Although a recognized condition, growing teratoma syndrome (GTS) has no guidelines for management, and patients diagnosed with the condition are managed empirically by the most appropriate teams. We report a case of GTS in a 33-year-old patient who was initially treated with unilateral salpingo-oophorectomy and subsequent chemotherapy for a germ cell ovarian tumor. GTS was subsequently diagnosed with massive pelvic and upper abdominal masses as well as lung tumors. We also conducted a literature review on cases of GTS presenting with large tumors. Based on this, we suggest a management plan to guide the care of women with GTS. The condition is best managed in a multidisciplinary team involving the relevant surgeons, including gynecologist, abdominal and thoracic surgeons. Growing teratoma syndrome (GTS) is a rare but recognized condition presenting with an abdominal mass in a woman with history of malignant ovarian tumor treated with chemotherapy. There are no established guidelines for the management of women diagnosed with GTS. We report a case of GTS in a 33-year-old patient requiring major abdominal and thoracic surgery. We also review the literature on previously published cases of GTS and propose a management plan to guide clinicians looking after women with GTS. Collaboration between different surgeons including gynecologists, abdominal and thoracic surgeons as well as radiologists and oncologist should be sought. Growing teratoma syndrome (GTS) is a rare condition, which presents with benign (mature) teratoma masses either during or after chemotherapy . It is aDiagnostic criteria of GTS include: normalization of serum tumor markers such as α-fetoprotein (AFP) and human chorionic gonadotropin; visualization of enlarging or new masses despite appropriate chemotherapy for NSGCT; and exclusive presence of mature teratoma only in the resected specimen . The exaIn order to highlight current insights into GTS management, we report a case of GTS, presenting with a large pelvic mass 2 years after chemotherapy for immature teratoma. We defined ‘large’ as a mass measuring above 10 cm in at least one dimension. We also conducted a literature review on cases of GTS presenting with large tumors. We chose this subgroup as tumors of this size are more challenging to manage surgically.Our aim was to improve our understanding of this rare condition and identify strategies to assist in earlier diagnosis and management.A 33-year-old multiparous woman of Indian descent presented in July 2009 to a district general hospital with abdominal bloating and pain. The patient underwent a transvaginal ultrasound scan, which showed a 12-cm left adnexal lesion with suspicious features, namely papillary projections, multiple loculations and increased vascularity. AFP was raised at 1100 ng/ml. The above prompted referral to a tertiary center and an urgent computed tomography (CT) of the chest, abdomen and pelvis did not show evidence of peritoneal or distant metastasis. Fertility-sparing surgery was performed with an open left salpingo-oophorectomy, omentectomy and peritoneal washings in September 2009. There was no macroscopic disease in the abdomen but the ovarian capsule was breached. Macroscopic clearance was achieved during surgery. Histology confirmed mixed germ cell tumor, consisting of a high-grade immature teratoma and a yolk sac tumor. Radiological and surgical staging were consistent with Stage 1C.Because of persistently elevated AFP levels postoperatively, she underwent two cycles of chemotherapy consisting of bleomycin, etoposide and platinum. Disease progression was noted during these two cycles so her chemotherapy was switched to irinotecan, paclitaxel and oxaliplatin for further four cycles. CT at the end of chemotherapy revealed multiple cystic/solid lesions in the pelvis (arising from the right ovary), abdomen  and lungs, which at that stage, were deemed inoperable. Positron emission tomography did not reveal any hot spots, and tumor markers were normal. At that stage, the patient did not complain any relevant symptoms and for this reason, she was managed expectantly.A year later, she presented with abdominal distension. A 20 cm pelvic mass arising from the right ovary was shown on CT. Given the size and location of the lesions, the decision was made to proceed with hemi-hepatectomy, cholecystectomy, resection of right hemidiaphragm and left lung lobectomy. The multidisciplinary team deemed surgical intervention was required to obtain a histological diagnosis and prevent morbidity due to pressure of vital structures. No gynecological surgery was performed at that stage because of the extent of the upper abdominal and thoracic surgery. All specimens showed a benign mature cystic teratoma. Three months later, completion debulking surgery was performed involving the removal of the uterus, remaining right tube and ovary, omentum and spleen. Again, histology showed a mature cystic teratoma in all specimens.Three years later (in July 2013), the patient underwent another operation to remove an anterior abdominal wall lesion, which was also reported as mature cystic teratoma on histology. Ten years later, in 2019, the patient is on hormone-replacement therapy, managing well with no evidence of recurrence in subsequent scans and tumor markers.Eleven case reports, published between 1983 and 2014, were identified in the literature where GTS presented with a tumor larger than 10 cm following chemotherapy for NSGCT  2,6–15]6–15]. WiIn all studies, the primary pathology was managed by debulking surgery. This was either unilateral salpingo-oophorectomy or hysterectomy with bilateral salpingo-oophorectomy followed by adjuvant chemotherapy ,6–15. Thet al. who performed a review of all cases of GTS until February 2016 reported a mean interval of 27 months from chemotherapy [In our review, GTS seems to present at an average 15 months from chemotherapy. Li otherapy . Any patotherapy .High index of suspicion and earlier diagnosis can have a significant effect on the management of the patient with GTS. Although a benign condition, GTS requires debulking surgery because of mechanical obstruction and pressure to vital structures . The earAlthough a recognized entity, no consensus or guidelines on the management of GTS exists. The patients are managed empirically, usually by a multidisciplinary team of surgeons, oncologists and radiologists. Our literature review confirmed the young age, which NSGCT, and subsequently GTS, present . This stresses the importance of early diagnosis and appropriate management to minimize the need for radical surgery and subsequent loss of fertility. We, therefore, propose an algorithm to guide the management of the condition .We suggest three-monthly follow-up appointments with tumor markers and ultrasound examinations for the first year after treatment of NSGCT to ensure early detection of disease recurrence or development of GTS. Six-month surveillance may ensue for the second year, followed by yearly surveillance for at least further 3 years post-treatment. CT scan surveillance can be six monthly for the first year, and yearly from then on. CT imaging is a useful modality and demonstrates characteristics of radiographic maturation including increased density, better circumscribed margins, onset of internal calcifications, amalgamation of fat and solid/cystic components . PositroManagement plans following a positive CT scan should be made within a multidisciplinary team setting involving surgeons, oncologists and radiologists. Surgery is the mainstay of treatment, and histology confirms the diagnosis. Furthermore, it avoids the need for further chemotherapy, which may have an adverse effect to the patient's reproductive potential. Surgical exploration and complete resection when possible is suggested to reduce the risk of recurrence necessitating more radical surgery, which is associated with increased morbidity and mortality. Fertility-preserving surgery may be reasonable in selected women who have been adequately counseled . CarefulWith partial resection, the recurrence rate for GTS has been quoted between 72 and 83% while with complete resection, the recurrence is considerably reduced to 0–12.7% ,19. MaliThe 5-year overall survival rate of patients who had undergone surgery following GTS is 89% ,29.GTS is a rare benign condition, which occurs following chemotherapy for NSGCTs. Improved awareness and follow-up may lead to earlier diagnosis and subsequently less extensive surgery. The condition is best managed in a multidisciplinary team involving the relevant surgeons, including gynecologist, abdominal and thoracic surgeons. In addition, oncologists, radiologists and menopause and fertility specialists need to be involved in the management of these women who need to be counseled about the short- and long-term effects of debulking surgery for GTS.We envisage that the management of GTS will be standardized based on international guidelines and protocols, and centralized in tertiary centers. We expect that, as the scientific community is becoming more aware of this rare condition, large cohort studies will be conducted to further add to the pool of knowledge. The latter, as well as the management algorithm proposed in this review, should promote standardization of care of GTS.Growing teratoma syndrome (GTS) is a rare condition presenting with benign (mature) teratoma masses either during or after chemotherapy for malignant (immature) teratoma.Although a recognized condition, GTS has no management guidelines.In this paper, we report a case of GTS managed successfully with multiple surgeries under a multidisciplinary team of gynecological oncologists, hepatobiliary and thoracic surgeons.After a thorough literature review we are proposing an algorithm to guide clinicians in the management of GTS.


