authors also concluded that from the several histologic features shared by both TVAs and TSAs, the predminance of mid-zonal nuclei, eosinophilic cells, slit-like serrations and ECFs proved to be the most discriminatory features for TSAs .KRAS mutation and carries the highest risk for associated invasive carcinoma. Our lesion showed only eosinophilic cytoplasm, not clear cytoplasm. The cells were not cuboidal, but were more columnar, elongated, focally with sratification of the nuclei. The least frequent oncocytic phenotype of IPNs forms arborizing papillae lined by oncocytic cells with atypia. Our lesion did not show arborizing papillae, the nucleoli were not large or eccentric. Conventional and serrated senescence/dysplasia dominated the structure, which is not a usual feature of oncocytic papillary neoplasms. Neither the slit-like serration and lots of ectopic crypt foci present in our case are characteristic for any type of IPN. Cytokeratin 7, MUC1 and HepPar1 immunohistochemical reactions could help us to exclude these entities. As the picture  was overwhelmingly suggestive of a TSA, we did not perform any additional immunohistochemical reactions.Intracholecystic papillary neoplasm (IPN) of the gallbladder also emerged as differential diagnosis. The gastric/pyloric and intestinal types were easily excluded by their appearance. The biliary phenotype usually forms papillae lined by cuboidal cells with clear to eosinophilic cytoplasm and enlarged nuclei with distinct nucleoli. This usually harbours BRAF mutations and trend to be MMR-deficient, the majority of TSAs are MMR-proficient and present with KRAS mutation (closely followed by BRAF mutations) [KRAS mutation and MMR-proficiency), which strengthened our diagnosis and hypothetized similarities to colonic TSAs [For decades, the conventional adenoma-carcinoma sequence was considered as the main pathway of colorectal carcinogenesis, but the serrated lesions with the interval cancers have driven our attention towards the serrated pathway , 24. HPstations) . There anic TSAs .Recently TSAs were also described in the upper GI tract with cases in the esophagus , stomachAdenomas are infrequent findings in gallbladders and serrated lesions are exceptionally rare in this location with only a single serrated adenoma with a concomitant carcinoma reported until now. TSAs are the least frequent serrated lesions of the serrated pathway, which is involved in colorectal carcinogenesis in almost one third of CRCs and usually follow the KRAS/BRAF-mutated pathway. Until now, the relevance of the serrated pathway in extracolonic neoplasias is not well known, thus our case may add some information to this field.Sometimes a least interesting specimen can bring huge surprise and serve as a unique case. We all know that common diagnoses are the most common ones, thought, we should not hesitate to diagnose something out-of-the box. Gallbladder specimens are usually handled with less interest, because of their usually uniform character. Still, we need to keep our eyes wide open towards morphological characteristics—even for other fields—to be able to find something extraordinary. To our knowledge, our case is among the pioneers of TSAs described in gallbladder.


Esophageal gastric heterotopia (GH), the presence of differentiated gastric tissue in the esophagus, is estimated in up to 14% of populations worldwide and has always been reported on the surface of the esophagus, where it is also known as inlet patch. However, submucosal GH, in any tissue, is a rare finding. We report the case of a 50 year-old male presenting with chronic cough, heartburn and raspy vocalizations. Endoscopic examination showed a single 7 mm esophageal nodule, 20 cm from the incisors, interpreted as a submucosal mass. Pathologic examination of the endoscopically excised nodule showed well-differentiated gastric mucosa within the submucosa underneath the overlying squamous mucosa, consistent with submucosal GH. This case raises the awareness of an atypical presentation and location of GH seen as a submucosal mass on endoscopy. Gastric heterotopia (GH) is defined as the presence of differentiated gastric mucosa (GH)-type tissue located outside the stomach. GH commonly occurs in the proximal esophagus and rarely in the distal esophagus, where it must be differentiated from Barrett’s esophagus. In the cervical esophagus, GH exclusively occurs at the level of or just distal to the upper esophageal sphincter . GH has A 50 year-old male with a history of chronic gastroesophageal reflux disease (GERD) and cough was referred to university hospital for upper endoscopic ultrasonography (EUS) to evaluate a submucosal mass. He had intermittent postprandial acid reflux attacks 3 years prior with increased frequency over the next 18 months. He also complained of intermittent, postprandial dry cough and raspy vocalizations. He had an episode of chest pain leading to an emergency visit to an outside hospital, at which time cardiac and pulmonary involvement was ruled out. Physical examination was only notable for hypertension. His symptoms were improved following 2 weeks of proton pump inhibitor treatment (PPI) and a GERD diet.Helicobacter pylori. He was then referred to our hospital to evaluate the submucosal mass. At the time of examination here, he reported that PPIs were no longer improving his symptoms.A follow-up esophagogastroduodenoscopy 1 year ago at the outside hospital revealed a submucosal mass, 20 cm from the incisors, as well as moderate reflux esophagitis and antral chronic gastritis without Upper EUS demonstrated a 0.7 cm submucosal nodule  in the pEndosonographic evaluation of the esophageal nodule showed a hypoechoic oval intramural  lesion with well-defined borders and apparent origin within the deep mucosa . A preliH. pylori organisms identified.Gross examination of the excised esophageal nodule showed a pink soft tissue nodule, 1.0 cm in diameter and 0.3 cm thick. Microscopic histopathologic examination showed orderly gastric epithelium and glands beneath the muscularis mucosae (MM) of the esophageal squamous mucosa (SM), consistent with submucosal GH . This GHThere was verbal follow up with the patient 2 weeks after upper EUS. It is unclear whether the patient derived benefit from the intervention as he reported ongoing symptoms of GERD not alleviated by PPI.Here, we report a case of submucosal esophageal GH in a 50 year-old man presenting with complaints of heartburn, chronic