Pituitary carcinomas are rare diseases defined as pituitary tumors with metastases. In this report, we describe a case of malignant prolactinoma with liver metastases masquerading as metastatic gastrointestinal stromal tumor (GIST). A 54–years–old woman received dopamine agonists for macroprolactinoma for 2 years, followed by transsphenoidal surgery due to a poor response to medical therapy. Despite the continuation of dopamine agonist after surgery, serum prolactin level progressively increased to above 8,000 ng/ml in 5 years. There was no evidence of disease recurrence on sella magnetic resonance imaging (MRI). She stopped medical therapy. Meanwhile, she was diagnosed with GIST accompanied by liver and peritoneal metastases. After a 2–months treatment with imatinib, she suddenly presented with headache and visual impairment. Sella MRI showed a 3.3–cm sized pituitary mass, and serum prolactin levels were still high. For the recurred mass, she underwent a second surgery followed by radiation therapy. During the imatinib treatment for GIST, main mass and peritoneal metastases were dramatically decreased, but liver metastases were markedly aggravated. Liver masses were eventually confirmed as metastases from prolactin-producing pituitary carcinoma and not from GIST by percutaneous biopsy. Unfortunately, she died 6 months after the second surgery due to acute renal failure and sepsis. This case suggests that highly sustained serum prolactin levels during the dopamine agonist may indicate prolactin-producing pituitary carcinomas with hidden metastases. Malignant prolactinomas are exceedingly rare diseases that are defined as prolactin (PRL)-producing pituitary tumors with cerebrospinal, meningeal, or distant metastasis . The incHere, we present an unusual case of malignant prolactinoma with liver metastases that were mistaken for metastatic lesions of a concurrently occurring gastrointestinal tumor (GIST).In 2011, a 54–years–old woman was admitted to the Department of Neurosurgery, at Chonnam National University Hwasun Hospital of South Korea. She presented with acute dizziness and visual disturbance. In 2003, the patient was diagnosed with a 1.3 cm intrasellar mass on brain computed tomography (CT) when she visited the emergency room because of headache, nausea, and vomiting. Further evaluation was not performed at that time due to loss of follow-up. Sella magnetic resonance imaging (MRI) revealed an ~1.8–cm sized pituitary mass with right cavernous sinus invasion. Serum PRL level was 958 ng/ml (reference range 0–25 ng/ml). DA therapy with bromocriptine was initiated for the treatment of macroprolactinoma. Since serum PRL levels continued to increase despite the escalation of bromocriptine doses, she underwent transsphenoidal surgery 2 years after. Prednisolone and levothyroxine was administered for postoperative panhypopituitarism, and DA was changed to cabergoline after surgery .A histopathological examination showed strong positive immunohistochemical staining for PRL. The Ki-67 index was increased (6%), mitotic index was not increased (2 per 10 high-power fields [HPF] at x400 magnification), and p53 positivity was negative (4 strongly positive nuclei/10 HPF at x400 magnification).The serum PRL level did not normalize after surgery; however, it reduced to <200 ng/ml in 2 years with cabergoline. Subsequently, in 2016, the PRL level abruptly increased to above 900 ng/ml despite the increase in cabergoline dose to 2 mg per week. A MRI scan revealed no recurrence of the pituitary mass. Considering that she was a postmenopausal woman, cabergoline therapy was temporarily suspended for a year, but was resumed because serum PRL levels reached 8,000 ng/ml. However, because her PRL levels did not decrease despite administration of cabergoline up to 3 mg per week for 6 months, cabergoline was discontinued.During the drug holiday, in 2018, she was newly diagnosed with GIST with liver and peritoneal metastases through routine health examination. She started to receive imatinib (glivec®) for GIST. Two–months after the imatinib treatment, she suddenly presented with headache and acute visual impairment. Sella MRI showed a 3.3–cm sized recurrent pituitary mass . CabergoWith the continuation of imatinib for GIST, main mass and peritoneal metastases were dramatically decreased, whereas the size and number of liver metastases increased. Percutaneous biopsy showed that the liver masses were metastatic lesions from malignant prolactinoma and not from GIST . AlthougPituitary tumors are considered benign, but heterogeneous lesions in terms of clinical behavior. Approximately 30–45% of them are invasive, and up to 15% are regarded as clinically aggressive . PituitaInvasiveness is radiologically or histologically determined, for example, the invasion of the cavernous sinus and the sphenoidal sinus. Histologically, aggressiveness is defined as the presence of at least two out of three proliferative markers as following: (1) Ki-67 index >1%, (2) mitotic index >2 per 10 HPF at x400 magnification, and (3) positive p53 nuclear detection (10 strongly positive nuclei/10 HPF x400 magnification) . Since tde novo tumors seems to be the main mechanism for the development of pituitary carcinomas. Moreover, there is a certain possibility of metastatic spread after therapeutic interventions including surgery and/or radiotherapy such as blood-borne dissemination, drop metastasis , or central venous dissemination  (mination .68Ga-DOTATATE positron emission tomography (PET)-CT shows higher resolution than 18F-fluorodeoxyglucose (FDG) PET-CT or regional MRI, and currently, it plays a major role in the diagnosis of neuroendocrine tumors including pheochromocytoma. Considering the usefulness of 18F-FDG PET-CT as an indicator for tumor aggressiveness, the combination of 68Ga-DOTATATE PET-CT and 18F-FDG PET-CT may be beneficial in improving diagnostic accuracy of malignant prolactinomas.Prolactinomas comprise the most common pituitary tumor accounting for ~40% of all functional pituitary tumors . Among eFor diagnosing malignant prolactinoma, the most crucial point is clinical suspicion for the development of metastatic lesions. Due to a very low incidence of the disease, it can be easily overlooked. In our case, despite a significant rise in PRL levels initially to 900 ng/mL, her MRI did not show any significant change. The discrepancy between the MRI and significantly elevated PRL levels should have prompted an investigation for metastatic lesions. In our case, metastatic lesions were incidentally detected during the evaluation of GIST ~2.5 years after the onset of significant rise in PRL levels.The prognosis of malignant prolactinomas is extremely poor, with the median survival duration of 10 months after the detection of metastases . This me6-methylguanine which promotes DNA damage by base mismatch repair. TMZ led to the reduction of tumor volume up to 47% with disease control rate of 80% in previous studies  is another challenging therapy for non-medical management of PRL-producing carcinomas. PRRT basically uses the binding affinity of radiolabeled peptide analogs to peptide receptors in tumor cells . There afor PRRT . Recent for PRRT –24.In conclusion, we presented a case of malignant prolactinoma with liver metastases, which were clinically considered as metastases of GIST but pathologically confirmed as metastases of PRL-producing carcinoma. In this case, metastases were identified incidentally during the evaluation of imatinib's response to GIST. Our case suggests that highly sustained serum PRL levels despite DA therapy may not indicate DA-resistant prolactinomas but rather malignant prolactinomas. Therefore, clinical suspicion is crucial to detect hidden metastases.The studies involving human participants were reviewed and approved by the Institutional Review Board of Chonnam National University Hwasun Hospital. The patients/participants provided their written informed consent to participate in this study.AH and HK managed the case. AH drafted the manuscript. AH, JY, HK, and H-CK reviewed the manuscript. All the authors agreed with the final version of the manuscript.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Laparoscopic reduction techniques for internal supravesical hernia have not been discussed much in literature.A 90-year old woman was admitted for symptoms of intestinal obstruction. She was diagnosed with small bowel strangulation by CT scan and laparoscopy was performed. Laparoscopy revealed a mass medial to the medial inguinal fold with tightly incarcerated small bowel. The bowel could not be reduced by traction or external compression, and required incision of the hernia ring. The tight incarceration posed a risk of bowel injury and so we performed peritoneal incision in a similar manner to the TAPP approach for loosening and precise incision of the hernia ring. The bowel was successfully reduced and the hernia was repaired by partial sac resection.Surgical methods as well as reduction technique were reviewed from previous literature. Procedures with open laparotomy, laparoscopy and anterior approach have been described, but details of reduction were not seen in many of these reports. Various methods have been described for bowel reduction in other hernias, but none involving peritoneal incision. This is the first report describing bowel reduction via the peritoneal incision technique.Internal supravesical hernia may pose difficulty in bowel reduction, but the peritoneal incision technique allows safe incision of the hernia ring under laparoscopic situations. Cases of surgical repair by open laparotomy, laparoscopy, and anterior approach have been previously reported, but not many include details of how the incarcerated bowel was reduced. We hereby report a case of internal supravesical hernia with tight incarceration of small bowel successfully reduced via laparoscopy by the peritoneal incision technique. This work has been reported in line with the SCARE criteria .2A 90-year-old woman with no history of abdominal surgery presented with vomiting and lower abdominal pain. On examination, she was afebrile with normal vital signs. Her abdomen was significantly distended with tenderness near her umbilicus. Laboratory tests revealed an elevation in inflammatory markers (white blood cell count of 8300/μL and a C-reactive protein level of 6.49 mg/dL), but a normal lactate level. Contrast computed tomography showed a small bowel obstruction with a transitional zone in the right lower abdomen. A right indirect herniation could be seen, but there was no bowel involvement and thus was not considered to be the cause of obstruction . She wasA laparoscopy with a three-trocars technique  was performed . Upon in3Internal supravesical hernia is a rare condition, first reported by Ring in 1814 . It occuThe rarity of this condition can be seen from the fact that there are only 41 results in a Pubmed search with the MeSH term “internal supravesical hernia”. Of these results, 16 cases were found with operative details  [[5], 6. The medDue to the rarity of the condition, there is no golden standard for surgical procedure in internal supravesical hernia, but trends can be seen from our literature review. First of all, the hernia orifice was closed with sutures in 15 out of 17 cases (88%), which is thought to be important in preventing recurrence. We did not close the orifice, partly due to our ignorance of the condition and its trending surgical procedure, but also due to concerns of abscess formation. However, post-operational abscess formation has not been documented for internal supravesical hernia, so perhaps it should not have been worried for. Instead of closing the orifice, our strategy for preventing recurrence of bowel obstruction was to partially resect the sac. Sac resection was only done in 2 out of the 17 cases, and is thought to be unnecessary. In fact, Marco et al. discuss that sac dissection poses the risk of bladder damage . In our Reduction of the incarcerated bowel is an essential step in surgical treatment of incarcerated hernia, whether it be internal supravesical or inguinal. Details of reduction were described in only a few cases in 4The peritoneal incision technique allows laparoscopic reduction of tightly incarcerated supravesical hernia.None.None.This case is not a research study.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Yugo Matsui: Responsible for literature review, writing and manuscript preparation. Operator of the surgery.Teppei Murakami: Responsible for manuscript preparation.Kenta Horita: Assisted in the laparoscopic surgery.Shotaro Matsuda, Aoi Tayama: Responsible for manuscript reviewThis is not a research study.Yugo Matsui.Not commissioned, externally peer-reviewed.