Transarterial selective internal radiation therapy with yttrium-90, also known as radioembolization, is a therapy based on the administration of resin or glass microspheres loaded with the radioisotope yttrium-90, via selective arterial catheterization of tumor-feeding vessels. It is classified as a type of locoregional therapy and its main goal is to treat patients with primary or secondary hepatic lesions that are unresectable and not responsive to other therapies. Since it is a new technology still restricted to very few hospitals in Brazil, but used in patients throughout the country, it is necessary to demonstrate the main aspects of hepatic lesions treated with selective internal radiation therapy found in magnetic resonance imaging, and to make specific considerations on interpretation of these images. The objective of this report is to demonstrate the main aspects of magnetic resonance imaging of unresectable primary or secondary hepatic lesions, in patients submitted to transarterial selective internal radiation therapy. The transarterial selective internal radiation therapy (SIRT) with yttrium-90 is a locoregional therapy based on administration of microspheres loaded with yttrium-90, through selective arterial catheterization of tumor-feeding vessels. The main objective is to treat patients with primary or secondary hepatic lesions that are unresectable and do not respond to other therapies.,2μ (20 to 60 μ ) and the microspheres do not cause an embolic effect, that is, there is no occlusion of the tumor-feeding vessels. Yttrium-90 is a high-energy radioisotope that emits beta radiation with a maximum reach of 11mm (mean penetration: 2.5mm) and a half-life of 64.1 hours.By passing the catheter through the common femoral artery, and using a coaxial catheter system, the selective catheterization of the celiac trunk and of the hepatic artery is performed; then, the microparticle set (yttrium −90) is selectively delivered to the hepatic arterial circulation , which feeds the neoplastic region. The microspheres are permanent implant (they are not absorbed) and used only once in each patient. Their mean diameter is 32.5 to 60 μ ),4 an(16)The procedure is performed by a team of interventional radiologists. However, a successful treatment demands involving a multidisciplinary team, comprising professionals from Diagnostic Radiology, Nuclear Medicine and Oncology, aiming to properly select the patients, check tumor staging, look for liver to lung shunt, calculate the yttrium-90 dose, and follow-up the treated lesions.It is a new technology available at three hospitals in Brazil, but used in patients from throughout the country. It is important to demonstrate the main aspects of images of hepatic lesions treated with SIRT and found in magnetic resonance imaging (MRI), and make specific considerations on interpretation of these images. Therefore, this report aims to contribute to expanding knowledge in Brazilian Radiology.The purpose of this study was to demonstrate the main aspects found in MRI of primary or secondary unresectable hepatic lesions treated with SIRT.All patients signed an Informed Consent before the procedure and follow-up exams. Our organization does not require approval by the Ethics Committee for case reports.We used MR images of patients treated by the SIRT technique at a quaternary care hospital, from November 2014 to July 2016. A total of 35 SIRT/yttrium-90 procedures were performed, and four cases were selected for presenting characteristic MR images found in this therapy.The images were acquired in 3T MRI devices  and 1.5T . The protocol used to assess the hepatic lesions included the sequences: coronal T2, axial diffusion, axial T2 (TE 80), axial T2 (TE 180), axial T1 gradient-echo (in phase/out of phase), fat saturation axial T1 before and after , and post-contrast fat saturation axial T1. Two control exams were performed, two and six months after the procedure.A 56-year-old male patient, diagnosed as pancreatic adenocarcinoma in March 2014, associated with multinodular hepatic metastasis. He initiated on chemotherapy with FOLFIRINOX for four months (discontinued due to systemic toxicity), changed to chemotherapy with gemcitabine, for 4 more months. He presented partial response of the pancreatic tumor, progression of hepatic lesions, and worsening of tumor markers .Abdominal MRI after second-line systemic chemotherapy showed growth of secondary hepatic lesions in segments II, IVa   A, VII  B and VIThree months after SIRT, there was virtually complete necrosis of the lesions observed in the previous MRI   D to 1 FA 66-year-old female patient, diagnosed as multiple hepatic metastases of an adenocarcinoma of unknown primary site, according to percutaneous hepatic biopsy. Past history of breast cancer appropriately treated six years ago.An abdominal MRI showed a bulky hepatic tumor occupying preferably the right hepatic lobe (white line), not involving the portal vein, but it was unresectable   A. ThereMagnetic resonance imaging was performed after 60 days and revealed extensive necrotic areas of infiltrating lesions in the right hepatic lobe, besides complete necrosis of the lesion in the gallbladder bed   E.A 57-year-old male patient was diagnosed, in 2012, as urothelial cell carcinoma in the left kidney, with lymph node involvement, after presenting macroscopic hematuria. He was submitted to nephroureterectomy followed by systemic chemotherapy with MVAC protocol  during 6 months. He remained with no evidence of the disease for 22 months.In March 2015, the MRI showed a single liver metastasis. Stereotactic body radiotherapy (SBRT) was performed, and he underwent four chemotherapy cycles of gemcitabine and carboplatin, remaining with no evidence of disease for 6 months. In December 2015, in a control MRI, multinodular liver metastases were observed   A, besidTwo months after SIRT, the MRI showed larger tumor lesions and exuberant necrotic areas   B. The mA 71-year-old patient suffering from hepatitis C, liver cirrhosis and advanced hepatocellular carcinoma with portal vein thrombosis, not eligible for transplant or surgical resection. Magnetic resonance imaging showed an expansive formation compatible with infiltrative hepatocellular carcinoma involving nearly all right lobe, several satellite lesions   A and thMagnetic resonance imaging performed six months after SIRT, displayed marked tumor involution and necrosis of the infiltrative tumor of the portal vein. In this case, two side effects of SIRT were observed: atrophy of the right lobe and mild hypertrophy of the left hepatic lobe    C.SIRT is a therapy performed in few hospitals all over the world and provides findings that are characteristic in MRI after treatment – some of them may raise doubts regarding the therapeutic response. In this report we outline the characteristic images of response to treatment; however they are not common comparing to the literature on other locoregional therapies.Hyperenhancement around the lesion is one of the findings more often observed in MRI after SIRT, corresponding to the inflammation area of the hepatic parenchyma, related to perfusion disorder, as described in case 1. This finding may be misunderstood as tumor viability if the MRI were performed after chemoembolization control.The particles administered in SIRT can be taken up in subcentimeter lesions that are not visible yet in the MRI before the procedure, and the control images may show necrotic areas in non-target regions, as reported in case 2.After SIRT, we often observe an increased volume of the tumor lesions, especially if the control image is performed within the first 30 days, what may lead to diagnostic misinterpretations, as shown in case 3. This paradoxical growth is due to appearance of a necrotic area, beyond the tumor margins seen in the previous MRI, and it does not necessarily correspond to poor response to procedure. Therefore, it is recommended to perform a control image at least 60 days after SIRT, to assess the real response to treatment.The paradoxical hypertrophy of the contralateral hepatic lobe in the SIRT, as seen in case 4, is a condition observed that mimics hypertrophy of the future remaining liver after embolization of the portal vein. The contralateral hepatic lobe may increase by up to 29%.7Considering the MRI to control the primary or secondary tumors after SIRT, and to avoid misinterpretation of results, the following imaging findings should be mentioned: necrotic areas in unexpected sites, paradoxical increased tumor volume, paradoxical hypertrophy of the contralateral hepatic lobe and atrophy of the hepatic lobe submitted to SIRT.


Bouveret's syndrome is a complication of cholelithiasis that presents with gastric outlet obstruction due to an impacted gallstone in the duodenum following cholecystoduodenal fistula. This is a rare presentation of biliary-enteric fistula; therefore, there are no standardized guidelines for the management of this disease. We present a case of a patient with Bouveret's syndrome managed with laparoscopic surgery after an unsuccessful attempt of endoscopic removal. A 58-year-old female was presented to the emergency department with a 4-day history of nausea and vomiting. The patient had a past medical history for Parkinson's disease and gallstones but denied having any previous abdominal symptoms. The patient referred abdominal distention with intolerance to oral intake and postprandial vomits of nonbilious characteristics, containing undigested food particles. She also complained of upper abdomen discomfort that relieved after vomiting.Clinical examination revealed blood pressure of 100/80 mmHg and heart rate of 97 bpm. Cardiorespiratory and neurological exam revealed no abnormalities.Abdominal examination revealed a soft abdomen with normal bowel sounds, mild epigastric tenderness, and no palpable organomegaly.Plain abdominal X-ray showed an irregular partially rim calcified focus in the right midabdomen and absence of air in the distal bowel . Abdomin3, urea 48 mg/dl, creatinine 1.02 mg/dl, AST 19 U/l, ALT 16 U/l, alkaline phosphatase 88 U/l, bilirubin 1.4 mg/dl, and amylase 102 U/l.Blood work showed hematocrit of 45%, white count of 7500 cells per mmInitial measures were resuscitation with fluids and gastric decompression with a nasogastric tube. Computed tomography of the abdomen revealed a multilithiasic gallbladder with alteration of the surrounding fatty tissue, pneumobilia, gastric distention, and a 45 mm × 32 mm calcic stone located in the duodenal bulb .We performed an upper endoscopy identifying an obstructing 4 cm stone in the duodenal bulb. Laser and mechanical lithotripsy were attempted using a Holmium probe and a Dormia basket achieving partial fragmentation of the stone , but dueTwo 12 mm and two 5 mm ports were used, all of them in the upper abdomen. Omental adhesions to the gallbladder were lysed exposing the cholecystoduodenal fistula. A decision was made not to treat the fistula due to the high risk of complications. A longitudinal gastric antrotomy was made with ultrasonic shears (harmonic), revealing a partially fragmented stone positioned in the duodenal bulb. The multiple fragments were extracted through the antrotomy with laparoscopic forceps.The antrotomy was closed with a longitudinal single layer uninterrupted 3/0 absorbable barbed suture. We performed an intraoperative upper endoscopy in order to rule out any air leaks and confirm adequate passage of the scope through the second and third duodenal portions. Finally, the stone fragments were extracted in a retrieval bag, and a Jackson-Pratt drain was placed in the parieto-hepatic recess. Wounds were closed with 2/0 vicryl and 4/0 monocryl.The patient was extubated after surgery and transferred to a general ward. On postoperative day (POD) 4, we performed a fluoroscopic contrast study showing no leaks or obstructions to the passage of the contrast solution . TherefoThe patient was discharged home on POD 12 without any major complications other than an epigastric wound infection that required drainage and oral antibiotics. Outpatient visit one week after discharge revealed adequate oral intake without vomiting or pain. Evaluation over the following months ruled out any complications or recurrence of symptoms.Common complications associated with cholelithiasis include acute cholecystitis, choledocolithiasis, and pancreatitis, while biliary fistula and gallbladder cancer are uncommon manifestations of long-term gallstone disease. Bouveret's syndrome is a rare complication of cholelithiasis that presents with gastric outlet obstruction due to an impacted gallstone in the duodenum following cholecystoduodenal fistula .Cholecysto-bowel fistula's physiopathology includes chronic inflammation of the gallbladder, firm adhesions to the bowel wall, and an impaired blood supply and venous drainage. This associated with the compression of the gallstone against the gallbladder wall results in necrosis, fistula formation, and subsequent passage of the stone to the bowel developing a gallstone ileus , 3. The Bouveret's syndrome was first described by Beaussier in 1770 and subsequently named after the French physician Léon Bouveret, who published two cases in 1896 . The disThese patients are usually screened with an abdominal X-ray. Some of the radiologic findings in these patients are pneumobilia (39% of the cases), ectopic gallstone (38%), and distended stomach or bowel (23%) . These cMost cases present with only one impacted stone, and its removal can be attempted endoscopically with limited success (9%). Usually a mechanical laser or extracorporeal shock wave lithotripsy combined with snares, baskets, and grasping forceps are used; however, most cases require surgery because of the size of the stone .In this scenario, treatment can be aimed at either removing the stone in order to solve the obstruction, removing the gallbladder, and repairing the fistula in a one-step surgery or removing the stone and differing the cholecystectomy and repair of the fistula for a second attempt . FurtherThe advantages of the one-stage approach include avoiding the necessity of future procedures and the risk of developing cholecystitis, recurrent gallstone ileus, cholangitis, cholangiocarcinoma, and gallbladder cancer, developing in 5-17% of the cases . The incConsidering this, we preferred the two-step over the one-step approach and opted for an initial endoscopic attempt, reserving the laparoscopic approach in case of failure. We believe one-stage procedure should be restricted to relatively young patients in good overall condition.Bouveret's syndrome is a rare condition that should be considered in elderly patients with a history of chronic cholelithiasis, epigastric pain, and vomiting.Surgical options include a combination of enterolithotomy plus cholecystectomy and fistula repair in a one- or two-stage approach.Endoscopic treatment should be considered as a first attempt, despite the low success rate reported in the literature. If endoscopic treatment fails, surgical treatment should be carried out.This case report suggests that a laparoscopic approach may represent a valid therapeutic option after unsuccessful endoscopic treatment.