cough and raspy vocalizations. The GH presented endoscopically as a nodule just distal to the upper esophageal sphincter. Histopathologic evaluation showed gastric-type mucosa without inflammation or dysplasia. To our knowledge, this is the first report of submucosal esophageal GH.et al. [Prior case reports of esophageal GH have been mucosal lesions where the GH interrupts the esophageal squamous epithelium or is located just beneath it . There iet al.  describeet al. .The earliest description of GH was a report of esophageal heterotopic GH discovered on postmortem examination by Schmidt in 1805. Islands of GH in the mucosa of the cervical esophagus are referred to as inlet patches. The prevalence of inlet patches ranges from 0.18 to 14%, though autopsy reports show an incidence of up to 70% , 6. AddiGH is widely considered to be congenital in origin, representing remnants of the columnar lining of the fetal esophagus. The embryonic esophagus, initially lined by ciliated columnar epithelium, is later replaced by non-keratinized squamous epithelium, beginning at the mid-esophagus and proceeding proximally and distally. Failure of this squamous epithelialization is thought to be the cause of GH. In contrast, some have suggested GH as an acquired condition, representing healing from chronic injury, similar to the pathogenesis of Barrett’s esophagus. Support for the acquired hypothesis includes the similar expression of cytokeratin and mucin protein in patients with inlet patches and Barrett’s esophagus.GH is largely asymptomatic and discovered incidentally. Some symptoms include heartburn, chronic cough and hoarseness, which may be attributed to the potential of inlet patches produce acid, but mucous production has also been implicated , 10. Com


Ameloblastoma (AMBL) is an odontogenic tumor, considered to be benign, but aggressive, whose principal risk is a recurrence. The growth can be enormous, and it can extend into the intracranial compartment with serious consequences. The intracranial involvement of AMBL is rare, and it may require an extensive surgery. Although it is a rare condition for the neurosurgeon to treat, knowing this condition can lead to a significant increase in survival for these patients. A case of a 56-year-old woman presented with a history of recurrent left maxilla AMBL with intracranial extension and dural involvement of the anterior and medial cranial fossa is reported, followed by a systematic review of the literature with the aim to identify the best surgical treatment. A total of 32 cases were included in the qualitative analysis. Management is varied and often not described, resulting in an almost complete lack of information and indications for treatment. Radical surgery tends to yield the best outcomes, and it is recommended to have adequate surgical margins when possible.Intracranial involvement from AMBL compartment is an uncommon manifestation of this rare pathology, but which deserves to be treated in a multidisciplinary way in order to ensure maximum surgical radicality. Recurrence reflects failure of the primary surgical resection. If recurrence is the major consideration, surgeons are encouraged to select radical surgery. Whenever a follicular-type maxillary AMBL is diagnosed, it is advisable to check for intracranial spreading and distant metastases during follow-up. Ameloblastoma (AMBL) is an odontogenic tumor, considered to be a benign, but aggressive, whose principal risk is a recurrence , 2. The We perform an accurate analysis of all cases reported in literature and present a case of a 56-year-old woman presented with a history of recurrent left maxilla AMBL with intracranial extension and dural involvement of the anterior and medial cranial fossa in order to retrieve information regarding clinical features and surgical treatment.A literature review was performed, and the following inclusion criteria were adopted: 1) studies that address the topic of AMBL that involves intracranial compartment; 2) review or collection of cases, case series and case report that describe diagnosis, treatment, surgical approach and prognosis of patients.Exclusion criteria were: (1) short reports or letter; (2) papers that study AMBL without a specific treatment of intracranial portion and (3) articles not written in English.The study was conducted in accordance with PRISMA statements. The English literature was systematically investigated using MEDLINE, the NIH Library, PubMed, and Google Scholar. The last search date was August 20, 2019.Search terms included: "Ameloblastoma" in combination with "brain" or "intracranial." All cases of AMBL with intracranial involvement were included. Backward citation tracking was applied to identify articles not retrieved by electronic searches. Two independent authors (D.A. and L.V.B.) conducted the first research of electronic media and any discordance was solved by consensus with a third author (A.S.). Due to the rarity in literature of these types of complications, case reports and articles not written in English were also included in the review. Searches were limited to human studies and systematic reviews were excluded Table The main search returned a total of 4783 papers on AMBL as a topic. The details were collected: authors and year of publication, the number of patients, race, age, sex, the site of origin at first diagnosis of AMBL, if local recurrence was reported before the diagnosis of intracranial tumor involvement, the site of intracranial involvement, the time between the first diagnosis of AMBL (expressed in years) and the detection of the pathology at the intracranial level, if neurosurgery has been performed on the tumor, whether patients performed adjuvant RT, finally if there was the radiological finding of distant metastases, recurrence and the final prognosis of the patients Table .Table 2CA total of 32 cases were included in the qualitative analysis. The series consisted of 18 males and 15 females, and the mean age was 50 years. It is no clear a predominance of African rather than European or Asian individuals in the occurrence of this disease course . 