Teaching Point: A submucosal bladder wall lesion with high signal on T2-weighted MRI warrants blood and urine analysis to rule out a paraganglioma. A 57-year-old male presented with right flank colic pain. Non-contrast computed tomography (CT) Figure  confirmeBlood and urine work-up for excess catecholamine production showed slightly elevated urine normetanephrine and noradrenaline, suggesting the diagnosis of a paraganglioma. A cystoscopic biopsy specimen was obtained and the pathology results showed a lesion morphologically resembling UCC but expressing synaptophysin, leading to the diagnosis of neuro-endocrine tumour (i.e. paraganglioma). A whole-body 18F-fluoro-deoxyglucose positon emission tomography coupled with CT (F-18 FDG PET-CT) Figure  showed mBladder paraganglioma  is an exceedingly rare tumour arising from the chromaffin cells of the bladder, which explains their intramural location. They account for 0.05% of bladder tumours and for less than 1% of paragangliomas [


Olfactory neuroblastoma (ONB) is a sinonasal malignancy seldom seen in clinical practice. It is also known by various other names like esthesioneuroblastoma, esthesioneuroepithelioma, esthesioneurocytoma, and esthesioneuroma. Surgery and radiation therapy are considered as standard treatment modalities for ONB; however, the role of chemotherapy is not well established.We aim to define the role of chemotherapy in the neoadjuvant setting in a case of ONB.We report a young female patient presenting with a naso‐facial swelling causing facial disfigurement, proptosis, decreased visual acuity, and poor performance status. She was diagnosed with advanced‐stage ONB. Prompt administration of chemotherapy led to the improvement in the symptoms and rapid regression of the tumor mass. Later on, the tumor mass was excised completely without any neurological deficit.This report justifies the role of neoadjuvant chemotherapy in the management of ONB. It usually arises from the sensory neuroectodermal olfactory cells situated in the nasal cavity. The estimated incidence is 0.4 cases per million populations.1.1A 32‐year‐old female from a remote hilly village presented with an 8‐week history of progressive swelling around the nose, and severe bouts of headaches. For the last 2 weeks, she was also experiencing episodes of recurrent epistaxis, diminution of vision, and double vision. According to her husband, the disease had rapidly progressed in the last 4 weeks leading to severe facial disfigurement and now it had become difficult to recognize her. She was unable to do household chores and self‐care, being mostly confined to bed. On examination, the patient had a swelling involving the whole of the nose with facial puffiness and proptosis of the right eye. The right vestibule of the nose was full of some necrotic appearing mass. Bilateral level I and II neck nodes were enlarged. She had marked anhedonia and was chair‐bound, not responding to any queries.A contrast‐enhanced magnetic resonance imaging (MRI) of the brain and neck revealed a multi‐lobulated lesion of size 8.2 × 4 × 6.5 cm involving the right nasal cavity. The lesion was reaching anteriorly up to the external nare with infiltration into the upper lip, bilateral gingivobuccal sulcus, right masticator space, hard palate, and projecting posteriorly into the nasopharynx Figure , panel AHer blood counts, liver, renal, coagulation, and the metabolic panel were within normal limits. A biopsy from the right nasal cavity mass was obtained and it showed a lobulated tumor composed of nests of cells with scanty to moderate cytoplasm with round to oval nuclei dispersed in a neurofibrillary stroma Figure . Tumor cA final diagnosis of ONB, Kadish stage D, Hyams grade III was reached.Her case was discussed in the multidisciplinary tumor board. Since it was very difficult to achieve an R0 resection without much morbidity and neurological deficits, neoadjuvant chemotherapy (NACT) was planned. However, in a patient with poor performance status (PS) and severe depression, it was difficult to administer chemotherapy. The patient's husband was counseled in detail regarding the guarded prognosis and pros and cons of chemotherapy and he consented for the treatment.2) and etoposide (75 mg/m2); however, the dose was reduced by 15% keeping in view the poor PS of the patient. There was a striking improvement in signs and symptoms. Her epistaxis and headache resolved on day 7 post‐chemotherapy. Visual acuity improved and the double vision disappeared by day 10. On day 14, facial swelling and proptosis resolved, and PS started improving , and sequencing of the therapy have made it difficult to formulate a definite recommendation. A recent systemic review and meta‐analysis of advanced stage ONB suggested the role of chemotherapy in combination with surgery and/or radiation therapy. The choice of the regimen was not specified.The available evidence now suggests that ONB is a chemosensitive tumor and there is a definite role of chemotherapy in the management, albeit not very well defined. Chemotherapy can be used as an induction or neoadjuvant component rendering tumor more amenable to definite therapy in the form of surgery or radiotherapy. The mutilating surgery and the comorbidities after the surgery or radiation therapy can be avoided by the use of upfront chemotherapy. The impact of chemotherapy on survival, role in the adjuvant setting, and the choice of the regimen are, however, not well defined.Our case has several salient features. The patient had a poor PS and she was in severe depression. Administration of chemotherapy under such circumstances is difficult as it may do more harm than benefit. However, a cautious decision was taken as biology and history of the tumor suggested that the tumor might be responsive to chemotherapy. The patient regained her facial features which were otherwise lost due to disfigurement caused by the expansile tumor. Her vision was restored. She achieved a PCR which has not been reported in ONB.3ONB can present like a locally aggressive tumor, and prompt evaluation and treatment is necessary to prevent disease‐related morbidities.The tumor is highly sensitive to chemotherapy and NACT should be administered to downsize the tumor amenable to R0 resection.A cautious trial of chemotherapy can be done in patients with highly chemosensitive tumors who are otherwise unfit for systemic anti‐cancer therapy due to poor PS or comorbidities.My wife was very sick and in great depression for the last 1 month. I had lost all hopes. I was very skeptical about chemotherapy as I had many myths regarding the ill‐effects of chemotherapy. The treating team explained to me about the treatment and I showed faith in them. Her condition started improving remarkably within a few days after starting chemotherapy. Her vision has become normal. She has now completed her chemotherapy and surgery and we are thankful to the treating team.The authors declare that they have no competing interests.Available on request from the corresponding author.Approval from the institutional ethics committee and consent from the patient was obtained for the publication of this report.DS, BJ, RB, and PK collected and interpreted the data. RB, AS, AS, and PK prepared the manuscript. DS, AS, PK, AS, and BJ were involved in patient management. All authors read and approved the final manuscript.