Surgical excision of the submandibular salivary gland in patients with chronic sclerosing sialadenitis can be complicated because of hyalinosis of the surrounding soft tissues. Patients with autoimmune diseases suspected of having salivary gland neoplasms or granulomatous disease should be carefully investigated for chronic sclerosing sialadenitis to prevent potential harm. She had a 1‐year history of a hard, painless mass present in the submandibular region, which worsened in the 10 days prior to admission, with swelling of the submandibular region and fever. On palpation, the right submandibular salivary gland was hard and painful. Palpation and massage of the submandibular duct was painful, and there was no saliva evacuated from the duct.Ultrasound examination revealed enlargement of the right submandibular gland measuring 54.4 × 29.9 mm, with unclear borders. The echotexture revealed diffuse changes with low echogenicity, local anechoic areas, and hardened zones with severe peripheral vascularization. The gland was surrounded by a small amount of free fluid, and there was mild enlargement of the lymph nodes, measuring 13.0 × 13.3 mm  count was −3.56 × 1012/L, her lymphocyte (LYMPH) percentage was 23.3%, and her erythrocyte sedimentation rate (ESR) was 50 mm/h. Submandibular gland extirpation by urgent indications was planned for this patient.After a 7‐day course of antibiotic therapy, the patient noted reduction in pain and swelling. However, a new manifestation of inflammation was observed, and the patient was hospitalized with fever, pain, and swelling. The swelling of the submandibular space was firm, without clear boundaries. The color of the skin had not changed. Her hemoglobin (HGB) level was 109 × 103. After formalin fixation, on the cut surface, the gland had a “pineapple‐like appearance with yellow and white layers”  was revealed Figure . The adj” Figure .Microscopically, histological examination revealed extensive periductal and perivascular fibrosis and sclerosis, foci of hyalinosis, infiltration by mononuclear cells (mostly lymphocytes and plasma cells), acinar atrophy, and obliterative phlebitis Figure A‐C. Thus3The exact etiology of CSS has not been established until now; however, various theories, such as salivary duct obstruction, salivary stasis, sialolithiasis, secretory dysfunction, and autoimmune response, have been proposed.Although CSS is a chronic inflammatory condition, it presents clinically as a malignancy, for example, a painless hard mass in the submandibular gland, thus creating an interesting diagnostic dilemma.The present patient had a long‐standing, painless, hard mass of the submandibular salivary gland that could manifest with features of submandibular salivary gland‐ and submandibular space‐specific infection, with symptoms of fever, pain, and firm subcutaneous tissues without skin hyperemia. The main problem in such cases is the possibility of an unclear preoperative diagnosis and unjustified scope of surgical intervention, which could occur in these patients. When the submandibular space is painful, diffuse and firm swelling in a patient with a high fever is not always responsible for space suppuration. In the present case, CSS exacerbation was manifested, which clinically mimicked acute specific infection, and the patient was operated on under urgent indications. However, there were no signs of acute inflammation intraoperatively.There are a few cases in the literature that report regional lymph nodal adenopathy in the CSS of the submandibular gland. In the presented case, regional lymphadenopathy was also reported.Ultrasound examination is a useful diagnostic tool and is initially used for the assessment of patients with enlarged salivary glands.On MR imaging, these lesions typically demonstrate homogeneous low‐to‐intermediate signal intensity on T2‐weighted images and low signal intensity on T1‐weighted images, with homogeneous enhancement. Areas of calcification that could be observed may simulate malignancy.FNA is frequently used to evaluate salivary gland neoplasms as it is a cost‐effective method with minimal risk to the patient.Surgically excised CSS lesions have a solid cut surface with preserved lobulations and fibrotic interlobular tissues that reflect the benign nature of the disease.All cases described in the literature note that the diagnosis of CSS is mainly based on histopathological examination. Intraoperative frozen section examination may be a helpful tool for intraoperative diagnosis and prevention of unreasonably large dissections.According to Seifert, the four histological stages of CSS present corresponding to Stage 3: even more prominent lymphocytic infiltration, with lymphoid follicle formation, parenchymal atrophy, periductal hyalinization, and sclerosis. Squamous and goblet cell metaplasia may also be found in the ductal system.Considering the fact that the present article describes one case report, the data may not be robust enough to explain complex issues, but this case could complement and refine the available data in the literature and provide additional information to explain this clinical situation.4Chronic sclerosing sialadenitis is a rare disease that is often clinically diagnosed as a malignant lesion. In the present case, the exacerbation period mimicked acute specific inflammation of the submandibular space, and urgent operative management was performed. Severe fibrosis and hyalinosis with the surrounded tissue made it possible to intraoperatively modify the operative technique and enlarge the volume of resection. CSS was diagnosed by the histopathologist. Patients with autoimmune diseases and salivary gland disorders that are suspicious for neoplastic or granulomatous disease should be carefully investigated for CSS to prevent potential harm.None declared.AYP: drafted the manuscript for important intellectual content and implemented the clinical work. MSM: made substantial contributions to the concept and design and was accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work were appropriately investigated and resolved. AMS: was responsible for performing an accurate literature review and implementing the clinical work. PSK: made contributions to the design of the histological examinations. GAH: was responsible for the histological data, design, and literature review.We confirm that explicit written consent to publish has been received from the described patient.


More common in major salivary glands, oncocytomas are very rare tumors. They commonly occur in the parotid gland and are painless slow growing predominantly benign tumors. The term “oncocytoma” was introduced by Jaffe to designate those tumors of the salivary glands that consist predominantly of oncocytic cells lining the salivary ducts (1) Similalry, Meza- Chavez had proposed the name “oxyphilic granular cell adenoma. (2) Oncocytomas are extremely rare, benign and slow growing in nature. Here we present a rare case of buccal oncocytoma which is to our knowledge the 19th case of intraoral minor salivary gland tumor and the 7th reported case of buccal oncocytoma.Here we present an exceedingly rare case of buccal oncocytoma in a 14 years boy who presented to the department of ENT with right buccal swelling for 6 months. He was posed the diagnosis of buccal cyst after cytological examination supported by CT scan. He then underwent an excisional biopsy where the final diagnosis was made as Buccal Oncocytoma.Though very rare in the picture, conditions like salivary gland oncocytomas still are reported on and off in the literature. The treatment of which is complete surgical excision. •Buccal oncocytoma is one of the rare entities salivary gland oncocytoma.•So far only 6 cases of buccal oncocytoma has been mentioned in literature.•The definitive treatment is surgical excision. The term “oncocytoma” was introduced by Jaffe  SimilarlThe study above was done in compliance with the SCARE 2018 statement [22 with ill-defined margins (3 lesion in the right buccal space arising from the buccinator muscle and displacing the zygomaticus major (2 in the right buccal space. The cyst contained thick mucinous fluid. The specimen was sent for histopathological examination. Findings of the histopathological examination were consistent with oncocytoma (Here we present an exceedingly rare case of buccal oncocytoma in a 14-year-old boy who presented to the Department of ORL Head and Neck Surgery with right buccal swelling for 6 months The swelling was progressively enlarging in size. There was no history of associated pain. There was no history of dysphagia. On examination, there was a single cystic swelling in right cheek around 2*2 cm margins . It was us major . With thcocytoma . He was 3Salivary gland oncocytomas are extremely rare tumors. They are benign slow growing in nature. Oncocytomas in head and neck region are more common in major salivary glands as compared to a minor. They comprise of 0.5%–1.5% of all salivary gland tumors. Meanwhile, the incidence of salivary gland oncocytomas in the parotid gland accounts for 78%–84%. It is exceedingly rare in minor salivary glands which include sites like, lower lip, palate, pharynx, tonsillar fossa, buccal mucosa. So far only 18 cases of intraoral minor salivary gland oncocytomas have been reported in the literature. Out of the 18 cases, 6 cases are of buccal oncocytoma . Thus he4Though very rare in the picture, conditions like salivary gland oncocytomas still are reported on and off in the literature. More localized minor salivary gland tumors can be treated with complete excision while those related to parotid gland might require total or superficial parotidectomy. Thus we can say that the location of the tumor is determining factor in the treatment procedure.As this is a case report, informed consent has been taken from the patient.No funding was available for the article.1-Dr.Sudha Shahi –Concept design, Review, literature search, writing paper, final decision to publish.2-Dr.Tika Ram Bhandari - Review, literature search, writing paper, final decision to publish.3. Dr.Prakash Bahadur Thapa: Supervised the writing of the manuscript, final decision to publish.4. Dr.Deependra Shrestha: Supervised the writing of the manuscript, final decision to publish.The authors state no conflict of interests.None.Dr. Sudha Shahi.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.Is a case report.Not commissioned externally peer reviewed.