23 out of 33 patients (69.7%) had the maxillary bone as the first site of origin of the tumor against 9/33 (27.3%) patients who showed the first site the mandibular bone and one patient who had shown the nasal bones as the origin of the tumor (3%). The histological report was reported in series on 19 patients, where 9 patients were diagnosed with follicular AMBL (47.4%), 4 AMBL mixed and AMBL Plexiform (21.1% respectively), 1 papillary form and 1 spindle-cell form (5.26% respectively). Before the radiological diagnosis of intracranial involvement of the tumor, patients reported the diagnosis and treatment of a local recurrence of primary AMBL in 27/33 cases (81.8%). The most frequent site of involvement was the orbit in 15/31 patients (48.4%), followed by the anterior cranial fossa floor 9/31 (29%) and mean 9/31 (29%). The cases in which there was no direct spreading of the lesion but a multifocal area distant from the site of origin was diagnosed is 7/31 cases (22.6%). The average estimated time for the detection of an intracranial lesion from AMBL is about 10 years with cases in which the finding was contextual (synchronous metastasis 3/33 cases—9%), short term (1 patient with 2 months finding) and a maximum of 30 years. Information on surgical treatment is available on 31/33 cases. It was possible to perform a radical surgical removal in 23/31 patients (74.2%), while for 8/31 cases (34.8%) it was only possible to biopsy the lesion to obtain a certain diagnosis. The reasons reported were the difficulty of planning the intervention (diagnostic impossibility), the patient's general clinical status and the patient's will. In 13/31 (41.2%) patients, disease recurrence was found at the intervention site, with a reported survival rate of 15/31 cases (48.4%) It was not possible to retrieve a statistically significant analysis of the studies reported.A 56-year-old woman presented with a history of recurrent left maxilla AMBL with multiple recurrences of a maxillary follicular AMBL that developed direct intracranial extension with dural involvement of the anterior and medial cranial fossa. The endonasal endoscopic assisted and open combined approach was performed with a successful complete tumor resection. Patient was treated in our institution in February 2019.Previous medical history: the disease's onset was five years ago with a history of facial pain, mild gaze palsy, decreased left facial sensation, and speech and swallowing changes. She was evaluated and treated at first by an odontologist who diagnosed a presence of AMBL invading the maxillary sinus. The initial tumor resection was performed in 2015 and consisted of a left high maxillectomy. The patient was closely monitored. The initial tumor recurrence has been noted on follow-up CT scans in the left posterior ethmoid after 6 months. Given the extensive local invasion of the lesion and new intracranial extension, a combined approach was planned to maximize cytoreduction. An image-guided, endoscopic transnasal approach was utilized with posterior septectomy and right sphenoidotomy. The histological analysis confirmed a recurrence of AMBL with follicular components a complete resection was performed with surgical soft tissue margins superior to 1 cm. A second recurrence located on the ethmoid and frontal sinus was found two years later. The extension was then further assessed by magnetic resonance imaging (MRI) and revealed an osteolytic soft tissue lesion with an extradural component resulting in a mass effect on the left and right cerebral frontal lobe  and Duragen  layer was applied before the galea patch were used to cover the intracranial area. The second part of the procedure consisted of a combined bifrontal and midfacial approach, exenteration of the right orbit, and extirpation of the perisinusoidal- and retropharyngeal tumor masses. The tumor mass was then resected en-bloc. A latissimus dorsi myo-cutaneous free flap was raised and used to fill the residual dead space. A subcutaneous tunnel in left parotid region was realized to allow the pedicle to reach the facial vessels and perform the anastomosis. Every step was accompanied by histological analysis of every resected margin, frozen margins were negative. The postoperative course of the intracranial surgery was uneventful. After 3 days in the intensive care unit (ICU), the patient was discharged to the normal ward. The patient developed no neurologic deficits postoperatively. A Contrast-enhanced brain MRI scan, performed the following day, did not demonstrate any residual enhancement. Nine days after, the patient was discharged. During the hospitalization no rhinoliquorrhea or otholiquorrhea were observed also. Six months after the operation, follow-up imaging remained stable with no dural enhancement. Postoperative follow-up MRI at 12 months showed no enhancing lesions to suggest tumor recurrence (Fig. AMBL is a benign odontogenic tumor but the high local recurrence rate and intracranial involvement make the management controversial. In a study of 315 AMBL patients published by Olaitan et al. , only th


Renal cell carcinoma (RCC) can invade locally through the renal vein and into the inferior vena cava (IVC) with tumor-thrombus formation reported in 5%-15% of patients. From the IVC, RCC can grow intravascularly and extend into the right atrium. We present a rare case with two uncommon findings: tumor-thrombus extension leading to a right atrial mass and initial presentation of RCC as heart failure. A 69-year-old woman presented with signs and symptoms of heart failure. Electrocardiogram was normal and the initial troponin level was mildly elevated to 0.09 ng/mL. Echocardiography revealed a dilated right atrium with a 6.9 cm x 3.8 cm echogenic mass consistent with a tumor impinging on the tricuspid valve leading to a functional stenosis. Computed tomography (CT) of the abdomen revealed a large right-sided renal mass with enlargement of the renal vein suggestive of tumor thrombus. Although the initial presentation of RCC with cardiac symptoms is surprising, this case highlights the importance of maintaining a comprehensive differential diagnosis. It also signifies the need for further imaging as not all atrial masses are cardiac tumors. Many other primary tumors - kidney, liver, lung, and thyroid - can directly invade or metastasize into the atrium by way of the vena cava. Renal cell carcinoma (RCC) accounts for 90% of renal malignancies . At the A 69-year-old Caucasian woman with a past medical history of hypertension and hypothyroidism presented with fatigue, progressive exertional dyspnea, and exertional chest pain for several weeks. The dyspnea on exertion worsened to the point where she was unable to get up from bed and walk to the bathroom without being completely out of breath . She also endorsed orthopnea requiring three