Chilaiditi syndrome is a rare disorder comprising of the interposition of the gut between the diaphragm and liver. This can lead to a spectrum of gastrointestinal and respiratory presentations, primarily in the elderly population in whom the disorder is relatively more prevalent. We present a case of a 63-year-old man who presented to our setup with abdominal pain and shortness of breath and later got diagnosed with Chilaiditi syndrome. He was managed conservatively and showed complete resolution of the symptoms. Abdominal pain in an elderly patient can present in a variety of ways. Chilaiditi syndrome is one such presentation in which the bowel gets interposed between the liver and diaphragm, causing a range of symptoms that can easily be confused with numerous other medical diagnoses. Radiographically, it can be confused with pneumoperitoneum and thus, create challenges in medical decisions . Here, wA 63-year-old elderly man, with a known case of hypertension and chronic bronchitis, presented with progressive abdominal pain in the epigastrium and right upper quadrant for the last eight hours. The pain was continuous, moderate in intensity, dull and achy in nature. He reported no shifting or radiation of pain but did complain of constant nausea and abdominal fullness that began a few hours after the onset of pain. The patient had also vomited three times before the current presentation; vomitus was nonmucoid, non-bloody, nonbilious, non-projectile, and watery in composition with no associated retching. Post-admission, the patient also complained of being short of breath while ambulating towards his bed. He had no preceding history of altered bowel movements, fever, trauma or similar prior episodes.On admission, the patient was fully responsive, alert, and oriented with a normal affect but appeared to be in obvious distress. He was afebrile with a pulse of 87 beats per minute (BPM), blood pressure (BP) of 135/89 mmHg, and respiratory rate of 18/min. On examination, his abdomen was marginally distended, tympanitic, mildly tender to deep palpation more pronounced in the epigastrium and right upper quadrant with normoactive bowel sounds and no hepatosplenomegaly. Pulmonary auscultation revealed bilateral audible breath sounds with bronchovesicular breathing and added rhonchi which cleared on coughing. Oxygen saturation was 97%. Neurological exam for bulk, tone, power, and reflexes was insignificant. The sensory and cerebellar system were also intact. Cardiovascular exam was unremarkable with regular rate, rhythm, and no extra heart sounds or murmurs. No cyanosis, edema, conjunctival pallor, jaundice, or clubbing were appreciated.Appropriate lab workup was done, which included: complete blood count, liver function tests, basic metabolic panel, and random blood sugar levels. The obtained results were insignificant with normal values except for a marginally low potassium level of 3.2 mEq/L . Erythrocyte sedimentation rate and C-reactive protein levels were also within normal limits and so was the Coagulation profile.Due to the concomitant presentation of abdominal pain and shortness of breath during ambulation; basic imaging studies including chest radiograph, and abdominal ultrasound were requested. Chest radiograph revealed gas between the liver and right-sided hemidiaphragm, which raised concerns for pneumoperitoneum Figure .Ultrasound (US) abdomen showed gas echoes in hepato-diaphragmatic space; echoes did not change location despite altering the patient’s position while imaging. This was a point of concern for the primary team due to this being a rather atypical presentation for pneumoperitoneum. The patient was given nothing by mouth during the initial phase, and intravenous fluids were administered to maintain euvolemia while awaiting computed tomography.Computed tomography (CT) of the abdomen was obtained to further resolve the mystery of subdiaphragmatic gas, which could either be free or intraluminal. CT displayed interposition of large intestinal loops between the diaphragm and the liver with no free intraperitoneal air or fluid. There were no signs of volvulus, ischemic colitis or perforation Figures -3.Based on these explicit findings, negating pneumoperitoneum, a diagnosis of Chilaiditi syndrome was consensed.The patient was aborted from an initially planned laparoscopy and rerouted to conservative measures: he was initiated on metoclopramide, enemas, and laxatives. Bowel decompression was started with a nasogastric tube attached to intermittent suction and a rectal tube attached to a gravity drainage system. Post-decompression radiograph was