A 69-year-old woman with a history of untreated hypertension presented with acute-onset monocular vision loss. Initial workup was delayed due to lack of immediate specialty consultation and dilated funduscopic exam. Point-of-care ultrasound in the emergency department identified a small hyperechoic structure within the distal area of the central retinal artery; in conjunction with specialty ophthalmologic evaluation in a tertiary care center, the diagnosis of central retinal artery occlusion was made. The patient was admitted to the neurology service for stroke risk stratification and was discharged in stable condition with re-initiation of her antihypertensive medication regimen. Painless vision loss represents a small proportion of emergency department (ED) visits but can portend not only an acute threat to vision, but also significant systemic pathology. Thromboembolic disease related to cardiovascular or stroke risk factors,A 69-year-old woman presented to the ED with painless vision loss in her left eye. She stated that over the course of minutes, she completely lost vision in that eye, with onset approximately six hours prior to evaluation. Initially, there was concern for posterior circulation arterial stroke, as the patient had elected to stop taking her antihypertensive medications one year prior. A computed tomography (CT) of the head showed no evidence of intracranial hemorrhage, and a magnetic resonance imaging (MRI) evaluation of the brain showed no evidence of acute stroke. The patient’s fundoscopic exam was limited by constricted pupils, and she was transferred to a tertiary care academic medical center for ophthalmologic evaluation.On arrival to the tertiary care center, the ED team performed a POCUS, linear probe, 12 MegaHertz (MHz) of the patient’s eye to evaluate for retinal detachment. No evidence of retinal detachment, vitreous detachment, or massive vitreous hemorrhage was found. However, the study demonstrated a widened and irregular optic nerve sheath, which measured over the normal limit of five millimeters (mm).The patient was admitted to the neurology service for monitoring of permissive hypertension initially, and then resumption of an antihypertensive medication regimen. The timing of symptom onset was a contraindication for thrombolytic treatment. Rapid stroke-risk stratification demonstrated no echocardiographic evidence of cardiac source of her embolus, no right-to-left cardiac shunt, and no significant carotid stenosis on CT angiography. She was re-initiated on her outpatient antihypertensive medications, and on two-month follow-up she had no significant return of vision in her left eye and had no evidence of further embolic events.POCUS is of increasing utility to the emergency physician. The optic globe, as a superficial fluid-filled object, lends itself to reliable ultrasound imaging with potential diagnostic utility in a number of acute ophthalmologic emergencies.3In another study, 59% of patients with CRAO were found to have an “intra-arterial spot sign,” which was associated with lack of response to thrombolytic therapy. This lack of response is thought to be primarily due to calcific components of likely carotid thromboembolic plaque, which is not amenable to thrombolysis.The finding of edematous optic nerve may provide another reproducible finding, though this has not been well described in the literature. A measurement pattern has been established to have a sensitivity of 88% and specificity of 93% for optic nerve sheath edema due to intracranial pressure of greater than 20 centimeters water based on an optic nerve sheath diameter of greater than 5mm.What do we already know about this clinical entity?These findings have been well documented in other imaging sources, particularly magnetic resonance imaging. Use of ultrasound has been reported and quantified but is not widely disseminated in the literature.What makes this presentation of disease reportable?The image gives a guide to practicing physicians as to appearance of the edematous optic nerve.What is the major learning point?Incorporating ultrasound in the evaluation and management of patients presenting with vision loss could potentially speed the diagnosis of central retinal artery occlusion.How might this improve emergency medicine practice?Emergency physicians reading this article would be able to incorporate ultrasound in evaluation and management of patients presenting with vision loss, potentially speeding the diagnosis of central retinal artery occlusion.Central retinal artery occlusion is a rare presentation to the ED; however, it represents significant pathology, with concerns for long-term disability (vision loss) and possible life-threatening associated conditions (stroke.) Use of point-of-care ultrasound represents an opportunity to make a rapid and accurate diagnosis of CRAO.


Pleuroblastoma (PPB) is a rare pediatric tumor which, in 30% of cases, is associated with cystic nephroma. It has been recently linked to the DICER1 mutation as part of a predisposition syndrome for various tumors. However, if DICER 1 anomalies have been reported in patients with Wilms tumor (WT), to date, no cases of PPB, WT, and DICER1 mutations have been reported in the same patient.We report the case of a 3-year-old patient, initially managed for metastatic WT. During his clinical course, the diagnosis of a PPB was made after detecting the DICER1 mutation and subsequent management was therefore modified.This case highlights that in case of simultaneous discovery of a renal tumor and a pulmonary lesion in a child, the DICER 1 mutations should be looked for as these could help adapt management and schedule the surgical procedures. Pleuropulmonary blastoma (PPB) is a rare pediatric tumor with around 500 cases reported . Its assA 3-year-old girl was admitted for fever and cough. She was diagnosed as having pleuresia and pleural drainage along with broad spectrum antibiotics was prescribed. Lack of improvement after a few days led to complete the work-up with a CT scan Fig. a, b. TheHistologic analysis confirmed the diagnosis of WT with an intermediate risk  which was classified as a stage IV. Lung surgery was scheduled several weeks later. During this period, using high-throughput sequencing of a panel of genes involved in endocrine tumor development, we identified a heterozygous pathogenic variant in exon 23 of the DICER1 gene (LRG_492). This variant c.4407_4410del, p.Ser1470Leufs*19, leading to loss of the RNase III active site, has been previously associated with pleuropulmonary blastoma [A left pleuro-pneumonectomy with intra-pericardic ligature of the vessels and without cardiac assistance was performed. The early postoperative course was uneventful. The pathologist confirmed the diagnosis of type 3 PPB with R0 resection. Postoperative chemotherapy consisted of eight courses (27 weeks) of vincristine, actinomycin, and doxorubicin. After 1-year follow-up, the patient remains asymptomatic and is considered to be in remission.The association of DICER 1 mutations and PPB is reported in approximatively 66% of recorded cases . MoreoveOur experience underlines two aspects. First, in case of associated renal and pulmonary tumors, preoperative chemotherapy could help determine the nature of the renal lesion. In our case, the WT could be easily removed without preoperative treatment because of its anatomic localization, but we considered it as metastatic disease because of the lung tumor. The diagnosis of DICER1 mutation after the nephrectomy brought us to consider the possibility of two synchronous lesions. It would have been helpful to determine this earlier as it could have modified the surgical strategy. We could also have combined both procedures, but it seemed too difficult for the patient to cope with such an operation.Secondly, DICER 1 mutations and WT have been reported in the genotype of tumor cells as in patients with PPB . HoweverSurgical decisions were linked to the evolution of the tumor after chemotherapy. The acute lung symptoms could have been due to necrosis of the tumor inducing massive pleural effusion or by rapid tumor growth. The emergency surgical procedure was meant more to improve the child’s breathing than to actually cure her, because we thought that local invasion would mean the tumor was not resectable. Surprisingly, complete excision of the PPB was possible despite what the preoperative workup suggested.Finally, this case should help inform parents with PPB and DICER1 mutations that WT can also occur in these circumstances and that ultrasound scan should be performed more easily in these patients to avoid late diagnosis.This yet unreported association highlights that in case of simultaneous discovery of a renal tumor and a pulmonary lesion in a child, DICER 1 mutation research could help adapt management and schedule surgery.


Intrahepatic subcapsular hematoma (ISH) is an extremely rare, life-threatening complication after laparoscopic cholecystectomy (LC). Only few cases have been reported. Herein, we reported a rare giant ISH after LC and summarized all of the reported cases.A 32-year old woman with recurrent acute cholecystitis for one year, underwent elective LC without intra-operative complications and was discharged 2 days after operation. On the next day after discharge, she developed severe right upper abdominal pain and was sent to our emergency department. The computed tomography scan showed a 10.9 × 12.5 × 6.6 cm ISH in the right liver without free fluid and the hemoglobin dropped to 86 g/l from 127 g/l. Postoperative hemorrhagic shock and a giant ISH after LC were diagnosed. After fluid resuscitation, the hemodynamic was still unstable and the hemoglobin kept dropping. An emergency laparoscopic exploration was performed and the ISH was confirmed, however no active bleeding point was found. A drainage tube was placed under liver for early warning of rupture. Patient was discharged home 10 days after readmission.Giant ISH is an extremely rare, life-threatening complication after LC. This case showed that the need to consider this rare complication in patients suffering abdominal pain after LC and timely and correct diagnosis and treatment were crucial to saving the lives of the patients. Laparoscopic cholecystectomy (LC) substantially reduced the incidence of surgical complications in experienced hands . At presThe patient was a 32-year old woman with a one-year history episodes of cholecystitis treated conservatively. She did not have any other disease history. After an abdominal magnetic resonance imaging that confirmed multiple gallbladder stones Fig. a, an eleTotally, 13 papers, including 16 cases of ISH after LC were reported from 1994 to 2015 , percutaneous drainage under CT or B ultrasound guidance, selective embolization of the bleeding vessel, laparoscopic exploration and laparotomy. Eighteen percent of patients had stable condition without fever and underwent conservative treatments. The only case of angioembolization was complicate by infection and required percutaneous drainage. For the patients with stable condition, fever and serious compression of inferior venal cava (IVC) always were indications for percutaneous drainage under CT or B ultrasound guidance. In these 17 cases, 29.4% of the patients underwent percutaneous drainage. For the patients with hemodynamic instability, emergent reoperation was adopted. Totally, nine cases underwent reoperation, including two case of laparoscopic operation and seven cases of laparotomy. For our case, we only performed laparoscopic exploration and did not perform evacuation or drainage of the hematoma, since the hemodynamic became stable after plenty fluid resuscitation and the hematoma did not expand, during the 3 h of intra-operative observation. For another case, laparoscopic exploration found small capsule laceration, and hemostasis was performed. In the seven cases of laparotomy, six patients underwent evacuation and drainage of hematoma, only one case underwent only laparotomy without evacuation or drainage.All patients survived. Most of patients stayed one to two weeks after readmission, however, the longest hospital stay was up to 31 days after reoperation.LC is a widely performed procedure for symptomatic gallbladder stone. Although the overall morbidity is less than 7%, it carries certain risks, the most common complications include infections, bile leak and postoperative bleeding . PostopeWhen the ISH is small, it is usually asymptomatic, therefore, it is very difficult to detect it at an early stage. However, when it keeps developing, it could be life-threatening, leading to hypovolaemic shock and even death. The treatment for ISH should be adopted according to the condition of the patients. When the ISH is small and the condition of patient is stable, conservative treatment is always preferred. Some other more aggressive treatments, including percutaneous B ultrasound -guided drainage, relaparotomy or relaparoscopy, evacuation or drainage of the hematoma, should be considered when the ISH is too big or the patient is hemodynamically unstable –11. In aDespite LC has been widely performed and the complications of LC is relatively low in the experienced hands, some serious complications can still occur. Giant ISH is an extremely rare, life-threatening complication after LC. The experience of how to treat this kind giant ISH remains largely limited. Herein, we presented a rare case and summarized all of the previously published cases which could substantially increase our experience to treat this rare condition. The most important key point for this giant ISH is to avoid sudden death after the rupture of hematoma, luckily, according to this literature review, only one case was ruptured and no mortality was reported.