pillows to sleep at night, bilateral lower extremity swelling, and exertional chest heaviness with a left-sided pressure like sensation that lasted a few minutes and resolved with rest. On the day of presentation, she experienced non-radiating, sharp, stabbing left-sided chest pain that was different from her previous chest pain. She denied any associated cough, pleuritic/positional variant chest pain, abdominal pain, hematuria, polyuria, dysuria, or urinary incontinence.Vitals showed a blood pressure of 119/71 mmHg, heart rate of 72 beats per minute, and respiratory rate of 20 breaths per minute. She was afebrile (97.9 F) with an oxygen saturation of 95% on room air. A pulmonary exam was significant for faint bibasilar crackles. A cardiovascular exam revealed a normal rate and regular rhythm with a 2/6 systolic ejection murmur heard at the lower left sternal border, jugular venous distension, and 2+ bilateral lower extremity edema. The chest wall was non-tender to palpation. The rest of the exam was unremarkable including an abdomen with no organomegaly.Electrocardiogram revealed normal sinus rhythm without any ischemic abnormalities. The initial troponin level was mildly elevated at 0.09 ng/mL with a delta troponin of + 0.02 ng/mL. B-type natriuretic peptide was elevated at 1,243 pg/mL. Complete blood count (CBC) revealed a hemoglobin of 16.2 gm/dL. Complete metabolic panel (CMP) revealed an elevated creatinine of 1.5 mg/dL and mildly elevated liver tests: alkaline phosphatase of 175 IUnits/L, aspartate transaminase of 66 IUnits/L, alanine transaminase of 92 IUnits/L, and total bilirubin of 2.5 mg/dL. Prothrombin time (PT) was 15.7 seconds, activated partial thromboplastin time (APTT) was 47.8 seconds, and international normalized ratio (INR) was 1.4.Chest x-ray revealed pulmonary vascular congestion and cardiomegaly consistent with heart failure. Transthoracic echocardiography (TTE) revealed a dilated right atrium with a 6.5 cm x 3.7 cm echogenic mass occupying the entire right atrial space and impinging on the tricuspid valve leading to tricuspid stenosis. Differentials for this mass included myxoma versus other tumors. A transesophageal echocardiogram (TEE) was obtained to better characterize the mass, which confirmed the presence of a dilated right atrium with a 6.9 cm x 3.8 cm echogenic solid mass Figures -1D. ThisComputed tomography (CT) of the chest/abdomen/pelvis revealed a multilobulated 12.8 cm x 9.3 cm solid and cystic right-sided renal mass that nearly replaced the right kidney with enlargement of the renal vein suggestive of tumor thrombus Figures , 2B, 3. She initially underwent diuresis; however, she received fluids once the renal mass was found in order to maintain euvolemia. The patient’s heart failure symptoms improved, and she was referred for oncological and surgical evaluation.Tumor-thrombus formation is a rare complication of RCC that occurs in approximately 5%-15% of patients ,4. The tThere are several case studies in the literature reporting functional stenosis secondary to atrial masses ,7. ApproRCC can cause many paraneoplastic syndromes, including hypercalcemia of malignancy and erythrocytosis. Our patient had a normal calcium level; however, her hemoglobin was elevated, most likely secondary to paraneoplastic erythrocytosis or reactive erythrocytosis secondary to her dyspnea.RCC can be diagnosed with abdominal imaging. Most patients are diagnosed with CT, but RCC can also be diagnosed with abdominal ultrasound or magnetic resonance imaging (MRI). Our patient initially underwent TTE, which revealed a mass in the right atrium, and a CT was ordered to look for possible causes of this mass. After the diagnosis of RCC is made, the patient should be evaluated for metastasis . Tissue The mainstay therapy for the treatment of RCC is radical tumor resection . For patThe differential diagnosis for an atrial mass includes a myxoma, thrombus, vegetation, and primary or secondary neoplasm. Many other primary tumors - kidney, liver, lung, and thyroid - can directly invade the atrium or by way of the vena cava. This report emphasizes the importance of further work-up and imaging of atrial masses.Our patient presented with two uncommon findings: tumor-thrombus extension resulting in a 6.9 cm x 3.8 cm right atrial mass leading to severe tricuspid valve functional stenosis and initial presentation of RCC as acute heart failure. Although the initial presentation of RCC with cardiac symptoms is surprising, this case highlights the importance of maintaining a comprehensive differential diagnosis. It also signifies the need for further imaging as not all atrial masses are cardiac tumors.


S. aureus grew in the tissue culture. Cutaneous botryomycosis was diagnosed, and oral clindamycin was initiated.A 20-year-old woman presented with a growing mass on the plantar surface of the left foot that had been present for one year. She reported laceration trauma in this region six years earlier. The wound healed initially with topical antibiotics, but eventually, a palpable mass appeared. The patient was afebrile, had no skin discharge, and her hemogram was normal. Magnetic resonance imaging (MRI) demonstrated multiple clustered oval lesions affecting the soft tissues of the left foot, with heterogeneous gadolinium enhancement . After s,S. aureus is most commonly implicated. Botryomycosis is a misnomer still used for historical reasonsBotryomycosis is a chronic suppurative bacterial infection that generally affects the skin and subcutaneous tissues. It has an insidious course, usually involving the extremities, and is associated with previous inoculating trauma, alcoholism, diabetes mellitus, and/or immunosuppression-The MRI appearance of botryomycosis has rarely been described