obtained which did not reveal air under the diaphragm. The patient gradually responded to conservative measures and a weekly follow-up documented complete resolution of symptoms.Chilaiditi syndrome is one of the rarest intestinal diseases, comprising of the interposition of the colon or small bowel in hepato-diaphragmatic space. It was first documented back in 1865 and it presents with a wide array of gastrointestinal symptoms .Chilaiditi sign is incidentally found in 0.025% to 0.28% of chest and abdominal X-ray films and around 1.18% to 2.4% of abdominal computed tomography (CT) scans. The frequency of the sign occurring in males is four times that of females. Though the sign has been reported in the age bracket of five months to 81 years, it is more evident in aged individuals where the incident rate is around 1% [Normally, suspensory ligaments of the liver, mesocolon, falciform ligament and liver itself are anatomically positioned in such a way that the area around the liver is minimized and this restricts interposition of the colon. Chilaiditi syndrome, however, can occur due to various congenital and acquired causes: congenital factors include the absence of suspensory or falciform ligaments, redundant colon, paralytic right hemidiaphragm, malposition, and dolichocolon while acquired factors include multiple pregnancies, obesity, liver pathologies such as cirrhosis and atrophy, ascites, liver ptosis caused by loosening of ligaments. Other risk factors include pathologies of intestine like megacolon, absence of peritoneal attachments, volvulus, meteorism, and colonic hypermobility. Diaphragmatic abnormalities such as phrenic nerve injury and intrathoracic pressure changes as seen in cases of chronic obstructive pulmonary disease (COPD) may also contribute to the pathogenesis of Chilaiditi syndrome -4.Anatomically, Chilaiditi sign is classified as anterior or posterior, depending upon the positioning of interposed bowel with respect to the liver. Most commonly affected bowel segments are the hepatic flexure, ascending colon and transverse colon. However, a few cases also reported the involvement of small bowel, either with or without colon . ClinicaIdentification of Chilaiditi sign is considered extremely significant prior to certain procedures. Its recognition helps in preventing the risk of bowel perforation during percutaneous transhepatic interventions or liver biopsies, especially in cirrhotic patients. Studies have shown that Chilaiditi sign also complicates colonoscopies due to continuous entrapment of air in the interposed bowel segment which increases intraluminal pressure and may eventually lead to perforation. It has, however, been observed that administration of carbon dioxide as an insufflating agent reduces the risk of bowel perforation .Treatment of Chilaiditi syndrome varies according to the severity of the clinical picture. Asymptomatic patients with Chilaiditi sign often do not require any intervention. Mild to moderate cases are managed conservatively which entails bed rest, nasogastric decompression, intravenous fluids, enemas, analgesia and introduction of high fiber diet and stool softeners. CT scan is routinely repeated which should correspond with clinical improvement and show resolution of the interposition. Surgical management is reserved for patients who do not respond to conservative measures or develop complications like perforation, bowel ischemia, cecal or colonic volvulus, subphrenic appendicitis, and internal herniation. Surgical interventions range from fixation of the gut to colonic resection; depending upon the length and condition of the involved bowel segment. Colopexy is recommended for uncomplicated cecal volvulus while resection is mandated for gangrene and perforation. However, colonic resection is the best intervention for resolving colonic volvulus. Since colonic volvulus has about 16% risk of gangrene development, colonoscopic reduction is not recommended ,7.Chilaiditi syndrome is an unusual manifestation of intestinal obstruction caused by the interposition of the gut between the liver and diaphragm. Abdominal pain with atypical findings of subdiaphragmatic gas in Chilaiditi syndrome can lead to incorrect and drastic decisions in the treatment of the patient. Hence, it is crucial for physicians dealing with the geriatric population to be vigilant of such findings and utilize CT scan to reach the diagnosis before proceeding to reckless laparoscopic explorations. Conservative measures, including bowel decompression, should be considered as the primary aim of treatment in patients with Chilaiditi syndrome.