Primary malignant peripheral nerve sheath tumour (MPNST) is an uncommon malignant tumour that arises from the peripheral nerves. Most of these tumours arise in the regions of the trunk, head and neck, or extremities and are rarely seen in the abdomen. In this report, we describe a case of MPNST of the greater omentum, which, to the best of our knowledge, is only the second case reported in the literature. MPNST is an uncommon tumour that can show local invasion and has a high risk of recurrence. We also discuss the utility of “omental vascular pedicle sign” to help establish the omental origin of intra-abdominal masses. The greater omentum is a mobile peritoneal drape in relation to the greater curvature of the stomach and is sometimes referred to as the policeman of the abdomen. Numerous primary and secondary tumours can arise from the greater omentum. Among them, malignant peripheral nerve sheath tumour (MPNST) is an uncommon entity. Identifying the site of origin of an intra-abdominal mass is critical in further management and the “omental vascular pedicle sign” helps in determining the omental origin of an intra-abdominal mass.part solid and part cystic lesion with internal vascularity in the subhepatic region, with loss of planes with the anterior abdominal wall muscles. The mass was seen separately from the gallbladder, which showed multiple intraluminal calculi with normal wall thickness. No other significant finding was seen in the abdomen. This was followed by a contrast-enhanced CT scan of the abdomen that revealed a large (approximately 7 × 6 cm sized) solid cystic mass lesion in the right subhepatic region showing intensely enhancing solid areas with peripheral cystic non-enhancing areas , metastases, leiomyomas, leiomyosarcomas, etc. with respect to their localization to the omentum or the mesentery, especially when it shows invasion of the surrounding structures. Localization of the tumour to one of these sites not only helps in narrowing down the radiological differential diagnosis but also in proper preoperative surgical planning, which is of paramount importance in resection of potentially resectable masses.4 There have been isolated reports of primary and secondary MPNST arising from the omentum.6 Miguchi et al4 reported the first case of a primary MPNST arising from the greater omentum. They described it in a 71-year-old male patient who was otherwise asymptomatic except for pain in the lower abdomen. His CT scan showed a large, well-defined, lobulated mass with central low density areas and peripheral enhancing components, which was managed with wide local excision. Rai Bansal et al5 have reported a case of primary malignant nerve sheath tumour arising from the retroperitoneum and displacing the surrounding structures, and showing local invasion of the adjacent large bowel. CT imaging in their case showed a large, predominantly cystic mass lesion with peripheral heterogeneously enhancing solid areas and small foci of calcification. Similarly, CT imaging in our case showed an invasive oval mass lesion centred in the omentum with intensely enhancing solid areas and peripheral non-enhancing cystic areas with dilated omental vein draining it . In rare cases, secondary metastatic MPNSTs of the omentum can present as omental caking.6MPNSTs are uncommon soft tissue tumours that arise from the peripheral nerves. Most of these tumours arise in the regions of the trunk, head and neck, or extremities and are very rarely seen in the abdomen.7 These tumours have been documented to be locally aggressive and recurrent. The recurrence rate after treatment has been noted to vary from 25% to 50%.8 There are a few case reports of malignant nerve sheath tumour of the peritoneum involving the anterior abdominal wall locally.8 Of these, in the report by Khorgami et al,7 a patient, with a known case of NF1, had local recurrence. There is no standard protocol for management of this condition. Tumour size, total excision, lack of S100 expressivity, absence of local invasion and neurofibromatosis have been seen to portend a better prognosis.7MPNSTs are often seen (up to 50%) in association with neurofibromatosis Type 1 (NF1).5 MPNST has a high rate of recurrence, as was noted in our case, and adequate initial treatment and close follow up are required to improve survival.5Radical surgical resection with post-operative radiotherapy despite having negative surgical margins is currently the recommended form of treatment for MPNST.MPNST of the greater omentum is an uncommon tumour that can show local invasion and has a high risk of recurrence and distant metastases. The knowledge of this uncommon entity is important for radiologists as well as clinicians for its early diagnosis and adequate management. The omental vascular pedicle sign is an important sign that can be demonstrated on a CT scan to help establish the omental origin of intra-abdominal masses, which is important in narrowing down the radiological differential diagnosis and preoperative planning of potentially resectable tumours.Omental vascular pedicle sign helps in localizing an omental mass lesion.Differential diagnoses of primary and secondary neoplasms  of omentum and other generalised diseases affecting the omentum are listed.Peripheral nerve sheath tumour of the omentum should be included in the differential diagnosis of a suspected solitary omental mass.Malignant nerve sheath tumour of omentum has high rate of recurrence; therefore, diagnosis is important for resectable masses. Adequate management and follow up  is advised.Informed consent has been obtained and is held on record.


Membranous nephropathy associated with malignant neoplasm may remit completely with treatment of the underlying disease. In such cases, recurrence is very rare. However, after a recurrence, attention should be paid to the possible recurrence of the underlying disease. On the other hand, few reports have described MN recurrence/relapse associated with the recurrence of malignant neoplasm. We report herein the case of a patient who was found to have gastric cancer at the onset of nephrotic syndrome associated with MN, which remitted completely after surgery and chemotherapy, and who experienced recurrence of MN 7 years postoperatively and was diagnosed with peritoneal dissemination 4 years later.218F‐fluoro‐2‐deoxy‐D‐glucose positron emission tomography showed findings suggestive of widespread peritoneal dissemination in the abdominal cavity. Esophago gastro duodenoscopy, total colon endoscopy, and contrast‐enhanced neck and chest computed tomography yielded no finding of another malignant neoplasm. Staging laparoscopy in July revealed widespread multiple peritoneal nodules  or paraneoplastic glomerulopathy. PNS has been defined as followsMembranous nephropathy is a rare disease  compared with malignancy .In most cases of MN considered to represent PNS, the corresponding neoplasm would develop within ±12 months.Primary MN has been said to be predominantly associated with deposition of immunoglobulin (Ig)G4, and secondary MN, with deposition of IgG1 and IgG2.Gastric cancer reportedly recurred postoperatively within 5 years in 66%‐77% of patients with early cancer and in 91.9% of patients with advanced cancer.We have reported a rare but theoretically possible case of MN that went into complete remission after treatment for malignant neoplasm, but recurred along with the malignant neoplasm. This case shall serve as a reference for future discussions in onco‐nephrology.The authors have no conflict of interest to declare with regard to this report.Consent: While ensuring the anonymity of the patient, we obtained the patient's written consent to report his case.NK: treated the renal lesions throughout their entire course and drafted the manuscript. NW and YA: treated the peritoneal dissemination. TK and HO: performed pathological examinations. KH: participated in the treatment of renal lesions. All authors contributed to the manuscript preparation and revisions and consented to be held accountable for the present report.