Choriocarcinoma syndrome is a rare phenomenon that occurs in male patients with testicular choriocarcinoma. Male patients who have a testicular non-seminomatous germ cell tumor (TNSGCT) with at least partial choriocarcinoma histology, and metastases to the lungs and/or other extragonadal sites, as well as a markedly elevated beta-human chorionic gonadotropin (HCG), have been prone to pulmonary bleeding, hypoxia, and acute respiratory distress syndrome (ARDS). The respiratory complications occur immediately after chemotherapy is administered or, in some cases, spontaneously. Paraneoplastic hyperthyroidism is another entity described in patients with testicular choriocarcinoma, whereby high levels of HCG  induce clinical and laboratory characteristics of hyperthyroidism. We present the case of a male patient diagnosed with TNSGCT and found to have both choriocarcinoma syndrome and paraneoplastic hyperthyroidism in the setting of only mildly elevated HCG levels. We compare our case with similar cases published previously while questioning the quantitative role of HCG. Testicular tumors, especially germ cell tumors (GCT), are the most common malignancies affecting male patients between the ages of 15-35 years old. GCT can be either seminomatous or non-seminomatous. Non-seminomatous GCT include yolk sac tumors, choriocarcinoma (CC), embryonal, teratoma, or a combination thereof. The mixed type composes 30-50% of all GCT . CC is cChoriocarcinoma syndrome (CS) occurs in patients with choriocarcinoma GCT and diffuse metastases, especially to the lungs, presenting with pulmonary hemorrhage and markedly elevated HCG levels. A comprehensive review by Rejlekova et al. describes high-risk patients for CS with testicular non-seminomatous germ cell tumors (TNSGCT) as having lung metastasis, CC pathology and high levels of HCG  ,7.In addition to CS, hyperthyroidism is a paraneoplastic syndrome that is well-described in CC patients and linked to elevated HCG levels. It is suspected that in most cases with high levels of HCG > 50,000 mIU/ml, patients are prone to developing a hyperthyroid state. The alpha subunit of the HCG hormone is similar to that of thyroid stimulating hormone (TSH) and thus could actually stimulate the TSH receptor and lead to hyperthyroidism or even thyrotoxicosis. Furthermore, the association of elevated HCG and hyperthyroidism extends to female patients with invasive moles, gestational choriocarcinoma, and placental site trophoblastic tumors.We present a unique case of CS with acute respiratory distress syndrome (ARDS) and paraneoplastic hyperthyroidism in a young male with TNSGCT and a mildly elevated HCG level. We then compare our findings to similar cases published in the literature.A previously healthy 29-year-old Caucasian male presented with two weeks of worsening dyspnea and one month of scrotal swelling. He had no prior smoking or drinking history and did not use recreational drugs. His family history was non-contributory. Upon presentation to the emergency room, his physical exam revealed tachycardia, decreased breath sounds at the right lung base, marked scrotal and lower extremity edema, and a right testicular mass. Labs on admission included an HCG level of 1,063 mIU/ml , alpha fetoprotein level (AFP) of 26,157 ng/ml , lactate dehydrogenase level (LDH) of 1,671 U/L , TSH level < 0.01 uIU/ml , and a free thyroxine level (T4) of 3.1 ng/dl . Computed tomography (CT) with contrast of the chest, abdomen and pelvis revealed multiple pulmonary masses, right pleural effusion, extensive bulky mediastinal and abdominal adenopathy, hepatic lesions, and an 11.7 cm right testicular mass. The patient underwent right radical orchiectomy and tumor resection, with pathology revealing high-grade mixed germ cell tumor composed of choriocarcinoma (50%), yolk sac tumor (45%) and embryonal carcinoma (5%). At that point he was diagnosed with a high-grade metastatic mixed germ cell tumor with metastases to the lungs and liver. Given the high burden of lung metastases, traditional BEP  chemotherapy was deferred in favor of VIP  chemotherapy. During his hospital stay he complained of persistent headaches, and a brain magnetic resonance imaging (MRI) showed multiple small brain lesions with the biggest measuring six millimeters in the right temporo-parietal region. He started the first cycle of his induction chemotherapy as an inpatient with VIP, as well as methimazole and propranolol for hyperthyroidism. On day two of chemotherapy, he became acutely hypoxemic and developed ARDS requiring intubation with intensive care unit (ICU) transfer. Chest CT demonstrated new ground-glass opacities surrounding the pulmonary nodules  and ARDS . Due to Using the search engine PubMed, case series and case reports documenting CS and/or ARDS developing shortly after chemotherapy were collected and summarized in Table The identification of CS and ARDS has evolved with time. As can be seen in Table The HCG level remains highly significant for testicular cancer prognostication and treatment. Post-orchiectomy HCG levels are key determinants of both non-seminomatous testicular cancer staging and risk group stratification. There are well-defined, evidence-based HCG thresholds of prognostic significance, including HCG > 50,000 mIU/ml which delineates the poor risk subgroup. Additionally, tumor markers including HCG hold such importance in testicular cancer that their levels are directly incorporated into testicular cancer staging, thereby extending far beyond the treatment-monitoring role assigned to tumor markers in other cancers. In extenuating circumstances, a markedly elevated HCG level in a male patient with a testicular mass prompts initiation of chemotherapy even without waiting for histologic confirmation of testicular cancer.The quantitative significance of the HCG level with respect to CS risk is not well-defined. Due in part to the rarity of CS, there are no evidence-based thresholds for HCG level in relation to CS risk. There are general trends that appear to be present in the reported cases, with a clear tendency toward markedly higher HCG levels in CS. This poses the question of whether the risk of CS can be mitigated both before and after chemotherapy treatment in a patient who has an especially high HCG level at presentation. It has been suggested to modify one’s treatment regimen for cases of elevated HCG with pure choriocarcinoma histology and widespread lung disease . One canOur patient had a similar presentation to the cases described in the reports in Table Another surprising finding was the low TSH measured on admission. Most cases of TNSGCT that present with hyperthyroidism usually have a markedly elevated HCG . In thisCS is an extremely lethal complication that may occur post chemotherapy in high-risk patients with TNSGCT of pure or mixed histology and even mildly elevated levels of HCG. Despite using the chemotherapy regimens recommended for this high-risk group, CS may still occur. Clinical and laboratory evidence are well-defined for the staging and risk stratification of testicular CC; however, more robust studies are required to define high-risk groups for CS and paraneoplastic hyperthyroidism.