Gastric fundus ischemia is a rare event, which does not account for many significant clinical studies. This disorder could have different etiologies, but authors agree that a prompt diagnosis and a proper treatment could avoid dangerous complications and ultimately the death of the patient.We herein report an interesting idiopathic case of acute gastric dilatation and fundus ischemia of an 83-year-old Caucasic woman who was admitted to the Emergency Department complaining of abdominal discomfort, vomiting and constipation.In literature, only a few case reports about this condition are reported. Possible risk factors, etiologies, and the different therapeutic options available for this condition are examined, in order to try to favor clinicians to formulate a timely diagnosis and provide patients with rapid healthcare services.Further investigations are still needed to analyze the pathophysiological pathways responsible of gastric fundus ischemia and to provide a definitive treatment to this dangerous disorder. Ulcers are breaks affecting the inner lining of the stomach. They are usually spotted in the duodenum or in the stomach antrum or pylorus, while they are less diagnosed in the stomach fundus .Only a few case reports are described in literature about acute necrosis and perforation of the gastric fundus, with different pathophysiological hypothesis. Gastric ischemic necrosis is characterized by inadequate blood supply to specific affected areas. There are several pathogenetic causes which may lead to a gastric necrosis, such as emphysematous gastritis , mucormy2An 83-year-old Caucasian woman with a history of hypertension, colon diverticulosis, hysterectomy and mastectomy after breast cancer was admitted to the Emergency Department. The patient complained of abdomen discomfort, vomiting and constipation for the last two days. After physical examination the abdomen was remarkable for tenderness with a positive Blumberg sign. Laboratory exams showed leukocytosis, with a progressive diuresis reduction. Abdominal CT scans demonstrated gastric distension and oesophageal dilatation, with free abdominal air and fluids . An emerOn 3rd post-operative day (POD) a parenteral nutritional therapy was initiated and general conditions were satisfying overall. A chest CT described pleural effusion, with no further alterations. However, the patient’s clinical conditions did not allow the execution of a gastrografin study. Therefore, a methylene blue oral test was performed, which resulted in no gastric leak. Hence, the nasogastric tube was removed and a hydric diet was well-tolerated.Unexpectedly, on 7th POD, a bile-like green liquid was seen both from the surgical incision and the surgical wound drains.An emergency re-laparotomy revealed a biliary peritonitis with minimal different perforation of the residual gastric fundus, next to the esophagogastric junction. Therefore, a formal sleeve gastrectomy was performed.After the second surgery, on 3rd POD, the nasogastric tube was accidentally removed by the patient and it was not repositioned due to the respiratory distress of the patient. Clinical findings showed a progressive lung function loss, while a routine arterial blood gas exam documented a metabolic alkalosis. High blood pressure, tachycardia, fever, oliguria and a surgical wound infection were documented. After nephrological counseling, plasma and high-dosage furosemide were prescribed to sustain the kidney function. Vancomycin and meropenem were administrated to prevent sepsis. On 4th POD lactates were found to be elevated and the echocardiographic ejection fraction was 35 %, with a biliary leakage from the abdominal drains and the surgical wound. A 3rd laparotomy was then performed. A biliary peritonitis with a surgical dehiscence was confirmed close to the gastric antrum. A gastrorraphy of the surgical leak was performed and reinforced with fibrin glue. The peritoneal cavity was abundantly debrided and irrigated with isotonic saline solution.The postoperative management was affected by a severe cardiorespiratory failure and a hydroelectrolytic discrepancy. After five days, the surgical drains and the wound showed a bile-leak yet again. Laboratory findings documented bile in the surgical drains. The patient died on 8th POD.After histopathological examination of both specimens, a gastric transmural ischemic infarction with serositis was documented.3Nowadays, gastric necrosis of the fundus is a rare condition. The actual incidence is unknown. In literature, less than 50 cases of AGD with ischemia and necrosis are reported . This coStreptococci, E. coli, Enterobacter spp., S. Aureus and Clostridium. CT scans are diagnostic because of the characteristic presence of gas in the gastric wall and stomach distention. Several factors might affect the integrity of the gastric mucosa, while others favor infections. Gastric infections, ingestion of corrosive substances, recent surgery, diabetes, rheumatic diseases and corticosteroids are included. Fulminant infections present with the classic pathognomonic sign of necrotic nasogastric aspirate. When a perforation is found, a surgical treatment is paramount [Emphysematous gastritis is an uncommon infection of the gastric wall usually caused by aramount .AGD is defined when the intra gastric pressure (IGP) from distention is above 30cmH2O. Then, IGP begins to impair the intramural blood flow, resulting in ischemia and necrosis of the stomach. However, besides eating disorders, other etiologies of this condition are undetermined .To the best of our knowledge, a gastric ischemic necrosis, which is not correlated with all of the abovementioned conditions, is an extremely rare event. Primitive gastric ischemia, either acute or chronic could be usually ascribed to systemic hypotension, gastric volvulus, disseminated thromboembolism, vasculitis or atherosclerosis. Gastric ulcers of the fundus are sporadically diagnosed, but when it presents as a chronic ischemia it is not advised to treat it with protonic pump inhibitors.In the current case report short gastric arteries and gastric arteries were completely pervious, so the most plausible pathogenesis seems related to a venous infarction. The hypothesis of gastric necrosis due to bacterial or fungal infections was declined by negative microbiological cultures. The idiopathic gastric necrosis of the fundus was described as a consequence of the acute increase in intragastric pressure. This event could, at first, reduce the local blood flow to the mucosa and subsequently to the general venous flow .Multiple reports were attempted to describe the etiology and pathogenesis of AGD. The simplest explanation and relatively frequent cause of AGD is extrinsic or intrinsic obstruction of the gastrointestinal tract. Nonetheless, in literature there are also multiple interesting correlations of AGD with different diseases such as muscular dystrophy  or diabeIn our case, there was no evidence of all the aforementioned causes of extrinsic or intrinsic obstruction of the gastrointestinal tract, and a similar case was already described by Todd et al. . DespiteThe second gastric perforation we described states the same histological conclusion of ischemic necrosis. This finding, when all other possible etiologies were discarded, suggests a plausible situation of idiopathic gastric paralysis with consequent AGD.A fast response is mandatory when AGD is encountered, since it might quickly become a life-threatening entity. Delaying the diagnosis process of AGD is proven to be fatal in more than 80 % of cases .According to our group, the surgical approach must be inspired by two general rules: 1) damage control, to stabilize the clinical situation of the patient; 2) sparing surgery, avoiding invasive gastric surgical operations every time it is indicated, saving as much healthy tissue as possible, in contrast with other authors . Since t4Several reports about acute fundus gastric necrosis are missing a definitive etiology, despite all of them containing risks. Different hypothesis were advanced, but a deeper knowledge of the disease is required to fully determine the best therapeutic strategy.None.None.Ethical approval has been exempted by our institution.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Valeria Tognoni: conception and design of the study, analysis of data, manuscript revision, final manuscript approval; Carlo Gazia: conception and design of the study, analysis of data, manuscript drafting, manuscript revision, final manuscript approval; Anton Mariani Ivanikhin: conception and design of the study, manuscript drafting, manuscript revision, final manuscript approval; Maurizio Rho: manuscript revision, final manuscript approval; Andrea Martina Guida: manuscript revision, final manuscript approval; Cristine Pathirannehalage Don: manuscript revision, final manuscript approval, Dario Venditti: conception and design of the study, analysis of data, manuscript revision, final manuscript approval.No registration for this case report is needed.Dario Venditti.Not commissioned, externally peer-reviewed.


A 36-year-old male patient presented at the Department of Maxillofacial Surgery, University Hospital Clinic Zagreb in December 2010 due to a swelling of the left body of the mandible that was noticed 4 months earlier. The patient was referred regarding an atypical clinical and radiological finding in the form of a multicystic appearance in the corpus of the left mandible and teeth mobility in the same region. A probatory biopsy was performed under local anesthesia and sent for histopathologic examination which reported odontogenic myxoma. The tumor was removed with a marginal resection of the mandible from the left first incisor to the left second molar. Two years after surgery, in January 2013, the patient was referred to the oral surgeons for implant-prosthodontic rehabilitation. Two narrow implants were placed at positions 32 and 36, and three months afterwards, implant-retained denture was made using locator connections to compensate lost teeth and to correct the ratio of soft tissues and facial contours. The patient was followed-up for 9 years without any functional and aesthetic problems. Loosing function and aesthetic morbidity, after radical surgical treatment, often have compromised the quality of life of this group of patients. It is important to highlight the need for multidisciplinary collaboration for the complete rehabilitation of the patient after surgical oncology of the maxillofacial region. Myxomas are very rare benign tumors of ectomesenchymal origin . These tDiagnosis of odontogenic myxomas is based on radiological, histopathological, and clinical findings . RadiogrThe aim of the present research is to describe a case of a 36-year-old man who developed an odontogenic myxoma of the mandible 9 years ago and successful implanto-prosthodontic rehabilitated after resective jaw surgery with a multidisciplinary approach. The purpose of this case report was to highlight the need for multidisciplinary collaboration for the complete rehabilitation of the patient after surgical oncology of the maxillofacial region.A 36-year-old male patient came to the Department of Maxillofacial Surgery, University Hospital Clinic Zagreb in December 2010 due to a swelling of the left body of the mandible that was noticed 4 months earlier. The patient did not report any other symptoms. After examination, the patient was referred to a maxillofacial surgeon regarding an atypical clinical and radiological finding in the form of a multicystic appearance (bubble-like) in the corpus of the left mandible and pathological teeth mobility in the region of the swelling. There were no enlarged lymph nodes in the neck and head area. Differential diagnosis included keratocystic odontogenic tumor, ameloblastoma, central giant cell granuloma, and odontogenic myxoma.The patient denied any systemic disease or condition and reported no previous surgeries. A general physical examination was unremarkable. The patient did not have any deleterious habits such as alcohol consumption or smoking and without previous history of swelling or trauma of the mandible. On extraoral examination, the patient displayed some facial asymmetry with an obvious firm and diffuse swelling on the left side of the mandible. The overlying skin was normal in appearance. Intraorally, there was a diffuse swelling in the buccal vestibule. Teeth in the third quadrant were without any pain sensation but with pathological mobility. Lower incisors and canine had mobility grade I and premolars and first molar grade II according to the Miller classification.The orthopantomogram (OPG) showed a multilocular radiolucency, with fine trabeculation, extending from the left canine, towards the first molar  with no In accordance with the ethical protocol of the School of Dental Medicine, University of Zagreb, Croatia, written consent was obtained from the patient before surgery in general anesthesia in January 2011. Lower incisors, canine, premolars, and first molar were extracted just before the first incision. A mucoperiosteal flap with full exposition of the buccal aspect of the tumor was performed. The tumor was removed with a marginal resection of the mandible from the left first incisor to the left second molar. The surgical site was examined and precociously cleaned of all myxomatous tissue, and a reconstruction plate was used to reinforce the resected mandible. The surgical specimen was a soft, gelatinous mass measuring approximately 1 cm in diameter, with a mucinous appearance. Histologically, the tumor was composed of randomly oriented spindle-shaped cells with long fine cytoplasmic processes, within an abundant, myxoid ground substance, containing small capillaries and fragments of bone trabecula. Odontogenic epithelium was absent, and clear margins were confirmed Figures . The patTwo years after surgery, in January 2013, the patient was referred to the Department of Oral Surgery, School of Dental Medicine, University of Zagreb for implant-prosthodontic rehabilitation. A control OPG  and clinDue to the deep bite, orthodontic therapy was started in the upper jaw, and in the lower jaw, implant-prosthodontic rehabilitation was planed with minimally invasive procedures because of the lack of a support zone and defect of soft and bone tissues. Regional nerve block anesthesia  was administered. After carefully raising the mucoperiosteal flap and isolating the mental nerve, the bone crest was expanded at the planned implant sites, with a piezosurgical bone saw , and two narrow diameter implants  were placed at positions 32 and 36 Figures  and 8. AThree months after surgery, a cover denture was made with locator connections to compensate for lost teeth and to correct the ratio of soft tissues and facial contours. No tumor recurrence was found on the follow-up OPG and CBCT for 7 years after implant placement and 9 years postresective surgery Figures 9. The patAccording to the literature , it is rClinically, the presented case showed symptoms that match all of these mentioned signs but without aggressive behavior and the lingual cortical plate was preserved.Odontogenic myxomas have been diagnosed with many different imaging features like conventional radiography such as panoramic, occlusal, and periapical, or more accurately like cone beam computed tomography/computed tomography (CBCT/CT) and magnetic resonance imaging (MRI) , 28. WanAlthough benign, odontogenic myxoma is invasive into surrounding normal bone, sometimes breaking through its boundaries . This inMicroscopically, the tumor has a mucoid-rich ECM, with scattered stellate cells, connective tissue fibers, irregular calcifications, bony trabeculae, sparse capillaries, and scant blood vessels. Nests of odontogenic epithelium are occasionally seen but not essential for diagnosis , 13, 23.Surgical treatment of odontogenic myxoma is usually invasive, and it depends on the size of the tumor from conservative, e.g., enucleation and surgical curettage, to radical treatment like partial or segmental jaw resection with free flaps. Smaller lesions can be treated only by curettage, but larger lesions need resection due to their infiltration to the surrounding bone. Large maxillary and mandibular bone defects can be reconstructed by fibular and iliac-crest osteomyocutaneous or osteomuscular free flaps . Free flLack of its encapsulation and loose of myxoid composition is the main reason of recurrence, so the method of removal is a crucial determinant . AccordiThe reconstruction of the mandible after radical treatment can be made by fixed or removable prostheses retained by a system attached to the implant. In some cases with reconstructed mandible with free flaps, there might be a problem due to inadequate mechanical retention during mastication so an implant-supported fixed dental prosthesis is the best solution for treatment . Meloni Implant-prosthodontic rehabilitation after marginal resection of the mandible requires filling the edentulous space and compensating the loss of hard and soft tissues, therefore presenting a particular challenge to prosthodontists. Removable dental prosthesis is preferred to an implant-supported fixed dental prosthesis to maintain oral hygiene and recurrent examinations and for long-term maintenance . Elsyad Quality of life in patients after radical surgical treatment of odontogenic myxomas is often compromised by loosing function and aesthetic morbidity. A multidisciplinary approach is the basis for complete implanto-prosthodontic rehabilitation of patients after resective surgical oncology.