Adenoid cystic carcinoma is an uncommon malignant neoplasm of the salivary gland.Liver metastasis from salivary gland cancer is a rare situation. In thisarticle, we report the case of a 29-year-old woman treated 5 years previouslyfor adenoid cystic carcinoma of the parotid gland by surgery and radiotherapy,who presented for a large hypervascularized hepatic metastasis of 20 cm. After3-cycle chemotherapy stability, hepatic surgery was successfully performed. Thepatient maintained disease-free period of 12 months after the surgicaltreatment. This rare case represents a therapeutic challenge for oncologists andsurgeons. Through this case and a review of the literature, we try to betterdetail the management of this uncommon entity. Furthermore, we review the literature concerning themanagement of liver metastases from ACC of the parotid gland.A 29-year-old woman was treated in 2013 for ACC of the parotid (T3N0M0). She hadparotidectomy of the left parotid gland with ipsilateral selective lymph nodedissection level II to IV preserving the facial nerve. The treatment was followed byexternal beam irradiation to the parotid area and the neck. Afterward, she wasregularly followed-up in our consultation. Five years after primary surgery, thepatient consulted with pain in the right hypochondrium. In the clinical examination,the patient was anicteric, and abdominal examination found an irregular mobileconsiderable tumefaction of the left liver. There was no recurrence in the surgicalsite and neck.A computed tomography (CT) scan revealed a single liver lesion that was suspicious ofmetastasis . TheCT Magnetic resonance imaging of the liver affirmed a solitary liver lesion. It was aheterogeneous, large mass measuring 21 × 9 cm with a contrast agent enhancement ofthe left lobe of the liver that was suspicious of metastasis. A CT-guided needlebiopsy of the mass was done and revealed a metastatic ACC of the parotid. Amultidisciplinary staff decision was made to perform chemotherapy to down-size thelesions, followed by hepatic metastasectomy. The young age and the presence of asingle metastatic localization were the arguments justifying the hepatic surgery onour patient.After 3 cycles of Navelbine-CDDP-based chemotherapy and in view of the stability ofthe lesion objectified on the CT scan, we decided to do a chemo-embolization, whichfailed due to extensive vascularization and of the technical difficulties.After an evaluation of liver function, calculated at 40% by volumetric CT, thepatient had a laparotomy and we found a voluminous exophytic tumor. It was whitegrayish color measuring 21 × 19 × 12 cm. The mass was appended to the liver segment surface. Wedge resection of the tumor while keeping sufficient liverparenchyma was done. Intraoperative ultrasound was performed and revealed no morelesions.The patient had no postoperative complications. There was no liver dysfunction, andshe left the hospital on the 15th postoperative day in good health condition.A histologic examination showed that the tumor measured 21 × 19 × 12 cm withdisease-free surgical margins. The microscopic appearance showed that the tumor massis pushing out toward the hepatic tissue. The tumor was organized in cribriformgrowth pattern displaying several prominent pseudocysts surrounded by basaloid cellswith hyperchromatic-angulated nuclei. These pathological findings are suggestive ofmetastases from the ACC  and D.At the 6-month postoperative follow-up examination, magnetic resonance imaging of theabdomen showed that the patient is currently disease-free. One year after thesurgery, she has regularly been followed-up at the consultation and has no signs ofrecurrence.2 Distant metastases most frequently were localized in the lung, followed bybone, liver, skin, breast, and, rarely, the brain.35Usually ACC has a slow growth. It is characterized by the frequency of localrecurrences and a high rate of distant metastases.1 Generally, metastases from ACC occurred many years after the diagnosis of theprimary tumor6 and could persist asymptomaticfor an extended period.5The occurrence of liver metastases is often synchronous or metachronous withmetastasis to other organs like the lung.79According to our current knowledge, only 3 cases of isolated liver metastasis thatpresented as the first clinical sign from parotid ACC were reported.10Few studies of isolated metastatic liver were reported in the literature. Theirmanagement was still debatable. The role of surgery was not clear and some studyshowed no benefit on overall survival.Often, it is hard to show whether liver resection could provide a survival benefitbecause of the frequent association of liver metastases with other metastaticsites.Our patient was young, and she had developed a unique metastasis in her liver 5 yearsafter the diagnosis of ACC of the parotid. Because of the large size of this tumor,chemotherapy was tried initially. However, it did not have any effect. Surgery wasthe only possible treatment, despite the high risk to which she was exposed.10 that were similar to ours.Both cases had isolated liver metastases and showed resistance to chemotherapy asfirst-line therapy.We found in the literature 2 cases2 the patient had multiple liver metastases with a low response tochemotherapy, so a right extended hepatectomy after a portal vein embolization wasdone. The patient was disease-free for 18 months before the final recurrence.In the case first reported by Balducci et al,10 described the second case of liver metastases, as the initial sign of an ACCof the left submandibular gland. She had multifocal liver lesions and showedresistant to systemic chemotherapy. She received a combination of adoxorubicin-eluting bead chemoembolization, intraoperative and percutaneousradiofrequency ablation, and radiofrequency-assisted surgical resection. At 1-yearfollow-up, the patient had no recurrence.Karatzas et al11 reported one case of recurrent liver metastasis that was efficiently achievedby a laparoscopic approach. The patient remained disease-free for 1 year after thesurgical resection.Scuderi et al12 Due to the slow growth rate ofACC, the role of chemotherapy, in a metastatic situation, is stillinefficient.14 However, surgical excision, with adequate margins, remains theonly possible therapeutic option.5Unfortunately, considering the rare cases of isolated liver metastases, it isdifficult to conclude whether liver metastasectomy could be advantageous. Somestudies had proposed no further treatment (radiotherapy or chemotherapy) whenasymptomatic distant metastases were detected.To conclude, liver metastasis from salivary gland cancer is an uncommon situation.Due to the absence of consensus concerning the appropriate treatment, amultidisciplinary approach was necessary to manage this rare and aggressiveneoplasm.Surgical resection of the metastasis is recommended particularly when metastasis issolitary in order to improve survival.