Congenital vocal fold paralysis (VFP) is an important cause of respiratory compromise in infants. It can either be unilateral or bilateral, while imaging is routinely performed for bilateral VFP to evaluate for potential neurological causes, and such a practice may not be routine for unilateral VFP. While many of the unilateral VFP cases are idiopathic, the cause may occasionally be more sinister in nature, such as tumors. Therefore, unless an obvious cause of unilateral VFP is present (such as cardiac surgery or birth trauma), routine imaging of the brain, neck, and mediastinum should be performed for congenital unilateral VFP. We describe a rare case of a cervical neuroblastoma presenting with unilateral VFP that was detected only on imaging, thus highlighting its value and importance. Congenital vocal fold paralysis (VFP) is an important cause of respiratory compromise in infants . It can A full-term 30-day-old girl born via normal vaginal delivery with no significant perinatal history developed increasing dyspnea, hoarseness, cyanosis with feeding, and lethargy. She was treated for respiratory syncytial virus (RSV) pneumonia and required supplemental oxygen and parenteral feeding. The feeding with cyanosis persisted after five days, prompting a visit to our hospital for a second opinion. Upon arrival, the patient had an oxygen saturation of 89% on room air and was admitted to the ward for further management. The oxygen saturation improved to 100% with supplemental oxygen. She was alert with no audible stridor at rest but had mild subcostal retractions. The heart and breath sounds were normal. Otolaryngology was consulted and bedside nasoendoscopy reviewed a left vocal fold paralysis. The right vocal fold was mobile and the glottic gap was three millimeters. The remainder of the scope findings was normal. There were no palpable neck masses, and thorough examination of the ears and oral cavity was normal as well. Chest X-ray showed mild air space consolidation at the right perihilar region and possibly the left retrocardiac region, likely related to underlying pneumonia. The patient was treated presumably for aspiration pneumonia and was weaned off supplemental oxygen after a few days.To evaluate the unilateral vocal fold paralysis, a magnetic resonance imaging (MRI) scan was performed. This revealed a nonenhancing left carotid space mass measuring 2.4 × 1.5 × 1.3 cm , with coThe patient underwent excision of the left neck mass. Intraoperatively, the mass was seen arising from within the carotid sheath and extending superiorly into the base of the skull. Attempts were made to completely excise the mass, but this was not possible due to tumor adherence to the skull base. The mass also encased the left vagus nerve and was adherent to the left hypoglossal nerve; it was dissected free from these nerves which were identified and preserved. A few prominent adjacent jugulodigastric nodes were also excised. Final histology of the mass returned as poorly-differentiated neuroblastoma, with metastatic tumor present in two of the three adjacent jugulodigastric nodes.Postoperatively, the patient underwent MRI of the neck, chest, and abdomen. This showed residual tumor at the skull base, a few prominent right cervical lymph nodes (likely reactive), and no metastatic disease elsewhere in the body, especially in the mediastinum, retroperitoneum, and adrenal glands. Aside from persistent dysphagia requiring nasogastric tube (NGT) feeding, the patient had an uneventful recovery and was discharged well on postoperative day seven.The multidisciplinary tumor board assessed the tumor to be of low-risk and recommended close observation, as neuroblastomas may spontaneously regress . UnfortuClinically, the patient's hoarseness improved over time, and she was initiated on oral feeding by her parents at eight months of age, who declined formal swallowing assessment before initiation. The patient tolerated this well with no clinical signs of aspiration. At age one year, she was developmentally normal and could babble, cruise, and wave on command. Nasoendoscopic examination at age 15 months showed a persistent left vocal fold palsy with right vocal fold compensation. The glottic gap remained adequate.The approach to VFP involves eliciting a proper history and performing a thorough clinical examination for evidence of congenital or neurological anomalies, iatrogenic surgical causes, or birth trauma . This isOnce the diagnosis of VFP is established, its underlying cause needs to be investigated. However, bilateral VFP cases routinely undergo imaging to evaluate for potential neurological causes , 12, sucIn this case, cervical neuroblastoma was the cause of the patient's congenital unilateral VFP. Neuroblastomas are the most common extracerebral solid tumors of infancy and childhood, with a reported incidence of 10.2 cases per million children under 15 years of age . They arIn conclusion, the authors propose that unless an obvious cause of unilateral VFP is present (such as cardiac surgery or birth trauma), routine imaging of the brain, neck, and mediastinum should be performed to investigate congenital unilateral VFP.


Primary right brachium pontis germinoma with hypertrophic olivary degeneration (HOD) is extremely rare. A preoperative diagnosis is challenging due to the absence of characterized clinical and neuroimaging features, and biopsy should be considered.A 20-year-old male patient presented with a case of primary intracranial germinoma originating from right brachium pontis with HOD manifesting as ocular myoclonus, nystagmus in both eyes, ataxic gait and incoordination of the limbs. Magnetic resonance imaging (MRI) revealed an irregular patchy lesion with hyperintensity on T2-weighted images (T2WI) and T2 fluid-attenuated inversion recovery (FLAIR) without enhancement by gadolinium (Gd). Furthermore, a focal hyperintense nodule on T2WI in the left inferior olive nucleus (ION) of the medulla oblongata was considered hypertrophic olivary degeneration (HOD) based on the patient’s symptoms and neuroimaging findings. Due to suspected demyelinating disease and low-grade glioma (LGG), a biopsy was planned. The pathological diagnosis was germinoma. Subsequently, he received chemoradiation therapy, resulting in the improvement of neurological deficits and the disappearance of the lesion on MRI.A case of “Primary right brachium pontis germinoma with HOD” is reported for the first time. A preoperative diagnosis is challenging due to the fact of absence of clinical signs and symptoms and neuroimaging characteristics. However, patients can have favourable prognoses with appropriate evaluation and treatment.The online version contains supplementary material available at 10.1186/s12883-021-02238-0. Central nervous system germ cell tumours (GCTs) represent a class of rare tumours with incidences ranging from 3.6% in North America to 15.3% in some areas of Asia, and they commonly occur in children and adolescents , ventric18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) examination found a hypodense lesion in the right brachium pontis showing slightly decreased metabolism with a maximum standardized uptake value (SUVmax) of 6.3  , which may occur secondary to pontine haemorrhage, tumour, demyelination, infection, or postsurgery. In the present case, the lesion involved the dentate nucleus, the dentato-rubro-olivary component, and the main pathway of the triangle of Guilain and Mollaret, which constituted the connection between the red nucleus, ipsilateral inferior olivary nucleus, and contralateral dentate nucleus. The pathological manifestations of HOD include vacuolation and enlargement of neurons, astrocytic hyperplasia, proliferation with gliosis and demyelination , and hype masses . On 18FDncreased . There isarthria .Germinomas are sensitive to radiation therapy, and patients with newly diagnosed intracranial GCTs should be considered for radiation therapy and synergism with chemotherapy to decrease the dose and volume of irradiation . Whole-bPrimary right brachium pontis germinoma with HOD is reported for the first time. A preoperative diagnosis is challenging on account of the absence of representative clinical and neuroimaging characteristics. In contrast, the patient could have favourable prognoses with appropriate evaluation and therapy.Additional file 1: Figure S1. The follow-up MRI (16 months later). Axial T2WI (A) and FLAIR (B) MRI demonstrate that the lesion in the right brachium pontis disappeared. Axial T2WI (C) and FLAIR (D) demonstrate that the hypertrophic left ION disappeared.Additional file 2: Figure S2. Axial T1WI (a) and T2WI (b) MR images revealed HOD after chemoradiotherapy (red arrow).