Multifocal glioblastoma is a rare type of glioblastoma with worse prognosis. In this article, we aimed to report two cases of classical multifocal glioblastoma.In case 1, a 47-year-old male presented with dizziness, and once had a sudden loss of consciousness accompanied by convulsion of limbs. Contrast-enhanced MRI showed multiple lesions with heterogeneously ring-enhanced characters in the left hemisphere, diagnosed as multifocal glioblastoma. He underwent a craniotomy of all lesions, concurrent radiotherapy and chemotherapy as well as additional chemotherapy of temozolomide. After 2 cycles, repeat MRI showed that the new lesions already occurred and progressed. Eventually, he abandoned the chemotherapy after the 2 cycles and died 1 year later. In case 2, a 71-year-old male presented with a history of headache, left limb weakness, and numbness. Discontinuous convulsion of limbs once occurred. Contrast-enhanced MRI showed multiple lesions located in the right hemisphere, diagnosed as multifocal glioblastoma. He underwent a right frontoparietal craniotomy of the main lesion. Hemorrhage of the residual tumor and pulmonary artery embolism occurred synchronously. Eventually, his family decided not to pursue any further treatment and opted for hospice care and he passed away within 11 days of surgery.We reported two cases of typical multifocal glioblastoma. Valid diagnosis is crucial; then, resection of multiple lesions and canonical radio-chemotherapy probably bring survival benefits. Glioblastoma multiforme (GBM) is the most common primary malignancy in central nervous system with high aggressiveness and extremely poor prognosis . AlthougMultifocal glioblastoma is a rare type of glioblastoma multiforme and it associates with worse prognosis compared with solitary ones . It maniHere, we present two cases of typical multifocal glioblastoma and we also have reviewed related literatures, and the aim of this report is to discuss the diagnosis and treatment of this peculiar malignancy.A 47-year-old male presented with dizziness for 10 days, and he also complained that he once had a sudden loss of consciousness accompanied by convulsion of his limbs which lasted for 3 min at the this duration. Syndromes of headache, vomiting, and hypoplasia were not described. Mild cognitive impairment and weakness of the right side were detected at admission.a–i).Computed tomography (CT) of the brain were performed, which manifested multiple intracranial lesions synchronously. Positron emission tomography-computed tomography (PET-CT) revealed no abnormal neoplasms in the other tissues of the body. The magnetic resonance imaging (MRI) revealed 4 occupying lesions with surrounding edema in the left frontal and parietal lobes. Deep-seated lesions invaded to the parenchyma adjacent to the left ventricle, contributing to the mass effect and middle line shift. The whole masses presented with a heterogeneously ring-enhanced character with necrosis and cystic changes after gadolinium (Gd) administration Fig. a–i.Fig.The patient underwent a left frontoparietal craniotomy of 3 lesions with the guidance of intraoperative navigation and sodium fluorescence-guided technique. Lesions were seen with grey appearance, obscure boundary, and rich blood supply and removed for decompression of the intracranial pressure. We successfully resected the masses micro-surgically for additional biopsy. Then, the gross total resection of these masses around their approximate boundary was achieved. We used the same way to resect the last lesion in occipital lobe under prone position.j, k). Immunohistochemical staining demonstrated that these lesions shared the similar characters of intensity of the biomarkers such as glial fibrillary acidic protein (GFAP) and Olig-2. The abnormal expression of Ki-67 (20%) revealed the potential proliferation activity of the tumor cells which indicated poor prognosis and high incidence of recurrence. Both the lesions showed the amplification of epidermal growth factor receptor (EGFR), which indicated the high aggressiveness. Pathological results were compatible with a diagnosis of multifocal glioblastoma. We also performed the whole genome sequencing (WGS) of the four lesions. The WGS results revealed that four samples had TERT promoter mutation C228T , but none of IDH1/2 mutation, ATRX mutation, and EGFRvIII were detected.Histopathological examination revealed that these tumor cells possessed increased necrosis, and significant heterogeneity of nucleus Fig. j, k. Imma–i). Concurrent radiotherapy and chemotherapy were then administered to the patient.The patient presented with no other abnormal neurological signs and his right limb’s strength had improved gradually after operation. There was no recurrence of his epilepsy at this duration. After 5 weeks, a follow-up MRI was performed before radiotherapy and revealed that local tumor control was achieved and the initial resection cavities were seen; however, two new lesions occurred adjacent to the primary lesion areas and exhibited intense enhancement; nevertheless, remarkable mass effect and middle line shift were not observed either Fig. a–i. ConcIntensity-modulated radiotherapy (IMRT) targeted these lesions and areas of enhancement. A dosage of 60.0 Gy in 30 fractions were performed and constantly orally administered temozolomide (TMZ), with a dosage of 120 mg (75 mg/m2/day) per day for 46 days were also performed to achieve the concurrent radio-chemotherapy. Side-effects and toxicity such as liver and hematopoietic dysfunction did not occur.a–i). Eventually, the patient abandoned the chemotherapy after the 2 cycles due to his low quality of life. The patient died 1 year later.The following adjuvant chemotherapy of temozolomide with 240 mg (150 mg/m2/day) for 5 days every 28 days was then administered to the patient. Hematologic and gastrointestinal toxicity was examined every cycle and no significant abnormality was detected. We also monitored the functions of liver and kidney constantly. Dysfunctions of the organs and anaphylaxis were also not seen. After 2 cycles, the repeat MRI revealed that the former two new lesions enlarged distinctly, manifesting ring-enhanced character, and two more new lesions had emerged again, infiltrating the white matter tract of ventricles and parenchyma Fig. a–i. EvenA 71-year-old male presented with a history of headache, left limb weakness, and numbness for 1 month. He also described that discontinuous convulsion of the limbs once occurred when he asked for medical service at this duration. Syndrome of vomiting, hypopsia, and cognitive impairment were not detected.a–f). PET-CT was also performed and showed hypometabolic foci of FDG corresponding to enhancing lesions seen on the MRI, but the chest, abdomen, and pelvis were negative for any primary lesion.Gadolinium-enhanced MRI was performed, demonstrating 4 well-defined, highly enhanced lesions located in the right frontal and parietal lobes which were suspicious for metastatic lesions, and one of them was adjacent to the right lateral ventricle  and no significant postoperative abnormal neurological signs were detected.To achieve the goals of safe resection and obtain the mass for histopathologic diagnosis, the patient underwent a right frontoparietal craniotomy of the main lesion near the surface of parenchyma with the guidance of intraoperative navigation and intravenous fluorescein. The mass was seen with grey and white appearance, obscure boundary, and moderate blood supply. The gross total resection of the mass around its approximate boundary was also achieved Fig. a–f and ng, h). Immunohistochemical staining demonstrated the highly expressed Ki-67 index (30%), positive GFAP, Olig-2, CD34, CD99, P53, and ATRX whereas, negative IDH1, NeuN, and Vimentin. The final results were also compatible with a diagnosis of multifocal glioblastoma.Histopathological examination revealed infiltrating highly proliferative cells with nuclear atypia which fulfilled the criteria of malignant glioma Fig. g, h. Immg); meanwhile, the CT angiography of pulmonary artery showed a sign of pulmonary artery embolism . Conservative treatment was implemented in consideration of the condition of the patient; eventually, the patient’s family decided not to pursue any further treatment and opted for hospice care and the patient passed away within 11 days of surgery.The patient suddenly presented a sign of dyspnea at the 10th day after operation, followed with decreased conscious level and blood-oxygen saturation. The emergent CT showed hemorrhage of the residual tumor located in the right parietal lobe closed to the mid-line Fig. g; meanwhism Fig. h. ConserNewly diagnosed multiple glioblastoma can be termed as multifocal or multicentric ; generalMultiple intracranial lesion imaging characters represent a diagnostic dilemma in that multiple ring-enhancing lesions usually were diagnosed as metastatic entities or brain abcesses , 15. In Local irradiation therapies like IMRT and three dimensional conformal RT (3DCRT), which cover the gross target volume locally, now remain the standard adjuvant treatments of unifocal glioblastoma due to their lower toxicity and accuracy. In our case 1, we adopted IMRT with a dosage of 60.0 Gy in 30 fractions, and side-effects were not detected during the follow-up period. Recent studies also investigated the safety and efficacy of whole-brain radiotherapy (WBRT) in GBM patients. Lahmi et al. reported their retrospectively study of patients received the WBRT with TMZ and confirmed the safety and efficacy of WBRT, but larger prospective studies are needed to support their results . The StuUnderlying genetic characteristics of multifocal glioblastoma have not been elucidated clearly. Liu et al. found that multifocal glioblastomas had no IDH1, ATRX, or PDGFRA mutations compared with solitary glioblastomas and were significantly associated with the mesenchymal subtype ; Abou-ElCurrently, there are still no uniform treatment guidelines for multifocal glioblastoma; thus, further investigation and evaluation are required for unveiling this rare malignancy.In summary, we have reported two cases of typical multifocal glioblastoma. The resection of multiple lesions and canonical radio-chemotherapy probably bring survival benefits; meanwhile, further investigation is required for understanding the behavior, management, and outcome of this rare malignancy.