Although uncommon and seldom experienced, intestinal perforation is a well-known complication of Hirschsprung’s disease (HD). A literature review revealed that the cecum, including the appendiceal base, is a site of perforation. The cecum is not suitable for making an ordinary loop colostomy, and the optimal operative strategy remains to be established.We present a combination technique composed of tangential cecostomy at the perforated portion and postoperative care with a transanal indwelling tube, which was used in the treatment of a 3-day-old boy with cecal perforation with long-segment Hirschsprung’s disease. A temporary simple blowhole stoma and continuous decompression with daily irrigation via a transanal indwelling tube in the distal colon achieved a secure recovery and was followed by a definitive operation in the early period. The combination of tangential cecostomy and transanal indwelling catheter management led to the preservation of the ileocecal valve.We review the Japanese literature and emphasize the usefulness of this combination technique by blowhole tangential cecostomy and transanal tube insertion for neonatal cecal perforation in patients with HD in today’s early definitive operation era. Hirschsprung’s disease (HD) is a common cause of distal bowel obstruction in the neonatal and infant period. Perforation of the intestine secondary to overdistention and/or enterocolitis is a well-known complication of HD. However, the actual occurrence of perforation in patients with HD is rare, and the incidence of intestinal perforation in HD was previously reported to be approximately 4% , 2.A literature review revealed that the cecum, including the appendiceal base and appendix, is a frequent site of perforation. The cecum appears to be unsuitable for making an ordinary loop colostomy; thus, the optimal operative strategy remains to be established.The authors herein report a case of cecal perforation in a 3-day-old male neonate with HD who was successfully treated with a temporary tangential cecostomy on the perforated site and management with an indwelling transanal tube, which enabled stable postoperative care followed by a definitive operation at 1 month of age.This case is reported to highlight this surgical strategy of choice for cecal perforation in neonates with HD, which is especially useful in the earlier definitive operation era.A 2-day-old boy born at 40 weeks of gestation was admitted to our hospital due to repeated vomiting and abdominal distension. His birth weight was 2850 g. A blood test at admission revealed an elevation of the patient’s WBC count (26600/μL) and CRP (7.4 mg/dL) and lactate 18 mg/mL) levels. An X-ray photography showed dilatation from the ascending colon to the transverse colon. At this point, we doubted Hirschsprung’s disease-associated enterocolitis, and we started intravenous antibiotics therapy. Gastrografin contrast enema on the same day showed a caliber change in the transverse colon , and ileum (n = 5). There were no patients with appendiceal perforation in this review. Various surgical procedures were selected, including ileostomy (n = 6), colostomy (n = 9), ileocecal resection and anastomosis (n = 3), and tangential cecostomy .After excluding cases of duodenal atresia, the mean gestational age, birth weight, and duration of perforation in 23 patients were 38.3 ± 1.6 SD weeks, 2999 ± 553 SD g, and 3.0 ± 1.8 SD days after birth. The most common sites of perforation were the cecum, ascending colon (Newman et al.  warned tIn the present case, we performed cecostomy. Because the remarkable caliber change was recognized at the mid-transverse colon, we were sure that the real transition zone was located in the ascending or right transverse colon. In most reported cases of cecal perforation, colostomy at the perforated site or closure of the perforated site with ileostomy was selected. Staged definitive surgery has been performed with intraoperative leveling biopsy and stoma closure several months later. However, management and closure of the colostomy at the ascending colon or the ileocecal region seemed to be more difficult than tangential cecostomy. In some cases, a sacrifice of a segment of the ganglionic colon and/or ileocecal valve was required due to stenotic change near Bauhin’s valve and ileo-anal anastomosis was reluctantly performed at the time of the definitive operation.Although the pathogenesis of intestinal perforation in HD is still unknown, most studies have proposed that inflammation may play a major role in its development at the site of bowel obstruction. In their review, Newman et al. reported that the mechanism of perforation appears to be directly related to increased luminal pressure from distal obstruction, as only a few cases showed signs of enterocolitis related to the perforation .It was previously reported that the Law of