Association between cerebral aneurysms and sellar tumors has been previously reported. Rupture of anterior circulation aneurysms during a transsphenoidal surgery causing massive subarachnoid hemorrhage (SAH) is uncommon, but rupture of a posterior circulation aneurysm is an infrequent event.We present three cases of SAH secondary to rupture of an undetected posterior circulation aneurysm during transsphenoidal surgery to treat a sellar tumor. The common factor in these cases was the adverse outcome despite treatment. The fatal outcome seen in all these cases questions whether to include a (magnetic resonance) MR angiography or (computed tomography) CT angiography during preoperative evaluation for sellar tumors in order to identify inadvertently associated aneurysms. Pituitary tumors coexisting with cerebral aneurysms are a well-recognized association, but their frequency has not been definitively established.Some studies report an incidence of 3.7%-7.4%, with 97% of the aneurysms located in the anterior circulation -4. The oThis work presents a case series of three patients operated on for a sellar tumor through a transsphenoidal route as scheduled cases. In all three, an unexpected sudden neurologic impairment established the diagnosis of a massive SAH, and rupture of a non-detected posterior circulation aneurysm on preoperative imaging studies was shown to be the cause.Case 1This 38-year-old male presented occasional galactorrhea for the past six years. He started experiencing progressive visual loss two years ago, leading to the ophthalmologist who referred the patient to neurosurgical evaluation because bitemporal hemianopsia was found. Prolactin levels (PRL) were 3430 ng/mL, and the magnetic resonance imaging (MRI) showed a sellar/suprasellar mass bulging into the prepontine cistern and involving both cavernous sinuses Figure -1C. MediDuring the transsphenoidal approach, a sudden diaphragma sellae descent with systolic hypertension at 240 mm Hg and bradycardia prevented from continuing the procedure. A considerable pinkish secretion came out of the endotracheal tube, with a suspicion of pulmonary edema. Marked bilateral dilated pupils required an emergent unenhanced CT, revealing a severe SAH centered on the peri-mesencephalic cistern with reflux into the ventricles and moderate ventricular enlargement Figure -1E. AfteThe postoperative course was complicated by diabetes insipidus, right middle cerebral artery vasospasm seen on transcranial insonation, and delayed cerebral ischemia. Diffuse infarcts from the right midbrain, thalamus, and cerebral hemisphere finally lead to the patient's death on day 15 Figure -1I.Case 2Six weeks before admission, this otherwise healthy 15-year-old male teenager initially presented with ocular pain, visual acuity impairment, frontal pulsatile exertional headache, recent nausea, and vomiting. The eye examination found papilledema, and loss of visual acuity on both eyes, while abduction of the left eye was limited. The imaging workup revealed a 43 × 27 × 28 mm sellar and suprasellar tumor, most likely a craniopharyngioma with secondary hypogonadotropic hypogonadism Figure -2C.The patient was uneventfully managed with a right frontal ventriculoperitoneal (VP) shunt for obstructive hydrocephalus and a transsphenoidal resection. Heavy bleeding from the sellar area was controlled during surgery, but a postoperative CT revealed a Fisher 4 SAH Figure -2F. An eCase 3A progressive visual impairment led this 45-year-old male to the ophthalmologist, who referred the patient with a typical chiasmatic visual field defect. The MRI study revealed a sellar lesion 1.8 × 1.0 × 1.5 cm in dimensions Figure -3C. As aTranssphenoidal surgery is one of the procedures of choice to remove pituitary adenomas and other sellar tumors, with mortality and morbidity rates below 0.5% and 1.5%, respectively . CommonlCerebral aneurysms coexist with pituitary tumors more often than expected, and their presence on the major arteries adjacent to the pituitary and suprasellar tumors entails considerable risks, particularly when the aneurysm is closer to the operative field ,11-13. TPrevious reports locate most of these aneurysms in the anterior circulation, particularly in the anterior communicating or the internal carotid arteries, possibly because these arteries are related to the blood supply to the sellar region -6,11,13.On the contrary, the occurrence with posterior circulation aneurysms is unusual and has been reported only twice. One was an autopsy case where a pituitary adenoma was associated with a giant vertebrobasilar aneurysm, and the other described a fatal SAH from a basilar apex aneurysm in the postoperative period ,14. RuptDespite its rarity, postoperative SAH can have dismal consequences, as shown in this series, and it might deserve further consideration. Given its technical feasibility during the preoperative imaging workup without significantly increasing healthcare costs or scanning time, we wonder if complementary preoperative MR angiography or CT angiography should be performed, mainly when there is a mechanical involvement or closeness to vascular structures. Additionally, DSA should be mandatory when an associated aneurysm is detected preoperatively. In such cases, a better plan can be done including the dome protection through an endovascular route before the transsphenoidal surgery to avoid an eventual rupture. Another option is the treatment of both, the aneurysm and the adenoma, through a transcranial route at the same time.Even when anterior circulation aneurysms are more commonly associated with pituitary adenomas , posterior circulation aneurysms are not common in such situations. Focused preoperative imaging might be a way to prevent unforeseen catastrophic events. Moreover, this point remains controversial and demands further investigations.The coexistence of sellar and suprasellar tumors and posterior circulation aneurysms are rare. The occurrence of SAH after tumor removal is exceedingly exceptional and has rarely been reported. However, the fatal outcome of this unexpected event in an otherwise nonmortal disease deserves further study to determine if these cases could benefit from a more extensive workup vascular imaging in the preoperative stage.