Chest wall or pleural-based tumours represent a heterogeneous group of lesions that are infrequent in children and infants; however, a large proportion of these lesions are malignant in nature. Categorising them on the basis of primary versus secondary, site of origin  and tissue composition may assist in narrowing the differential diagnosis. We present a case of a 7-year-old boy with a progressive history of dyspnoea. The initial chest radiograph (CXR) demonstrated complete opacification of the left hemithorax with no air bronchograms. This was associated with the cut-off of the left main bronchus and mediastinal shift to the right. The post-contrast computed tomography (CT) of the chest showed multiple left-sided enhancing pleural-based masses with collapse of the left lung. These lesions were locally invasive as demonstrated by the intra and extra-thoracic extension. There were no associated erosions of the adjacent ribs or intra-tumoural calcifications. Based on the imaging findings, the diagnosis of extra-skeletal Ewing sarcoma (ES-EWS) of the chest wall was made with a differential diagnosis of rhabdomyosarcoma. A core biopsy was performed of the pleural-based mass, and histology with immunohistochemistry confirmed the diagnosis of a malignant small round blue cell tumour; subtype Ewing sarcoma family tumour (ESFT). The child was subsequently commenced on chemotherapy. The diagnosis of ES-EWS should be considered when a child or adolescent presents with an ill-defined, eccentric, chest wall mass in the absence of a lesion with a primary osseous origin. Imaging plays a key role in tumour staging, therapeutic planning and follow-up of patients. A chest radiograph (CXR) was performed which demonstrated complete opacification of the left hemithorax with no air bronchograms. This was associated with a cut-off of the left main bronchus and mediastinal shift to the right . An ultrBased on the imaging findings, the differential diagnosis was categorised into three main groups:Malignant soft tissue tumour ESFTMetastases Another differential consideration includes inflammatory myofibroblastic tumour (IMT) of the lung or pleura.Biopsy of the anterior pleural-based mass was performed for histopathological confirmation. On microscopy, the specimen was composed of a proliferation of small round blue cells arranged in solid sheets. The background stroma was myxoid to oedematous in areas. Large areas of haemorrhage were also noted. The individual neoplastic cells were intermediate in size. The cells demonstrated oval nuclei with irregular contours and finely dispersed chromatin. Pinpoint nuclei were also noted in other areas. The cells had moderate amounts of pale eosinophilic cytoplasm. Brisk mitotic activity was noted with atypical forms seen. No rosettes were identified and a fibrillary background was not identified. No rhabdoid cells were seen. Periodic Acid-Schiff (PAS) and Periodic Acid-Schiff with diastase (PAS-D) stains highlighted focal glycogen within the neoplastic cells. These features were consistent with a malignant small round blue cell tumour of the chest wall/pleural biopsy.The immunohistochemistry of the biopsy specimens was then performed to further subtype this neoplasm, which were consistent with ESFT.No patient identifiable information has been presented.6 The origin of EWS from structures other than bone is considered rare and is termed ES-EWS.1 In 1969, Tefft et al. initially described an extra-osseous, paravertebral soft tissue, small round cell tumour with morphological features similar to EWS.7 Angervall and Enzinger in 1975 later established the entity ES-EWS in their review series of 39 cases of extra-osseous examples of EWS.7J. Ewing first described EWS in 1921, and it is now believed to be the second-most common malignant bone tumour in children and adolescents after osteosarcoma.2 The lesion within the paravertebral region may either be intradural extramedullary or evident as an extradural mass.2 Although ES-EWS is primarily a soft tissue tumour, it may invade adjacent osseous structures.6 Some authors use the term Askin tumour for the specific localisation of ES-EWS within the chest wall.5The most common sites of occurrence of ES-EWS are the paravertebral region, lower extremities, chest wall, retroperitoneum, pelvis and hip, upper extremities, and head and neck region.2 The radiological evaluation of these patients is multimodal; however, the imaging features may be non-specific.2 The imaging findings described below are specific to ES-EWS of the chest wall in conjunction with our case presented. It may present as a solitary or multiple extra-pulmonary masses, demonstrating a pattern of eccentric growth along the chest wall.5 Other reported findings include pleural effusions, lymphadenopathy, lung and bone metastases.3 The expansion of this chest wall lesion may result in the secondary collapse of the lung, or it may directly invade the lung parenchyma.5 A paravertebral origin of this lesion may result in extension through the vertebral foramina and can cause secondary bony changes of the adjacent vertebral body.5Extra-skeletal Ewing sarcoma typically manifests in children and young adults, with 85% of the reported cases being between 20 months and 30 years of age.2 Doppler interrogation of the masses reveals associated vascular flow.2 Commonly, an associated pleural effusion may be demonstrated.2Ultrasound (US) offers the advantages of absence of ionising radiation, easy accessibility and a dynamic study that can be performed at the patient’s bedside, which can also guide needle-biopsy for the histological diagnosis. The US shows that these chest wall masses are often hypoechoic but can have anechoic regions if they are necrotic or haemorrhagic.2 Low-attenuation regions within the lesion may also be apparent and are representative of haemorrhagic or necrotic areas.2 Associated calcifications are rare, as evident in our case, and have been reported in 10% of the cases.2 Associated rib destruction has been reported in 25% – 63% of the cases.2The CT findings depict an ill-defined, large, unilateral, soft-tissue chest wall mass, with heterogeneous enhancement post contrast.8 The MR depicts an ill-defined, eccentric soft-tissue mass with intermediate signal on T1 weighted imaging (T1WI) and T2 weighted imaging (T2WI) demonstrating a high signal intensity lesion.5 Areas of haemorrhage or necrosis may be evident as regions of high signal intensity on T1WI and T2WI compared to the skeletal muscle.2 Gadolinium administration results in intense enhancement of the solid components of the tumours and is representative of its hyper vascular nature.5 These tumours are often locally invasive with direct invasion of the musculature of the chest wall, mediastinum and lung, with subsequent lung collapse, as evident in our case.2The magnetic resonance imaging (MRI) findings, although non-specific, offer the added advantage in better delineating the margins of the mass and assessing its relationship with the surrounding structures and tumour staging.2 The use of other nuclear studies such as Indium 111 (111In) penetetreotide and Technetium 99m (99mTc) setamibi (MIBI) scans also offer promise in the evaluation of treatment response after surgical resection and chemotherapy.2Fluorodeoxyglucose Positron Emission Tomography (FDG PET) demonstrates increased radionuclide uptake and may be used in the detection of the primary lesion, residual or recurrent tumour or metastatic lesions.1 This should be differentiated from other examples of small round blue cell tumours which may also occur in the chest wall such as neuroblastoma, embryonal rhadomysosarcoma and lymphoma.6 The diagnosis is based on the combination of both the clinical and imaging features, as well as the immunohistochemical staining and cytogenetic analysis.6 Definitive diagnosis is made with Fluorescence in-situ hybridisation (FISH) to detect the rearrangement of the 22q12 (EWSR1 gene).1Histologically, ESFT are small round blue cell tumours – a malignant group of neoplasms with a wide range of differentials.8 In the presence of a primary oncological diagnosis, metastatic lesions such as neuroblastoma, rhabdomyosarcoma and lymphoma/leukaemia need to be excluded.9 Metastatic lesions to the chest wall typically present on imaging as lytic bone lesions with associated cortical disruption and periostitis.9 Neuroblastoma may present as a posterior mediastinal mass with direct invasion of the chest wall and may extend into the adjacent neural formamina.9 Lymphoma is often associated in those with a compromised immune system .5 The patient in this case report was not immune-compromised; so, the diagnosis of lymphoma was considered unlikely. The absence of constitutional symptoms in conjunction with the associated negative biochemical and bone marrow findings excludes leukaemia. The absence of a lesion with primary osseous involvement excludes EWS of the bone and osteosarcoma; in addition, osteosarcoma is typically associated with centrally dense calcifications.9 Rhabdomyosarcoma of the chest wall cannot reliably be differentiated on imaging from ES-EWS, as both demonstrate non-specific findings of an aggressive chest wall lesion.2 Bone involvement typically occurs in the late stage of the disease but is reported in ~20% of patients.9 Embryonal rhabdomyosarcoma is a subtype which is unique to the paediatric population.10 Malignant peripheral nerve sheath tumours arise within the nerve sheaths and may either occur sporadically or within plexiform neurofibromas in patients known with neurofibromatosis type 1 (NF1).9 The presence of a mass lesion that courses along the expected location of a peripheral nerve favours the diagnosis of a peripheral nerve sheath tumour and may be associated with underlying erosions of the adjacent bone.9 Pleuropulmonary blastomas typically affect infants and children .11 Type II and III pleuropulmonary blastomas have been reported in older patients, and Type I (purely cystic) pleuropulmonary blastomas exclusively occur in those younger than 3 years of age.12 Type III (purely solid) pleuropulmonary blastomas are typically well-circumscribed with heterogeneous enhancement, and although primarily intra-thoracic (arising from the lung or pleura), it may involve the chest wall.9 Type II (mixed cystic and solid) pleuropulmonary blastomas are less aggressive and rarely involve the chest wall.9Malignant paediatric chest wall tumours represent a diverse group of lesions; however, categorising them on the basis of primary versus secondary, site of origin  and tissue composition may assist in formulating the differential diagnosis.13 Calcifications occur more commonly in children compared to adults.14 Pleural IMT is exceedingly rare, with only a few published case reports, and may appear locally invasive with the infiltration of the adjacent chest wall.13Inflammatory myofibroblastic tumour of the lung usually appears as a solitary, well-marginated, peripheral mass on CXR with heterogeneous enhancement on CT.2 The primary method for local control is surgical; however, this is dependent on various factors based on the tumour staging.2 This would include factors such as patients’ age, tumour size, location and extent of involvement of adjacent vital structures; presence of encasement of adjacent neurovascular bundles and whether the primary lesion can be completely excised with acceptable margins.2As with all oncology cases, the mainstay of treatment is multidisciplinary. Biopsy is necessary to confirm the diagnosis after which neoadjuvent chemotherapy is initially used with the aim of decreasing the size of the primary lesion and eliminating micrometastases.2 Recurrence has also been reported in more than 50% of the cases and may be evident as local recurrence, mediastinal lymphadenopathy or metastatic lesions.2The thoraco-pulmonary location of ES-EWS is associated with a poorer prognosis in comparison to ES-EWS at other sites with the 2- and 6-year survival rates reported as 38% and 6%.Extra-skeletal Ewing sarcoma is regarded a rare entity that has not been commonly reported but is an important consideration, given the poor prognosis. This diagnosis should be considered when a child or adolescent presents with an ill-defined, eccentric, chest wall mass, in the absence of a lesion with a primary osseous origin. The differential diagnosis should also include rhabdomyosarcoma of the chest wall, as this cannot reliably be differentiated from ES-EWS on imaging. Imaging plays a key role in tumour staging, therapeutic planning and follow-up of these patients.