We report a case of an isolated schwannoma of the urinary bladder, and also searched the English literature as we had access to bladder schwannoma.The urinary bladder schwannoma is an extremely rare primary urologic tumor. Schwannoma arises from the Schwann's cells in the nerve sheaths and is almost associated with von Recklinghausen's disease. We report a case of schwannoma in a 35-year old female who presented with urinary frequency for 2 months with absence of evidence of von Recklinghausen disease and successfully treated with transurethral resection of bladder lesion (TUR_B). To the best of our knowledge, this represents only the 10 Peripheral nerve tumors are rare and may arise from the Schwann cells, ganglion cell or capillaries of the nerve cells tumors; among them, schwannoma which is the most common tumor of the peripheral nerves is a benign tumor ,2. TheseThe patient was a 35-year old female who presented with urinary frequency for 2 months. Her past medical history showed a nonsmoking history and no significant family history. The physical examination was unremarkable. She was treated as urinary tract infection, but there was no improvement in her symptoms. Urine analysis and culture were performed several times that was negative for infection. Other laboratory data were also insignificant. Through a routine evaluation, urinary system ultrasonography (US) revealed a bladder lesion measuring 7 mm. In order to confirm the diagnosis, magnetic resonance imaging (MRI) of the abdomen and pelvic cavity was done; it revealed a lesion in the dome of the bladder . To confrd to 6th decades of life [The nerve tumors are named peripheral neural sheath tumors (PNSTs) and it may be benign or malignant. Benign PNST are schwannoma and neurofibroma . Schwann of life . Extracr of life . Patient of life . Treatme of life . Schwann of life ,8. We foSchwannoma of the bladder is rare as a primary urologic tumor and the optimal treatment is surgery with excellent prognosis.The authors declare no competing interests.


Epignathus is a rare congenital orofacial teratoma. Teratomas are tumors that originate from all three germs cell layers. Tumor size is an important prognostic factor, and we describe the case of massive epignathus identified by sonography at 25 weeks. Our case was a 35-year-old pregnant woman that was subjected to a routine ultrasound at 25 weeks of gestation, and epignathus was diagnosed. Labor pain started in the 28th week of the pregnancy, so the dead fetus was aborted, and curettage was conducted. A pathologic sample was sent to the laboratory, and benign teratoma was diagnosed. Because fetal epignathus has a wide range of outcomes, early prenatal diagnosis is essential for optimal management. Head and neck tumors are rare. Epignathus is a rare congenital orofacial teratoma of Rathke’s pouch, and it usually appears as a mass that involves the sphenoid and the skull base -3. AlthoPolyhydramnios due to impaired fetal swallowing, intrauterine death due to fetal hydrops and pre-eclampsia are all acute presentations . In the A female 35-year-old patient was referred to Imam Reza Hospital, Kermanshah, for the first time, to be evaluated by routine sonography. She did not have any previous systemic disease. She had two curettages two and four years ago because of abortions in the second and third months of pregnancies. The patient complained of mild vaginal discharge but denied any amniotic fluid leakage. Based on the sonography, the fetus was female, and it was in cephalic presentation. The fetus was alive, and the patient was 25 weeks pregnant. When assessing the head and face, a mass was observed in the anterior part of the face. There were also some echogenic spots with posterior shadows in some parts of the tumor -2.Although the tumor was massive and it concealed the face, the main source of the tumor was not evident; nonetheless, the fetus’ mouth was wide open and it was supposed that the tumor is originating from the hard palate. No other anomaly was found in other parts of the body. The level of amniotic liquid was normal. No other apparent anomaly was reported regarding the rest parts of the body. Labor pain started in the 28th week of the pregnancy and the dead fetus was aborted. A large, 180 × 130 × 110 mm lobulated mass was extruded from the extremely opened mouth of the fetus . A pathoTeratomas are rare tumors that may arise from different parts of the body. However, tumors arising at the level of the skull base are even rarer and are called epignathus . Up to nThe largest tumor was reported by Yanez et al., and it was 30 cm × 40 cm .Epignathus can originate from face bones, especially the palate, sphenoid, and ethmoid; in some cases, the tumor is too big and comes out of the mouth . This maPolyhydramnios was not observed in our case or Hassan’s case . The likEarly diagnosis of the disease is possible, and some authors have reported this type of tumor even by the 15th week of pregnancy . AccuratTeratomas have varied etiologies. Chromosomal abnormalities are associated with cases of congenital teratomas, which commonly include trisomy 13, ring X-chromosome, mosaicism with inactive ring X-chromosome, gonosomal pentasomy 49, gene mutations, or abnormalities in the early embryonic development . The tumBecause the prognosis and management of epignathus depend on tumor size, early prenatal sonographic diagnosis is an important aspect in terms of follow-up and decision making.The authors declare that there is no conflict of interest.


Polycythemia vera (PV) is the most common myeloproliferative neoplasm, characterized by erythrocytosis, leukocytosis, and thrombocytosis and often associated with splenomegaly . NeuroloExtramedullary hematopoiesis (EMH) is a compensatory physiological response of the organism to the failure of hematopoiesis at the bone marrow, which occurs commonly in patients with chronic hemolytic anemia or diseases with ineffective erythropoiesis . UsuallyA 48-year-old man with known polycythemia vera for 6 years presented with acute tinnitus. Six months before this, he was diagnosed with disease progression due to splenomegaly, anemia, leukocytosis , and thrombocytopenia.Magnetic resonance (MR) imaging of the brain was performed on a 1.5 T scanner , consisting of axial fluid attenuation inversion recovery (FLAIR), sagittal T1-weighted (T1WI), coronal T2-weighted (T2WI) MR images, diffusion-weighted imaging (DWI), and a post-contrast T1WI study in two planes. In the right temporal lobe, there was a T1WI isointense (Fig. Elective splenectomy was performed 2 days later, and histology revealed extramedullary hematopoiesis. On a follow-up brain MR scan, 6 weeks after the initial exam, the lesion showed a significant reduction in size, with central T1W- and T2W hyperintensity and a dark peripheral rim. No edema and no contrast enhancement were detected. Six weeks later, on the last follow-up MR scan, there was only a non-enhancing linear scar with hemosiderin deposits (Fig. We present the first unique case of intracranial intraparenchymal EMH in a patient with polycythemia vera. PV is a myeloproliferative disorder, presenting with neurological complications mostly related to hypercoagulability and elevated hematocrit, such as ischemic strokes, TIAs, and atypical TIAs . EMH is Imaging features of the lesions in our case were highly suggestive of extramedullary hematopoiesis; T1W isointensity and T2W hypointensity of the lesion were consistent with a high-cellularity lesion. Elevated diffusion excluded thromboembolic etiology. On SWI, the lesion showed susceptibility artifacts, suggesting the presence of blood products. Finally, there was a pattern of nonspecific, patchy enhancement, thus excluding lymphoma or sarcoid granuloma. The lesion showed reduction in size after treatment, resulting in a scar with a small hemosiderin deposition. Signs of radiological regression followed the clinical improvement of the patient.One limitation of the study is the lack of histological proof of the diagnosis. However, imaging features of the lesion and the almost complete resolution of the lesion after splenectomy, in our opinion, exclude all potential differential diagnostic options.The tinnitus in our patient can be explained by the extramedullary hematopoietic lesion in the temporal lobe that caused a disturbance in the network of the anterior cingulate cortex, the anterior insula, and the amygdala . To conc