Grossly, a large encapsulated tumor was identified arising from the mesentery of the small bowel. The histological examination showed a tumor consisting of epithelioid to spindle cells loosely arranged in a myxoid background with numerous blood vessels and lymphoplasmacytic inflammatory infiltrate. On immunohistochemistry, the tumor cells showed positivity for ALK1 (nuclear), desmin, SMA, CD68, and focal positivity for CD30. A final diagnosis of EIMS of the small intestine was rendered. To the best of our knowledge, this case is the youngest reported case in literature.Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of the inflammatory myofibroblastic tumor. It has an aggressive clinical course and a high rate of recurrence. EIMS primarily affects children and young adults. Hereby, we report this entity in a 4-month-old infant who presented with an abdominal mass. Imaging studies revealed a large hypodense mesentery-based lesion involving the right half and mid-region of the abdomen. The mass with an attached segment of the small bowel was excised  Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm with borderline malignant potential. It often affects children and young adults with the mean age at diagnosis of 10 years. It mostly arises in the mesentery, omentum, and abdominal soft tissue, followed by lung, mediastinum, and head and neck.in toto, along with mesenteric lymph nodes. On gross examination, a large encapsulated tumor (12x11x7cm) was identified arising from the mesentery. The external surface of the mass was encapsulated and lobulated. The cut section was greyish-white and glistening with a myxoid appearance and soft to firm in consistency  revealed a large hypodense abdominal lesion (11.4x11x8cm) involving the right half and mid-region, displacing the bowel loops posterolaterally. However, tumor invasion was not seen. No evidence of septations or solid enhancement was seen within the lesion. Clinically, the diagnosis of mesenteric cyst or teratoma was considered. The infant was operated on, and an encapsulated irregular mass attached to a small bowel segment was excised sistency .On the histopathological examination, the tumor consisted of epithelioid to spindle cells loosely arranged in a myxoid background with numerous blood vessels .The tumoBased on the morphology and immunohistochemistry results, a final diagnosis of epithelioid inflammatory myofibroblastic sarcoma of the small intestine was made. Both gross and microscopic examinations from the small bowel’s attached segment were unremarkable and not involved by the tumor. Few lymph nodes were dissected out which showed reactive lymphoid hyperplasia. The immediate postoperative period was uneventful. The patient was discharged after ileo-ileal anastomosis, in a stable condition and normal passage of stool. On follow-up after 6 months, the general condition of the patient was fair, and she did not have any complaint.-th case of small bowel mesentery and the youngest among all the reported cases.EIMS occurs mostly in children and adolescents and has a male predilection, contrary to the conventional IMT, which has a slight female predominance. The exact frequency of EIMS remains underestimated, as it has not been widely documented. Only 43 cases have been reported in the literature.ALK gene located on chromosome 2p23.-EIMS often displays the following common histomorphological and immunohistochemical characteristics: i) round to epithelioid tumor cells with scattered inflammatory infiltrates, ii) abundant myxoid stroma, iii) expression of ALK (nuclear membrane), and desmin .,Microscopic diagnosis of EIMS can be challenging due to its unique epithelioid to round cell morphology and atypical nuclear features, which resembles many other tumors like a gastrointestinal stromal tumor (GIST), anaplastic large cell lymphoma (ALCL), and epithelioid leiomyosarcoma (ELMS), rhabdomyosarcoma (RMS), low-grade fibromyxoid sarcoma (LGFMS) and malignant mesothelioma (MM). ALCL can be difficult to distinguish from EIMS, as the rare sarcomatoid variant of ALCL can exhibit spindle cell morphology and overlapping immunostaining, including reactivity for CD30, ALK, and SMA and non-reactivity for EMA. However, strong expression of desmin and the distinctive nuclear membrane pattern of ALK staining are not observed in ALCL. The RANBP2-ALK has never been reported in ALCL either. Epithelioid GIST is positive for CD117, DOG1, CD34 while negative for ALK staining. Mutations of c-Kit and platelet-derived growth factor-α are also present in GIST. ELMS usually displays higher cellularity and pleomorphism. Also, it generally lacks an extensive myxoid background, inflammatory infiltrates, and ALK expression. The solid variant of alveolar RMS is frequently ALK-positive; however, it lacks fibrovascular stroma and forms sheets of round cells with variable rhabdomyoblastic differentiation. Myogenin and MyoD are highly specific and sensitive for its diagnosis. LGFMS shows bland spindle cells with a whorling growth pattern and arcades of curvilinear blood vessels in an admixture of collagenous and myxoid stroma. LGFMS is positive for MUC4 and EMA, focal SMA, and desmin positivity while not expressing ALK. CK5 and calretinin are positive in MM, but ALK and desmin are absent.3The mainstay of treatment for IMT/EIMS is complete surgical resection when possible. However, recurrence is common. A few case reports described the combined therapy of surgery and systemic therapy with ALK inhibitors when an ALK mutation is present.It is important to recognize EIMS as a distinct variant of IMT as patients with ALK-rearranged EIMS may benefit from targeted therapy.th case reported in the mesentery of small bowel and youngest among all the reported cases to the best of our knowledgeEpithelioid inflammatory myofibroblastic sarcoma is a rare and distinct variant of IMT, which mainly consists of cells with an epithelioid or round cell morphology, with a high potential of recurrence and a poorer prognosis. Only 43 cases have been reported in the literature, of which only 9 were located in the mesentery of the small bowel. This is the 10