The purpose of this paper is to present an overview of the imaging features of the most prevalent benign bone tumors involving the spine. Benign tumors of the osseous spine account approximately for 1% of all primary skeletal tumors. Many lesions exhibit characteristic radiologic features. In addition to age and location of the lesion, radiographs are an essential step in the initial detection and characterization but are limited to complex anatomy and superposition. CT and MR imaging are often mandatory for further characterization, assessment of local extension and guiding biopsy. As most of benign tumors of the osseous spine are asymptomatic, their real incidence is unknown. The prevalence is estimated for less than 10% of all spinal tumors [Osteoid osteoma (OO) is characterized by the formation of a nidus of vascularized osteoid tissue surrounded by a halo of reactive sclerotic bone. The average size of the nidus is less than 1.5 cm. It commonly occurs in the second decade. OO accounts for 1% of all spinal tumors. The lumbar spine is most commonly affected 59%), followed by the cervical, thoracic, and sacral spine . Most sp%, followAlthough spontaneous resolution has been reported rarely, prompt treatment is preferred. Indeed, treatment delay in the growing child may result in a permanent structural scoliosis . Alterna◽ Percutaneous curettage of the nidus under CT guidance.◽ Percutaneous CT-guided thermo-coagulation. This technique achieves the same clinical outcome as the operative excision with significantly lower costs.◽ Percutaneous CT-guided interstitial laser thermotherapy .Regrowth of OO is generally due to incomplete removal rather than multiple nidus. CT is the preferred technique for follow-up after treatment.Osteoblastoma is a benign bone forming tumour which is histologically similar to OO. In comparison to osteoid osteoma, osteoblastomas tend to be larger in size >2 cm), often present with cortical breakthrough and a soft tissue mass, and may rarely undergo malignant transformation 14. Spina cm, ofteSurrounding bone marrow edema in adjacent vertebrae, paraspinal soft tissues and ribs enhances after contrast administration, often resulting in overestimation of size of lesion. Adjacent bone remodeling at the level of the articular facet may present as facet hypertrophy . TechnetResection is the best treatment option for osteoblastoma, but local recurrence, especially after incomplete resection, has been reported in approximately 50%. Some studies have found beneficial effects of radiotherapy following subtotal excision .Osteochondroma (OC) is the most common benign tumor of the bone. Osteochondroma of the spine is rare and comprises only 1.3–4.1% of all osteochondromas . SolitarSpinal osteochondromas may be part of Hereditary Multiple Exostosis Syndrome. The clinical manifestations of osteochondroma vary widely, ranging from back pain to neurological deficits mimicking myelopathy or radiculopathy 22. BecauWhen the tumor causes pain or neurological complications due to compression, or if the diagnosis is indeterminate, osteochondroma should be excised at its base .Vertebral hemangiomas are the most common benign tumors in the spine, accounting for 2% of skeletal benign tumors. They are considered as dysembryogenetic disturbances, affecting the proper differentiation of blood vessels . There iMost vertebral hemangiomas are asymptomatic. The patients present with pain, spinal cord or nerve root compression in case of aggressive hemangiomas.Histopathologically, hemangiomas are predominantly composed of vascular lined spaces and nonvascular components that may include adipose tissue, smooth muscle, fibrous tissue, bone, hemosiderin and thrombus .polka dot on axial images or corduroy appearance on coronal or sagittal reformatted images respectively. On MRI, fatty hemangiomas represent inactive forms. Low T1 signal intensity indicates a more active lesion with the potential to compress the spinal cord. Haemangiomas show avid enhancement. Aggressive hemangiomas involve the entire vertebral body with extension to the neural arch, cortical expansion, irregular honeycombing, soft tissue mass and contrast enhancement  are quite rare and comprise less than 5% of the primary bone tumors of the spine . GCTs arPatients typically present with pain, neurologic deficits (72%) such as radicular pain and motor weakness from nerve root or spinal cord compression .Histopathologically, GCTs consists of abundant osteoclastic giant cells with intermixed spindle cell stroma. Erythrocyte lakes  and xanthomatous changes within the focal collections of histiocytes may be encountered .On radiographs, an expansile osteolytic lesion is typically seen. The lesion often causes collapse of the vertebral body, ranging from mild collapse to a complete vertebra plana . In the On CT, GCT has a heterogeneous density with foci of low attenuation (in keeping with haemorrhage or necrosis) with no evidence of mineralized matrix Figure , b. On MComplete surgical resection of SGCT is the standard treatment . AdjuvanAneurysmal bone cyst (ABC) is an expansive and hemorrhagic primary tumor, usually showing a characteristic translocation placed on 17p13 . It can Clinically, ABC might even be asymptomatic but usually presents with pain and swelling. Despite being benign, they can be locally expansile and destructive, resulting in pathological vertebral fractures and neurological complications. Histologically, ABC is characterized by blood filled cavities without any endothelial lining or smooth muscle .Radiographs and CT findings depend on the stage of the ABC. Eccentric bone rarefaction area is seen during the osteolytic phase Figure . Active Differential diagnosis of ABCs includes giant cell tumor, chondroblastoma, chondromyxoid fibroma, simple bone cyst, osteoblastoma, and plasmocytoma .The treatment options of ABC are curettage with or without bone grafting, complete excision, arterial embolization and intralesional drug injection  .Fibrous dysplasia (FD) is a benign medullary fibro-osseous lesion, which may be monostotic or polyostotic. Monostotic FD is more common than polyostotic FD. However, polyostotic disease occurs in the spine more frequently . FD affeThe treatment of monostotic fibrous dysplasia of the spine varies widely, from wait-and-see policy to biopsy and surgical resection.Langerhans’ cell histiocytosis (LCH) is a rare disorder characterized by proliferation of histiocytes or macrophages . LCH canThe most common presenting symptom is dull pain. Neurologic complications are rare and usually mild .Histopathologically, eosinophilic granulomas display a characteristic “tennis racquet”-shaped “Birbeck granule” in the cytoplasm .Radiographs show an anterior wedging with a characteristic “vertebra plana” appearance Figure . EpiduraThe differential diagnosis include other destructive lesion including Ewing sarcoma, lymphoma, leukemia, and metastasis. Trauma should be considered as well in case of vertebra plana .Therapeutic options range from clinical/radiological monitoring to cervical or lumbar orthoses, CT-guided intralesional infiltration of corticosteroids, surgical curettage and bone grafting, or even radical resection of the affected bone .Enostoses are relatively common lesions with an incidence of up to 14% in cadaveric studies. These lesions are found in all age groups with no significant gender predilection . HistoloThe combination of age, location, and imaging appearance is often needed for a correct diagnosis of benign spinal tumors. Careful analysis of semiological signs may allow precise characterization of most benign bone tumors of the spine and helps in differentation from malignant primary spinal tumors, metastases or other non tumoral lesions.


Pyogenic Granuloma (PG), also known as lobular capillary hemangioma, is usually seen as a polypoid red lesion found on the skin or the mucosal surface of the oral cavity. PG of the gastrointestinal tract is rare, in particular involving the esophagus, only 14 cases have been reported in the English literature. We present an 80-year-old male who underwent endoscopy for evaluation of dysphagia and was found to have a single, red, bilobed 10 mm polyp with adherent white exudate approximately 19 cm from the incisors. Endoscopic ultrasound was performed with a 20 mHz miniprobe which showed the lesion contained to the mucosal layer with no muscularis propria invasion. A decision was made to perform endoscopic mucosal resection (EMR). A mixture of saline and methylene blue was injected into the submucosal plane to raise the lesion with subsequent successful mucosal hot snare resection. The resection defect was then approximated and closed with a hemostatic clip to prevent bleeding. Pathology of the specimen revealed small capillary vessels growing in a lobular architecture with an edematous stroma and a florid inflammatory infiltrate representing a pyogenic granuloma. EMR allows for an en bloc resection of mucosal lesions with tumor-free margins, thereby providing both diagnostic and prognostic information. Comparing EMR with the novel technique of endoscopic submucosal dissection (ESD), the incidence of bleeding and perforation is much lower; making EMR the best and safest resection option for this rare hemangioma. In this case, we demonstrate that EMR is a safe technique in removing a pyogenic granuloma in the esophagus. Pyogenic Granuloma (PG) is a polypoid form of a hemangioma that frequently occurs on the skin or in the oral cavity. It is a benign tumor with frequent recurrence, with no cases of malignant transformation having been reported . PG of tAn 80-year-old male with a past medical history of hypertension and diabetes mellitus type II underwent endoscopy for evaluation of dysphagia and was found to have a single, red, bilobed 10 mm polyp with white exudate in the proximal esophagus measuring 19 cm from the incisors . EndoscoPG or lobular capillary hemangioma is a hemorrhagic, benign, polypoid neoplasm which was first described in 1897, at that time it was called botryomycosis hominis –5. The lMost patients who are found to have PG are asymptomatic. However, some symptoms associated with PG are upper gastrointestinal bleeding, epigastric discomfort, chest discomfort, dysphagia, and/or heartburn.Staphylococci, Helicobacter pylori, Botryomycosis, Campylobacter enteritis, and Candida which may affect the morphology of the lesion [Histological characteristics are proliferation and lobular arrangement of multiple capillaries, inflamed, and edematous stroma, and endothelial cell swelling , 2. The e lesion , 10, 11.e lesion . In addiWith the advent of new technology and rapid progression of minimally invasive techniques, endoscopy is becoming integral in providing both diagnostic and therapeutic interventions with virtually no morbidity and mortality . EndoscoPyogenic granuloma or lobular capillary hemangioma is a polypoid form of a hemangioma that is usually found as an incidental lesion; however, patients can present with symptoms of dysphagia, bleeding, or pain. Therefore, when discovered on endoscopy, these lesions should be resected to prevent possible complications. EMR provides a lift from the muscularis propria layer to ensure an adequate cushion which minimized the perforation risk, thus entailing a safer resection technique than a polypectomy. We illustrate that EMR, if done appropriately, is the most safe and best treatment modality for such high-risk bleeding lesions.