Congenital intrahepatic portosystemic venous shunts are rare vascular malformations which are incidentally discovered on imaging or once hepatic encephalopathy becomes clinically apparent. Surgical ligation and endovascular embolization are potential treatments. Congenital intrahepatic portosystemic venous shunts are rare vascular malformations which are incidentally discovered on imaging or once hepatic encephalopathy becomes clinically apparent. Surgical ligation and endovascular embolization are potential treatments. They often go undiagnosed and are incidentally discovered on imaging or once hepatic encephalopathy becomes clinically apparent. We present two cases of incidental congenital portosystemic shunts in noncirrhotic patients who went on to develop refractory encephalopathy without having prior history of liver disease. Both patients were successfully treated with embolization. Familiarity with the pathogenesis and imaging features may enable prompt diagnosis and help guide appropriate patient endovascular or surgical management.Congenital intrahepatic portosystemic venous shunts (IPSVS) and extrahepatic portosystemic shunts make up the so called congenital portosystemic shunts (CPSS). Congenital portosystemic shunts are an important disorder in children and should be differentiated from metabolic deficiencies involving hyperammonemia or galactosemia.. These abnormalities include intrahepatic connections between branches of portal vein and hepatic veins.Congenital intrahepatic portosystemic venous shunts (IPSVS) have a prevalence of 1 in 30 000 births and are caused by abnormal involution of the fetal vasculature. In these patients, there is a persistent communication between vitelline veins of the omphalomesenteric system and the sinus venosus due to a focal absence of sinusoid formationsKanasawa et al proposed a classification based on the correlation of the severity of portal hypoplasia  with portal venous pressure, histopathological findings, postoperative portal venous flow, and liver regeneration.Congenital IPSVS can present with various symptoms and ultimately can lead to long‐term complications by permitting hepatic bypass of mesenteric venous blood return.Over time, unprocessed portal metabolites result in hyperammonemia and subsequent hepatic encephalopathy.If the medical or operative treatment fails, liver transplantation is the only therapeutic option being cautious with major hemodynamic changes that can occur in the intraoperative period. Resection is the treatment of choice in patients when liver tumors are associated with extrahepatic portosystemic shunting.We describe two cases of congenital intrahepatic portosystemic shunts, discovered incidentally in noncirrhotic adult patients who develop refractory encephalopathy without having history of liver disease or prior surgical intervention. The two patients were successfully treated using transcatheter embolization with clinical improvement. Endovascular treatment offers a safe alternative of therapy after evaluation of anatomical and pathophysiological characteristics of the anomaly.1.1A 75‐year‐old woman with no significant history of liver disease or neurologic disorder was evaluated for altered mental status. Her initial ammonia level was 33 mmol/L , and liver function tests (LFTs) were within normal range. A CT of the abdomen was performed which demonstrated an intrahepatic shunt between the right posterior portal vein and the right hepatic vein  was manipulated through the shunt into the main portal vein. Prior to embolization, a temporary balloon‐occlusion test of the shunt was performed using a 12‐mm Berenstein occlusion balloon catheter (Boston Scientific). The test did show a portal pressure of 9 mm Hg before and 16 mm Hg after balloon occlusion, and this lasts remaining unchanged after several minutes. The findings were interpreted as no significant hemodynamic changes or risk of portal hypertension that would preclude shunt occlusion. Therefore, the shunt was then successfully embolized with Penumbra POD coils (Penumbra). Due to the high flow nature of the shunt, it was necessary to use coils that were approximately 50% oversized for the targeted vessel diameter to prevent potential coil migration and nontarget embolization. Following POD embolization, an Amplatzer Vascular Plug II  was deployed as a safety measure to further avoid nontarget coil migration into the right atrium and pulmonary vasculature.Following embolization, a contrast‐enhanced CT of the abdomen and pelvis was performed which demonstrated successful exclusion of the venous‐venous malformation with thrombosis of the posterior segment branch of the right portal vein Figure . Follow‐1.2A 72‐year‐old man with no significant history of liver disease, neurologic disorder, or prior relevant surgical intervention presented with the acute onset of lower extremity weakness and confusion. A full neurological workup was performed which included a normal electro encephalogram (EEG) and an unremarkable MRI of the brain. An ammonia level of 145 mmol/L  was measured which suggested hepatic encephalopathy as the cause of the patient's altered mental status. A contrast‐enhanced CT of the abdomen and pelvis was then performed which demonstrated an intrahepatic portosystemic shunt between the left portal vein and the left hepatic vein with multiple venous connections (Figure 2Intrahepatic portosystemic shunts are often incidentally detected on imaging or diagnosed after symptoms of hepatic encephalopathy develop. The incidence has been reported as 1/30 000. They have been associated with other types of anomalies such as cardiovascular, hepatobiliary, urogenital, and gastrointestinal among others. In addition, complications like portopulmonary hypertension, reported in 13%‐66% in children,Multiple imaging modalities are useful for diagnosis of an intrahepatic portosystemic venous shunt including color Doppler ultrasonography, contrast‐enhanced CT, MRI or conventional catheter‐directed angiography. The imaging finding most consistent with diagnosis of a portosystemic venous shunt is visualization of a direct communication between portal and hepatic veins; however, this is not always demonstrated on imaging. Secondary findings on imaging suggestive of a shunt include abnormal blood pooling from a dilated portal branch with early visualization of the hepatic venous system.Multiple challenges can be present during the embolization including diminutive portal vein in large portosystemic shunts, difficulty to determine the anatomy especially in types II or IV if multiple venous communications can be present or large veins with potential risk of coils or plug migration. Optimal oversize of the plugs or coils is recommended to avoid any potential undesired migration to the systemic circulation. Patient follow‐up is mandatory to evaluate for the ammonia levels and to determine whether further embolization is required.3Intrahepatic portosystemic venous shunts are rare hepatic vascular malformations that often remain asymptomatic and are discovered incidentally on imaging. Alternatively, these patients may present with hyperammonemia and encephalopathy among other symptoms, and with proper endovascular management, this detrimental sequela is potentially reversible. Technical challenges during endovascular treatment could be overpassed with detailed evaluation of the anatomy as well as hemodynamic changes including identifying multiple potential venous communications, measuring portal pressure, and selection of the more appropriate embolization material.None declared.MPBM: served as the primary author; was responsible for accuracy and integrity of the case report; and involved in the conception of the article, performed the interventional radiology procedures of each case, collected the radiologic imaging studies, reviewed the manuscript drafts, and approved and modified all versions of the manuscript. AK: did the literature review and wrote the background of the manuscript. JCB: served as the coauthor; did the modifications and corrections suggested by the reviewers; complemented the background, results, and description of the cases; and additionally corrected and supplemented the references of the last manuscript. CL: served as the coauthor and helped to write the first draft of the manuscript. PC: served as the coauthor, and interpreted and read the diagnostic CT images. AS: is the corresponding author; involved in design and writing of drafts including all the manuscript sections; uploaded the images; collected the cases; and submitted the case report every time when needed. SB: served as the coauthor; reviewed the drafts; helped in the interpretation of interventional radiology images; and helped in maintaining the accuracy of all aspects of the manuscript.


Gossypiboma is a term used to describe a pseudotumor caused by accidental retention of surgical swab or sponge in the body after surgery. The abdominal cavity is the most common site of retained surgical sponge. It is quite an infrequent surgical complication which is usually rarely reported because of the fear of medico-legal consequences. Here, we are reporting a case of a 26-years-old woman referred to our outpatient surgery clinic (OPD) from another hospital with complaint of intermittent abdominal pain, fever, and abdominal lump for 4 months following removal of IUD, which was attempted laparoscopically, and later converted to open laparotomy. She also had a history of cesarean section done one and half year ago in the same hospital. Clinical examination revealed a palpable abdominal mass in the para-umblical region. However, a computed tomography (CT) revealed a huge intra-abdominal mass. A diagnosis of intra-abdominal gossypiboma was suggested and the patient underwent exploratory laparotomy where the diagnosis was confirmed and the mass was excised. Gossypiboma, textiloma or more broadly retained foreign object (RFO) is the technical term for surgical complications resulting from foreign materials, such as a surgical sponge, accidentally left inside a patient's body. Gossypiboma is a mass formed around involuntarily retained surgical sponge or swap after surgery due to foreign body reaction. The term is derived from a Latin word “Gossypium” that is cotton wool and the suffix word - oma” that means a tumor or growth, and describes a mass within a patient's body comprising a cotton matrix surrounded by a foreign body granuloma . It’s quoC, and purulent fluid discharge from the site of surgery. The patient was reassured and discharged on oral antibiotic. During the following months her condition did not improve and she had recurrent episodes of vague abdominal pain. However, within the past two months, the pain increased in intensity and the patient started to notice an abdominal lump which was progressively increasing in size. The patient was then referred to our hospital.Physical examination revealed palpable abdominal mass in the para-umblical region, measuring 16cm x 14cm in size, rounded, firm in consistency, and mildly tender. All routine investigations were within normal limits. Contrast enhanced computed tomography (CT) revealed well defined heterogeneous intra-abdominal mass measuring 15 cm x 14 cm x 12 cm, located in the umbilical region, showing enhanced wall  is a quite rare surgical complication, it can lead to serious complication and increase in the mortality and morbidity rate. The inflammatory reaction to the non-absorbable cotton sponge leads to a variety of costs to society, including pain and emotional distress experienced by the patient, imaging studies to diagnose the problem, a subsequent hospitalization for reoperation, litigation expenses and patient compensation for perceived negligence of the facility and staff . The truThe possible causes of sponge retention are emergency surgery, unexpected change in the surgical procedure, disorganization (e.g. poor communication), hurried sponge counts, long operations, unstable patient condition, inexperienced staff, inadequate staff numbers, and obesity . The thrThe reported cases in literature have shown that some cases may remain silent and not detected for many years after surgery. However, the main signs and symptoms are abdominal pain (42%), palpable abdominal mass 27%), and fever (12%) . Less co%, and feGossypiboma or retained surgical swap can be difficult to diagnose due its various and vague clinical presentations. However, in patients who present with palpable abdominal mass and a history of previous abdominal surgical operations, the diagnosis of Gossypiboma should be included in the differential diagnosis. Even though it’s a rare condition, it can cause serious complications and has medico-legal implications. Therefore, strict measures should be implemented to avoid it.


Objective: To emphasize the importance of an early diagnosis and an adequate treatment in conjunctival tumors.Methods: We present two clinical cases and compare the course of each case: one of conjunctival intraepithelial neoplasia (CIN) which took a positive course, and a fatal case of squamous cell carcinoma (SCC) with intraocular and orbital extension.Results: Epithelial conjunctival malignancies are one of the most prevalent ocular surface tumors. Among these, CIN are the most common. CIN have an excellent prognosis, given adequate treatment. However, when the diagnosis of CIN is late, the epithelial basement membrane will be affected, resulting in SCC. SCC may have poorer results due to its capacity to infiltrate near tissues and create distant metastasis.Conclusion: It is not common today to treat patients with orbital extension of SCC; however, it is crucial to note the importance of an early diagnosis of conjunctival malignancies. An early diagnosis is essential to prevent the transformation to other life-threatening types. Conjunctival tumors are one of the most common ocular surface malignancies , 2]. Th. Th2]. TEpithelial neoplasms can be divided intocarcinoma in situ/conjunctival intraepithelial neoplasia (CIN), which exclusively affect the epithelium;invasive squamous cell carcinomas (SCC), when the epithelial basement membrane is damaged and neoplastic cells are present in the corneal stroma .We report two clinical cases of conjunctival neoplasm. The first one exhibits a patient affected by CIN with an early diagnosis and effective treatment based on topical chemotherapy and surgical excision. In contrast, the second case shows a patient with SCC with an aggressive clinical course and intraocular and orbital extension.The fact that the cell origin was the same in both cases shows that an adequate diagnosis and optimal treatment are required to control the progression of these types of tumors. Intraocular and orbital extension is infrequent but commonly fatal.A 70-year-old man presented with a conjunctival lesion in his left eye that had appeared four months earlier. Visual acuity (VA) was 20/200 in both eyes. Slit-lamp examination showed a nasal limbal gelatinous mass with inferior corneal infiltration and cataract Figure 1 . IntraocA 90-year-old man was referred to us with a mass in his right eye that had first been noticed three months earlier and had been growing progressively since then. VA was 20/200 in the right eye and hand movement in the left eye. Slit-lamp examination revealed a gelatinous temporal conjunctival mass  with dilated superficial vessels  overexpression was an important aspect when considering targeted therapies in cases of inoperable advanced orbit and periocular SCC. EGFR inhibitors such as erlotinib have shown the effect of a significant decrease of SCC tumor size. Targeted therapy might be appropriate for non-surgical candidates, comprising patients with advanced metastatic orbital disease involving regional lymph nodes, advanced age, and/or patients with multiple comorbidities. This therapy is associated with fewer side effects and is better tolerated by patients .Patients who reject treatment have a high mortality risk: it has been shown that these patients die after 13 months due to cancer progression . The patAll patients must be checked every 3–6 months because most recurrences occur during the first year of follow-up, and 30% of recurrences occur more than one year after surgical treatment.Miller et al. recommended that advanced stage SCC should be closely monitored as recurrences can occur even more than 5 years after treatment .Early diagnosis is essential in epithelial conjunctival tumors. Histopathological examination allows a classification of the lesions as premalignant or malignant, which is crucial for the development of a therapeutic strategy.An excisional biopsy with cryotherapy and free margins is sufficient in most cases of CIN. Topical chemotherapy can be used additionally. In cases of SCC, surgical excision may be sufficient, but may also be combined with adjuvant topical therapy, which may enhance treatment in cases of insufficient surgical removal. In addition, when SCC penetrates the intraocular or orbital septum, enucleation, exenteration, radiotherapy, or targeted therapy have to be performed on each specific case.The authors declare that they have no competing interests.Informed consent has been obtained from the patient for the publication of this case report.