Laplace dictates that the intraluminal pressure needed to stretch the wall of a hollow tube is inversely proportional to its radius; thus, the tension required to distend a hollow tube is lowest at the widest point. Clinically, this explains why the cecum is the most common site of perforation in cases of distal large bowel obstruction , 4.During emergency laparotomy, the pediatric surgeon should be aware of this possibility and the cecum should be inspected for perforation at the time of surgery.Cecostomy is a useful and less invasive surgical procedure for patients presenting with perforated cecum due to colonic obstruction; however, it is less frequently used and considered to be one of the oldest operations in surgery. In contrast to loop colostomy, a tangential-type stoma can act as a decompression and diversion window but cannot accomplish the complete division of the fecal stream. A transanal indwelling tube can manage the inflow feces and gas into the distal aganglionic bowel until definitive surgery –7. HirosA recently published series suggests that the current era is characterized by earlier definitive surgery with increased utilization of laparoscopic and transanal approaches –11. The The incidence of neonatal gastrointestinal perforation in patients with HD is thought to be decreasing. However, we hope that cecostomy and catheter management will be a helpful approach in the current early definitive operation era. The blowhole cecostomy procedure at the site of perforation may still be indicated for selected cases of colonic perforation in patients with HD.


Pineoblastomas (PBs) are rare and aggressive malignancies of the pineal gland. They are more commonly diagnosed in children between 1-12 years old, and are very rarely diagnosed in adults. For this reason, evidence in literature for adults is scarce and mainly derives from the paediatric practice. For their clinical behaviour and embryonal histology, PBs are often grouped together with medulloblastomas in clinical trials. In this report, we describe an adult PB case who was treated at our institution. We reference the literature to explain the clinical reasoning behind our decision-making process.A 46-year-old male patient was referred to our institution in November 2015 with three months history of headache. Imaging confirmed localised disease of the pineal gland. He underwent surgery which was radical and clinically uncomplicated. Histology showed PB. He then received adjuvant craniospinal radiotherapy with a boost to the tumour bed followed by consolidation chemotherapy. After 36 months follow-up, he remains disease-free without significant toxicities.Surgery followed by craniospinal irradiation and consolidation chemotherapy can be a safe and effective treatment option in adult PBs. The most common malignancies which arise from the pineal gland are germ cell tumours, pineal parenchymal tumours and gliomas. Pineal parenchymal tumours are more common in the paediatric population, although accounting for only 0.5% of all central nervous system (CNS) tumours in adults . They reTraditionally, pineal tumours fell into the broader category of CNS supratentorial primitive neuroectodermal tumours (SPNETs). SPNETs are rare intra-axial brain tumours which have the potential to differentiate along multiple cell lines.The treatment of PB mainly derives from the paediatric practice and chemotherapy and craniospinal irradiation (CSI) are often advocated, for their propensity to disseminate along the cerebrospinal fluid (CSF). The role of radiotherapy is not clearly defined in adults PBs due to the low incidence in this age group thus preventing the adoption of standardized strategies. Aim of this report is to describe a successful adult PB case treated at our institute.A 46-year-old male patient was referred to our institution in November 2015 with three months history of headache. At the time of admission, a plain head CT scan showed an enlarged pineal gland with smooth margins and several peripheral calcifications. The pineal parenchyma appeared hyperdense to cerebral cortex because of a possible haemorrhagic component. The brain magnetic resonance (MR) showed a well-defined lesion in the pineal region , responsible for a slight impression on the tectal plate underneath and minimal internal cerebral veins dislocation along the top. The lesion was iso-hyperintense on T1  with no evidence of further bleeding. The neuro-radiological hypothesis favoured a growing pineal parenchymal tumour. Therefore, the patient underwent craniotomy and maximal safe resection of the tumour. Despite the deep location of the lesion in the pineal region abutting the superior quadrigeminal plate and the close proximity to internal cerebral veins, macroscopical complete resection was achieved. The patient fully recovered after surgery and neurological examination was