Monostotic fibrous dysplasia (MFD) involving the spine is rare, and the treatment options are controversial. Surgery is needed when patients suffer from persistent pain, spinal cord compression/injury, and vertebral collapse/instability. Treatment methods include biopsy/observation, corpectomy with instrumented fusion, posterior fusion, vertebroplasty (VP), curettage and bone graft, and complete removal of the vertebra with a combined anterior and posterior fusion procedure.The patient was a 56-year-old woman with a 2-year history of neck pain. No obvious abnormalities were detected on neurological or physical examination, and laboratory findings were all within normal limits. An imaging examination suggested a C7 vertebral bone tumor. The patient refused to continue conservative observation treatment and requested surgery. Open VP of the C7 vertebral body was carried out, and her postoperative neck pain was completely relieved. The postoperative pathological results supported the diagnosis of fibrous dysplasia, and the patient was ultimately diagnosed with MFD. At the 12-month follow-up visit, the patient reported no clinical symptoms, and no signs of tumor recurrence were detected.VP can relieve pain while stabilizing the spine. Thus, the surgical treatment of MFD vertebral lesions by VP is a valuable option. Fibrous dysplasia (FD) was first reported by Lichtenstein in 1938 , represeA 56-year-old female patient was admitted to our hospital with a 2-year history of neck pain. No obvious abnormalities were detected on neurological or physical examination, and laboratory findings were all within normal limits. Computed tomography (CT) Fig. a, b demoFD is a non-hereditary disease; one possible mechanism is the multi-synthetase-activating mutation of the GNAS1 gene on chromosome 20q13.2-13.3 in somatic cells during embryonic development , 6. BecaOur literature review revealed 17 cases of surgically treated MFD involving cervical vertebrae, and the characteristics of these cases (including the one case presented in this study) are summarized in Table Our literature review revealed only 7 patients (including the 1 patient reported in this study) who underwent VP for the treatment of spinal fibrous dysplasia, as shown in Table The injection of bone cement increases the strength of the vertebral body and effectively improves the stability of the spinal system . The cheThere are several challenges in the application of VP for MFD: (1) It is difficult to accurately diagnose the disease preoperatively because there is a lack of characteristic imaging manifestations, and the FD diagnosis depends on pathology results; in addition, CT-guided percutaneous biopsy for the evaluation of spinal lesions has a reported diagnostic accuracy of approximately 90% . HoweverVP has been widely used for the treatment of bone tumors, but its uses for the treatment of spinal FD is relatively rare. To the best of our knowledge, this report represents the second case in which VP is used for the treatment of spinal MFD. MFD is a benign tumor with slow progression. As VP can relieve pain while stabilizing the spine, the surgical treatment of MFD vertebral lesions by VP is a valuable option.


With current life expectancies exceeding 78 years on average, to be confronted with the discovery of a rare cancer often found in advanced stages is a startling devastation. Angiosarcoma of the intestine is a rare and aggressive tumor that is not often considered in the differential diagnosis of intestinal obstruction. Once found and accurately diagnosed, it is a bewildering race against time as its median survival time is 150 days from diagnosis. This case report details a rare small intestinal angiosarcoma with its host surpassing current epidemiological standards of survival time despite only being eligible for chemotherapy. Due to its rarity, non-specific presenting symptoms, difficulty in diagnosing, aggressive sequelae, and high mortality rate, intestinal angiosarcomas define a perfect storm of a medical disaster. This case report details a case of a stage IV angiosarcoma that occurred in a rare and unusual location of the small intestine with an unexpected outcome of the patient living four times the average lifespan from diagnosis despite being treated with chemotherapy alone.A 76-year-old male patient presented with complaints of epigastric abdominal pain with nausea, fatigue, and unintentional weight loss of 12 pounds over one week with normal bowel movements. His family history is significant for a sister with gastric cancer. An initial computed tomography (CT) scan without contrast displayed a circumferential, mass-like thickening at the distal transverse duodenum with abdominal mesenteric and retroperitoneal lymphadenopathy. A subsequent esophagogastroduodenoscopy demonstrated an infiltrative, fungating, circumferential, 2 cm mass in the third part of the duodenum that caused a partial obstruction where the scope could not traverse  was performed. The malignant cells were positive for vimentin, CD31 , environmental toxins, and genetics . It affeOnce appropriately diagnosed and staged, many angiosarcomas are treated with complete surgical resection with wide margins for localized tumors or as a palliative measure when metastatic. In cases where surgery was combined with chemotherapy, patients have a marginally longer life span of 420 days versus 96.5 days of extended survival with just surgery alone . CurrentA stage IV angiosarcoma partially obstructing the lumen of the third part of the duodenum defied norms by being managed by chemotherapy alone, when the current standard involves surgery. This case delved into an extensive review of angiosarcomas, while shedding light on an alternative treatment plan.


Introduction: Extragonadal locations of teratomas are uncommonly reported in the literature. Teratomas are neoplasms usually found in the gonadal organs: ovaries and testis. The majority of teratomas are found in the pediatric age group. Furthermore, teratomas originating in the liver are exceedingly rare with only 11 case reports in adult populations.Presentation of case: We present a case of a 65 year-old female who presented to hospital with sudden onset abdominal pain from a centrally located ruptured hepatic teratoma on CT scan. The patient underwent urgent surgery. The diagnosis of cystic mature teratoma was confirmed on histopathology. Patient was discharged on post-operative day 5. At 12 week follow-up, no post-operative complications were identified.Discussion: Hepatic teratomas are a rarely encountered neoplasm, especially in the adult population. Our case report is unique, as it represents the only clinical presentation of mass rupture in an adult liver teratoma. CT scan identified a well circumscribed mass containing adipose tissue, fluid, and calcifications characteristic of teratoma. Complete surgical resection is mainstay treatment. A definitive diagnosis of a mature teratoma is confirmed by histopathological findings.Conclusion: Presented is a rare case of ruptured hepatic teratoma in an adult who underwent surgical resection. Etymologically, the word teratoma is derived from the Greek word “teratos” which means monster . By defi2A 65-year-old Punjabi-speaking female with treated hypocholesteremia presented to the emergency department with sudden onset right upper quadrant pain. The patient had not had any previous surgeries, denied any smoking, alcohol or recreational drug use, and did not remark any medical conditions in her past family history. Of note, on her immigration examinations a decade early, she was told of a small abnormality on her liver. No management or follow-up was recommended at that time. On physical exam, she had some fullness and mild abdominal tenderness to right upper quadrant with no diffuse peritoneal signs.Initial contrast enhanced CT imaging scan demonstrated a large mass in the right upper quadrant measuring approximately 15 × 12 x 11 cm. A conspicuous fat fluid level is present, as well as partial rim calcification . There wMultisequence contrast-enhanced MRI imaging again demonstrated the presence of a fat fluid level , and theBased on a tentative diagnosis of teratoma and evidence of acute rupture of the mass, surgical intervention was recommended after patient was treated with fluid resuscitation and broad-spectrum antibiotics. A preoperative anesthetic consult was obtained and she was brought for surgery with a hepatobiliary surgeon. The patient underwent a laparotomy and partial hepatectomy. Intraoperatively, the tumor was found to be unresectable with R0 intent. The tumor demonstrated involvement the bifurcation of the porta hepatis and the retroperitoneum, including the retro-hepatic inferior vena cava. Based on the fact that the tumor previously ruptured, the anterior wall of the tumor was resected removing liver segments 5, 8 and a portion of 4, thus debulking the tumor by removing 95% of the tumor volume. The small amount of remaining tumor remained on the left and right biliary pedicles. Intraoperatively, the tumor contained thick sebaceous liquid and debris. The patient experienced no intraoperative complications with an estimated blood loss of 100 cc.Gross pathological examination revealed a firm rubbery textured cyst wall (13 × 9 x 0.8 cm) and cyst contents . MultiplMicroscopic exam of the cyst wall demonstrated extensive areas of hyalinization with areas of dystrophic calcification . CysticaThere were no significant acute post-operative issues and patient was discharge home safely on post-operative day 5. There were no postoperative complications at 6 weeks during follow-up visit. A follow-up CT scan was obtained which showed status post partial hepatectomy with thickened tissue along the bile duct at the porta hepatis, consistent with residual teratoma cyst wall.3Hepatic teratomas are a rarely encounter tumor with less than 30 case reports can be found in the literature and the majority in pediatric populations . Only 11Primordial germ cells follow a midline path and descend into the pelvis as ovarian and testicular cells, which explain their more common midline and paramedian locations. It is hypothesized, during the organogenesis period, arrest of these germ cells in migration along this path can lead to teratoma formation in extragonadal locations ,5. HepatA CT scan showing a well circumscribed mass containing adipose tissue, fluid and calcifications is characteristic of the radiologic findings of teratomas ,11,12. AA definitive diagnosis of a mature teratoma is confirmed by histopathological examination . Microsc4In summary, we present an exceedingly rare clinical presentation of an acute hepatic teratoma rupture in an adult patient who underwent near complete 95% surgical resection. Clinical work-up includes a CT scan, with confirmation of diagnosis of hepatic teratoma on histopathology. Resection remains the mainstay of treatment. This case adds to the limited literature of the patient presentation, clinical work-up, and management liver teratomas.None.None.Not applicable for case reports as per journal policy.Written informed consent was obtained from the patient for publication of this case report and the accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Jonathan Ramkumar: drafting of the article, critical revision of the article for important intellectual and clinical content.Andrew Best: image creation, critical revision of the article for important intellectual and clinical content.Ananta Gurung: image creation, critical revision of the article for important intellectual and clinical content.Anne-Marie Dufresne: critical revision of the article for important intellectual and clinical content.Elena Vikis: critical revision of the article for important intellectual and clinical content.George Melich: critical revision of the article for important intellectual and clinical content.Shawn MacKenzie: drafting of the article, critical revision of the article for important intellectual and clinical content, final approval of the version to be submitted.Not applicable.Shawn MacKenzie.Not commissioned, externally peer reviewed.