Inguinal hernia is probably one of the most common surgical conditions, with strangulation accounting for a good number of acute surgical admissions. It has always been known that direct hernias are less likely to strangulate due to wide hernial defects in comparison to indirect hernia. For that reason, some surgeons do not attempt repair of direct hernias in elderly patients. We present a relatively uncommon case of a 58-year-old gentleman who presented with clinical signs of an incarcerated inguinal hernia; which was found at exploration to be a strangulated direct hernia with small bowel perforation. We believe that direct inguinal hernia should always be included in the differential diagnosis of incarcerated or strangulated groin hernia. Inguinal hernia repair is probably one of the most common procedures in general surgery. Yet, sometimes it can be a diagnostic challenge a fact that can be attributed to the complex anatomy of the groin. It has always been believed that direct hernias are less likely to present with strangulation due to the relatively wide neck in comparison to indirect inguinal hernia. Based on that, some surgeons do not attempt to direct inguinal hernia repair in older patients. However, one study showed that age is the most important prognostic factor for strangulation, with increased incidence in older patients; they also found that the average diameter of the transversalis fascia hernia defect was 23.8 mm. Hence, they concluded that the diameter of the hernial defect is not directly related to the incidence of strangulation .We are presenting a case of a 58-year-old gentleman who presented to the accident and emergency department with a tender right groin swelling of about 36-hour duration, following heavy object lifting, pain was dull aching in character and progressively increasing in intensity. His past medical history included that he was a heavy smoker who smoked about 20 cigarettes per day for about 20 years, and he has been suffering from right groin pain every time he lifted or pushed any heavy object with no other medical conditions of significant importance. General examination was unremarkable; his vital signs were within normal apart from tachycardia of 90 bpm. Abdominal examination revealed lax abdomen with no palpable masses or organomegaly, a tender right direct/indirect inguinal hernia, with no impulse on cough, scrotal examination was normal Figure .All his blood were within normal range; no scans were done at that time. The provisional diagnosis was an incarcerated inguinal hernia, so he was consented and taken to the theatre for repair of the inguinal hernia. At operation, it was found to be a strangulated direct hernia containing a perforated loop of the terminal ileum Figures , 3. A laparotomy was carried out through an infra-umbilical midline incision, revealing perforated distal ileum, resection-anastomosis of the perforated segment of the bowel, peritoneal lavage was carried out, two Robinson drains were left in the peritoneal cavity, and the hernia was repaired by Lichtenstein technique Figure .The patient passed through a smooth postoperative period; he was discharged home on the fifth postoperative day and seen in the clinic after six weeks with no complications.While inguinal hernias are relatively common, with an estimated prevalence of 6% ; incarceIncidence of strangulation varies from 3.25 to 7.16 per 100,000 population per annum .Watkin et al. reported that in their cohort the risk of strangulation was inversely related to the diameter of the hernia defect , they also reported that incidence of strangulation was only 3% in direct hernias . On the When Hair et al. looked into intraoperative findings of patients presenting with strangulation they found that 50% of hernias emerged through an indirect defect, 30% were recurrent hernias, 10% emerged through a pantaloon hernia (having both direct and indirect components), and 10% emerged through a direct hernia .While it is well established that elective inguinal hernia mesh repair is associated with superior results when compared with Bassini repair; there has always been a debate regarding mesh repair in strangulated inguinal due to the fear of increased incidence of infection hernia. This debate was resolve following multiple studies confirming that even in cases with strangulated inguinal hernia, Lichtenstein “tension-free” mesh repair is safe and effective with low postoperative complications and recurrence rates ,11.In our case, the preoperative diagnosis was an incarcerated inguinal hernia; we initially thought that strangulation is unlikely due to the absence of systemic manifestations and signs of bowel obstruction and also the fact that strangulation is very rare with direct hernias. At operation, the hernia was found to be a direct one containing a strangulated perforated loop of the terminal ileum, a rather uncommon finding in a direct hernia. Resection anastomosis of the perforated segment of the bowel was carried out with uneventful intraoperative and postoperative courses.Inguinal hernia is a common surgical condition accounting for a good number of elective as well as acute surgical admissions; for the experienced surgeon, diagnosis and repair should be straightforward; however, unexpected operative findings may be encountered requiring an expert approach. The urgent need to repair strangulated inguinal hernias should not have a negative impact on adequate preoperative imaging to ascertain an accurate diagnosis preoperatively and rule out concomitant intra-abdominal pathology. We recommend reporting direct inguinal hernia cases to have an extensive database which will allow us to come up with more informative recommendations.


Paraduodenal pancreatitis (PP) is an uncommon abdominal pathology characterized by scarring of the pancreaticoduodenal space. Diagnosis of this inflammatory process is challenging as its clinical presentation is similar to that of pancreatic cancer. Currently, no definitive radiologic or pathologic features have been established to permit diagnosis of PP without surgical resection. However, the presence of eosinophilic concretions has been reported with increasing frequency in the histologic evaluation of PP. To the best of our knowledge, these concretions are distinctive for PP and not reported in neoplasms commonly involving the pancreaticoduodenal space. Herein, we discuss the case of a 60-year-old man who was found to have PP after pancreaticoduodenectomy for a paraduodenal mass with an initially nondiagnostic biopsy. Retrospective review of the preoperative FNA samples revealed eosinophilic concretions like those found in the final surgical specimen. If the identification of eosinophilic concretions in a background of inflammatory changes was to be accepted as a diagnostic criterion for PP, patients such as ours could be spared the morbidity associated with surgical resection. Paraduodenal pancreatitis (PP) is a rare chronic inflammatory process involving the pancreaticoduodenal space. The presenting symptoms of PP are often similar to those of pancreatic malignancies . While pThe patient discussed herein is a 60-year-old male who presented to the surgical oncology clinic with a one-year history of intermittent abdominal pain. He was previously evaluated by gastroenterology at an outside institution and had undergone a CT scan and an endoscopic ultrasound (EUS). This imaging revealed an approximately 4.5 cm mass between the duodenal bulb and the head of the pancreas with both solid and cystic components. A biopsy was performed during the EUS, but a tissue diagnosis could not be made.At the time of our initial interview, the patient reported persistence of his abdominal pain as well as 15 pounds of weight loss over the preceding three months. He was otherwise asymptomatic. He admitted to occasional smoking and alcohol use. His family history was negative for GI malignancy. On exam, he was noted to be anicteric and nonjaundiced. Abdominal exam was normal aside from a reducible nontender 3 cm umbilical hernia.After the patient's office visit, an MRI was performed for better characterization of the previously identified mass. This showed a 4.4 cm predominantly solid mass in the bulb and second portion of the duodenum with extension into the pancreatoduodenal groove as well as fluid in the right pararenal and paraduodenal spaces Figures . A repeaGiven the remaining diagnostic uncertainty, the patient was discussed in a multidisciplinary fashion at our GI tumor board. It was ultimately decided that the patient should undergo surgical resection for definitive diagnosis. A pancreaticoduodenectomy was performed without intraoperative complication. The patient's postoperative hospitalization was prolonged due to the ileus which was managed with NGT decompression. This appeared to resolve by postoperative day 8 at which point he was discharged. The patient was readmitted two weeks later for severe hiccups and vomiting. He was treated for gastroparesis with erythromycin and Reglan and discharged on hospital day 4. On postoperative follow-up at one and six weeks, the patient reported tolerance of regular diet and freedom from hiccups and nausea.Gross and microscopic pathologic findings of the surgical sample were suggestive of PP Figures  and 3. TParaduodenal pancreatitis is a chronic inflammatory process characterized by fibrotic change within the pancreatoduodenal groove. This is an anatomic space bordered by the C-loop of the duodenum, the head of the pancreas, and the common bile duct –8. This The reported incidence of PP varies significantly in associated surgical reviews of pancreaticoduodenectomy specimens. In a series of 123 surgical Whipple specimens removed for chronic pancreatitis, Stolte et al. identified 30 cases of PP 24.5%) . Becker .5% . BecThe pathogenesis of PP is not well understood. It has been suggested that it may be secondary to chronic inflammation of heterotopic pancreatic tissue in the minor papilla triggered by alcohol use . InvolveRisk factors for PP include alcohol use, peptic ulcer disease, gastric and duodenal resection, pancreas divisum, and duodenal wall cysts . Many ofThe diagnosis of PP requires a high degree of clinical suspicion. Patients with PP are frequently middle-aged men with a history of alcohol use or smoking. They generally have an insidious onset of vague gastrointestinal symptoms. The most common presenting symptoms are postprandial abdominal pain 92%), weight loss (78%), and recurrent vomiting (31%) %, weight.While a definitive diagnosis of PP cannot be made on imaging alone, some radiologic features are suggestive of this disease process. On ultrasound, there is thickening of the medial wall of the duodenum with cystic spaces and a sheet-like hypoechoic area in the groove. CT scan with IV contrast will show hypoattenuation with a delayed enhancement of the pancreaticoduodenal groove, fat stranding in the adjacent tissues, and accumulation of fluid in the right anterior paraduodenal and pararenal spaces. MRI with contrast will show delayed or partial enhancement of the soft tissue occupying the groove, appearing hypointense on T1WI (weighted image) and hyperintense on T2WI relative to the pancreas . A hypovTissue biopsy must be performed in cases of suspected PP to assess for a neoplastic process. Definitive diagnosis is complicated by the fact that the mucosal and submucosal changes are often subtle and nonspecific. The presence of increased numbers of Brunner glands, spindled stromal cells, and prominent nerve bundles may be used to diagnose PP in cytology specimens, especially if repeat FNAs and biopsies are negative for malignancy . HoweverIn cases where it is felt that PP may be diagnosed without surgical resection, treatment is dependent upon the severity of symptoms. For some patients, even if pancreatic adenocarcinoma is not suspected, pancreaticoduodenectomy may still be appropriate. This is true for individuals suffering from advanced sequela such as intractable pain or severe duodenal stenosis . HoweverWith a clinical presentation and radiographic characteristics similar to those of pancreatic and periampullary cancers, paraduodenal pancreatitis possesses a unique diagnostic challenge. Definitive diagnosis often requires formal surgical resection which exposes patients to a relatively high risk of perioperative morbidity for a disease that might otherwise be managed through less invasive means. Surgeons should be aware of this inflammatory process and should discuss the possibility of PP with their pathologists when evaluating patients with a pancreatic groove mass for pancreaticoduodenectomy. In conjunction with imaging findings suggestive of paraduodenal inflammation, the identification of eosinophilic concretions in a background of inflammatory changes may be adequate criteria to confirm PP. While physicians might not presently be willing to diagnose this disease based on such FNA findings, further exploration of those pathologic features may lead to a new standard for PP diagnosis that avoids unnecessary surgical procedures in the future.