unremarkable.Histopathology showed PB (Grade IV WHO 2016 Classification) with MIB-1 index of 27% [The patient was then referred to our unit for clinical evaluation and consideration of adjuvant treatment. The treatment strategy agreed during multidisciplinary tumor board was to give adjuvant radiotherapy followed by consolidation chemotherapy. Therefore, the patient underwent a CT scan for radiotherapy (RT) planning purposes and was started on external beam RT using tomotherapy. He received 36 Gy in 18 fractions (2 Gy per fraction over four weeks) to the neuraxis technique according to ICRU 83 recommendation, prescribing 100% of the dose to the median of the volume, making sure that the 95% isodose covers at least the 98% of the volume and the 107% isodose does not exceed 2% of the volume . The CHTAt present, after 36 months follow-up, he remains disease free. Literature evidence suggests that relapses in older patients tend to occur after a median time of 18 months and therefore 36 months follow-up was considered adequate in our case .PBs are very unusual in adults and this has prevented randomised controlled trials (RCT) from being undertaken in this age group. For this reason, the evidence in literature is meager and mainly based on small retrospective series. As said, for similarities in the embryonal histology and in the routes of spread, the current practice tends to follow the treatment philosophy of high risk medulloblastomas, their infratentorial counterpart.For instance, the National Comprehensive Cancer Network (NCCN) guidelines do not distinguish between medulloblastomas and SPNETs and their recommendations are common for the two groups. Multimodality treatment comprising surgery and various combinations of CHT and RT is suggested .The National Institute for Health and Clinical Excellence (NICE) guidelines in the United Kingdom highlights the importance of establishing a national pineal tumour group to unify management approaches, develop national standardised guidelines and treatment protocols, and determine research programs .Mynarek et al. run a retrospective analysis of 135 cases collecting data from 11 international brain tumour groups . They deRole of surgeryGross total resection (GTR) of the primary tumour together with CSF diversion when needed, should be used upfront in PBs, regardless of patients’ age. In fact, about half of the patients present with symptoms of raised intracranial hypertension (IH) for CSF obstruction at the third ventricle level and consequent hydrocephalus. Surgery relieves symptoms of IH, provides tissue for histological diagnosis and reduces tumour burden. However, it is associated with significant morbidity and mortality and its complications can adversely affect quality of life. These include Parinaud syndrome, ptosis, hearing loss, quadriparesis, thalamic infarcts and even death . The proRole of radiotherapyThere is general consensus in literature that adjuvant RT should be considered standard (category 2A in the NCCN guidelines) . NeverthThe prognostic significance of RT dose has been outlined in literature, with several authors reporting better outcomes with higher RT doses. Schield et al. concluded that patients with PB have a high risk of spinal failure and should receive 30-45 Gy on the cranio-spinal axis with a prophylactic intent and at least 50.4 (up to 54 Gy) Gy on the primary tumor . Lee et When it comes to the technique, standard conformal X-ray techniques have been and still are widely used for craniospinal irradiation. However, it can be challenging to achieve satisfactory dose coverage of the target including the area of the cribriform plate, while sparing organs at risk. Moreover, another problem with the standard techniques is the matching of the fields, which leads to “migrate” the junction during each session of the radiotherapy treatment in order to reduce the possibility of having overdosed regions . For thiRole of chemotherapyThe role of chemotherapy is unclear for adult patients and mainly derives from that of medulloblastomas. A Phase III trial which enrolled 421 standard risk medulloblastomas reported an encouraging five-year survival rate of 86% in patients receiving concurrent weekly Vincristine and adjuvant Vincristine-Lomustine-Cisplatin . The samThis case is an aggressive malignancy of the central nervous system which presented in an uncommon age group of the fifth decade. Despite the scarcity of literature evidence, the current case successfully demonstrates that aggressive surgery, whenever is technically feasible, followed by adjuvant RT and consolidation CHT may be the optimal strategy. Better understanding of biological features could lead to the identification of subgroups for improved risk stratification of patients and more targeted treatments, similarly to what happened in medulloblastoma during the past decade.