Primary oral malignant melanoma (OMM) is a rare malignant lesion with a relatively poor prognosis. The clinical presentation may be nodular, macular, or mixed type, with or without pigmentation. The pigmented lesions in the oral cavity may be either melanocytic or non-melanocytic and neoplastic or non-neoplastic lesions. For all the pigmented lesions of the oral cavity, clinicians must consider OMM as a differential diagnosis. Hence, a histopathological examination is required. Herein, we report a case of pigmented growth over the central arch and gingival mucosa, which turned out to be an OMM. Melanocytes are more commonly found in the skin; however, they are also allocated along the mucosal membrane of the anorectal region, cervix, vagina, nasal cavity, paranasal sinuses, larynx, esophagus, and oral cavity. The most common site for oral mucosal melanoma (OMM) is the upper jaw. Mucosal malignant melanoma constitutes approximately <1% of all melanoma, out of which 10% occurs in the head and neck region . ExposurA 50-year-old male patient presented with the complaint of a painless, blackish growth measuring 2x1.5 cm over the central arch of the mandible for the last six months. No pigmented cutaneous lesions were found. A punch biopsy revealed a diagnosis suggestive of OMM. The computed tomography of the face and neck revealed a lytic lesion measuring 1.9x1.4 cm in the central arch of the mandible. The multiple subcentimetric lymph nodes were seen bilaterally, and the metastatic submental node (1.7x1.5 cm) was also noted. After the radiological evaluation for the extent of the lesion, wide local excision with modified radical neck dissection was done. Grossly, a blackish lesion measuring 3.5x3x3 cm involving the gingivobuccal sulcus was identified. Microscopy revealed a tumor arranged in diffuse sheets and nests composed of oval to spindle cells showing moderate nuclear pleomorphism with vesicular to hyperchromatic nuclei and prominent eosinophilic nucleoli. The intracellular and extracellular pigment was also noted. The tumor cells were immunoreactive for HMB-45, CD117, and BRAF while negative for pan-cytokeratin  Banff workshop recommended to classify the OMM into 1) an in situ pattern, 2) an invasive pattern, and 3) combined pattern of 1 & 2, and they contribute about 15%, 30% and 55% of cases OMM, respectively , 8. The Meleti et al. suggested that surgery was the mainstay of treatment, supplemented by radiotherapy . The surPrimary OMM is a very uncommon malignancy with a relatively dismal prognosis. Because of its aggressive nature, all clinicians dealing with pigmented oral mucosal lesions should raise suspicion of OMM in their differential diagnosis, and adequate biopsy from the lesion is mandatory for early diagnosis and therapeutic intervention.


Extraskeletal osteosarcoma is a malignant tumour composed of an osteoid and/or cartilaginous matrix; it arises in soft tissues without connection to the skeleton, and to our knowledge, this type of tumour is extremely rare.The present study reports a 57-year-old man with primary orbital extraskeletal osteosarcoma who presented with a history of painful swelling in the left orbit that had occurred for 11 months. Imaging of the orbit showed an atypical, well-defined heterogeneous mass attached to the posterior globe of the left orbit. The patient underwent an anterior orbitotomy and complete excision of the tumour. The mass was originated from neither the globe nor the bony orbital wall but from the soft tissue. Histopathology demonstrated an extraskeletal osteosarcoma. After 13 months of follow-up, there was apparent recurrence of the tumour. The medical history showed no complaints of previous trauma or radiotherapy.ESOS is a highly malignant tumour. Immunosuppression, trauma and adjuvant radiotherapy are possible predisposing factors in the development of this tumour. Prompt recognition and thorough treatment are essential for preventing orbital lesions and presence of metastasis from other organs. Extraskeletal osteosarcoma (ESOS) is an extremely rare tumour that is not usually located in the orbit. It is defined as a malignant mesenchymal neoplasm that produces an osteoid and/or cartilaginous matrix and arises in soft tissues without connection to the skeleton. It is rare and comprises fewer than 5% of all osteosarcomas. Only 4 previous cases of orbital ESOS have been reported. Extraskeletal osteosarcoma primarily affects patients over the age of 50 years and has a poor prognosis. Our patient is the oldest and the fourth case ever detected with primary orbital ESOS without a history of previous radiotherapy or trauma. In this report, we describe the clinical, radiologic, and pathologic records of a rare case of primary orbital extraskeletal osteosarcoma.A 57-year-old male presented with painful swelling in the left orbit that had occurred for 11 months. The medical history showed no chronic systemic disease, sinus infection, mucocele, previous trauma, previous eye surgery or irradiation.We performed a complete ophthalmic examination. Best-corrected visual acuity was 20/25 OD and 20/70 OS. Examination of the left eye revealed a 3 mm proptosis and maximal restriction of extraocular movements in all gazes. Funduscopic examination revealed diffuse choroidal fold in the left eye Fig. . Right eComputed tomography (CT) of the orbit revealed an atypical, well-circumscribed homogeneous calcified mass attached to the posterior globe of the left orbit Fig.  a-b. OrbThe patient underwent an anterior inferior orbitotomy, and complete excision of the mass. Intraoperatively, the tumour was attached to the sclera but not attached to any of the orbital structures. The tumour wraps the inferior oblique, lateral rectus, and part of the inferior rectus muscle. The sclera was intact and the surface was slightly rough. The tumour caused indentation of the globe but did not enter the globe. Gross examination revealed an atypical, well-encapsulated grey-brown bony mass with several small, separate, nodules. The cut surface was grey-white, lobulated and bony hard in consistency. Microscopic examination revealed malignant spindle cells with abundant neoplastic bone and cartilage formation Fig.  a-b. ImmThe 13-month follow-up included CT scans every 3 months and subsequently every 6 months. The patient encountered recurrence of the tumour. A complete ophthalmic examination was performed. Visual acuity was 20/25 OD and 20/200 OS. Examination of the left eye revealed a 1 mm proptosis and restriction of extraocular movements in all gazes. Funduscopic examination revealed diffuse choroidal fold in the left eye. Right eye examination was unremarkable. The latest orbital CT Fig.  showed aThe pathological diagnosis was recurrent orbital extraskeletal osteosarcoma.ESOS is a rare malignant mesenchymal neoplasm, which located in soft tissue without connection to the skeleton. Only 4 cases of ESOS in the orbital region have been reported in the literature Table  1–4]. S–4. S1–4]Research focusing on the clinical behaviour of ESOS is limited. Localized pain, swelling and oedema are the most common presenting symptoms . The durIn our case, the differential diagnosis should be as follows:(1) choroidal osteoma,(2) mesenchymal chondrosarcoma. Choroidal osteoma has not been suggested because the imaging studies showed only the limited lesions of the choroid with the presence of cancellous bone and vascular channels within the choroid, and this type of tumour commonly occurs in healthy females in their 2nd or 3rd decades of life. Without the component of cartilage and accordant pathological examination, the diagnosis of mesenchymal chondrosarcoma was not supported.The most common genetic changes associated with osteosarcoma are the alterations of the p53 tumour suppressor gene and loss of the retinoblastoma gene RB1on the chromosome . In the The common metastatic sites of involvement are the lungs, followed by the lymph nodes, kidneys, thyroid, liver, spleen and other viscera . The treIn summary, ESOS is a highly malignant tumour. If a patient shows the orbital signs of suspicious of ESOS, we recommend that a diagnosis should be promptly performed, and appropriate surgery in terms of excision is a prerequisite. Patients should be carefully monitored with very close follow up and should be warned about symptoms and signs of recurrence after surgery, which can rapidly progress and cause severe visual impairment. Prompt recognition and thorough treatment are essential for preventing orbital lesions and presence of metastasis from other organs.