Insulinoma is a rare tumour representing 1–2% of all pancreatic neoplasms and it is malignant in only 10% of cases. Locoregional invasion or metastases define malignancy, whereas the dimension (> 2 cm), CK19 status, the tumor staging and grading (Ki67 > 2%), and the age of onset (> 50 years) can be considered elements of suspect.We describe the case of a 68-year-old man presenting symptoms compatible with hypoglycemia. The symptoms regressed with food intake. These episodes initially occurred during physical activity, later also during fasting. The fasting test was performed and the laboratory results showed endogenous hyperinsulinemia compatible with insulinoma. The patient appeared responsive to somatostatin analogs and so he was treated with short acting octreotide, obtaining a good control of glycemia. Imaging investigations showed the presence of a lesion of the uncinate pancreatic process of about 4 cm with a high sst2 receptor density. The patient underwent exploratory laparotomy and duodenocephalopancreasectomy after one month.The definitive histological examination revealed an insulinoma  with a low replicative index (Ki67: 2%).This report describes a case of malignant insulinoma responsive to octreotide analogs administered pre-operatively in order to try to prevent hypoglycemia. The response to octreotide analogs is not predictable and should be initially assessed under strict clinical surveillance. Insulinoma is a rare tumour representing 1–2% of all pancreatic neoplasms . The dimIn affected patients, the control of glycemia before surgical excision, or for a prolonged time if surgery is not feasible, may be very problematic. Admittedly, the description of new cases may be of help for clinicians, who have to deal with similar situations. We report a case of malignant insulinoma associated with local infiltration, neoplastic thrombosis and lymph node metastasis, in which medical treatment with octreotide effectively counteracted hypoglycemia before surgery. One peculiarity of this case is represented by the fact that short acting octreotide was used, in agreement with the surgeon, in order to avoid any possible pharmacological interference caused by long acting formulations at the time of surgery, which was performed shortly after the diagnosis.A 68-year-old man presented a weight increase of 7 kg during the last year and symptoms compatible with hypoglycemia . During some of these episodes low blood glucose levels (< 40 mg/dl) were documented by glucometer measurement. The symptoms regressed with food intake. These episodes initially occurred during physical activity and later also during fasting.He had no family history of endocrine disease.2. The rest of the physical examination was unremarkable. Biochemical assessment did not show any abnormality, and glucose level was 70 mg/dl (n.v. 65–110). Plasma cortisol at 8 a.m. was in the normal range , anti-insulin antibodies were negative, chromogranin A was 69 ng/ml (n.v. 10–185), prolactin was 247 mU/l (n.v. 53–369), gastrin was 12.7 pg/ml (n.v. < 180), PTH was 6.4 pmol/l (n.v. 1–6.8) and serum calcium level was 8.8 mg/dl (n.v. 8.6–10.4).At admission, his body mass index was 28 kg/mThe fasting test was performed, which was interrupted after 12 h due to the onset of symptomatic hypoglycemia . Plasma glucose level was 41 mg/dl, insulin level 16.3 U/L and C-peptide 1.27 nmol/l  density Fig. .Abdominal CT scan with contrast, which revealed a polylobed lesion with sharp margins at the level of the uncinate pancreas process, in contact with the superior mesenteric vein and reaching the margins of the superior mesenteric artery. No signs of vessels infiltration were observed.Additional procedures included:All these imaging procedures were indicative of the presence of an insulinoma as the cause of endogenous hyperinsulinemia.Surgical treatment was scheduled. Before surgery, considering the response to the short octreotide test, the patient was treated with short acting octreotide (0.1 mcg twice a day), obtaining a good control of glycemia by glucometer measurement, disappearance of hypoglycemic symptoms and no recurrence of hypoglycemic episodes.The patient underwent exploratory laparotomy and duodenocephalopancreasectomy after one month.The postoperative course was complicated by the appearance of a pancreatic (grade B)  and a biThe definitive histological examination revealed a neuroendocrine tumor with insulin receptors (insulinoma) of 3.5 cm infiltrating the duodenum, retroperitoneal adipose tissue, with widespread neoplastic thrombosis, and with metastasis in 3 pancreatic-duodenal lymph nodes among the 48 that had been surgically removed , with a low replicative index (Ki67: 2%); radical resection (R0). Vascular and adipose tissue infiltration by the tumor, which are indicative of malignancy, were observed. Immunohistochemical staining was positive for insulin and synaptophysin (Fig. The most recent abdominal MRI, performed 6 months after surgery, did not reveal any recurrence of disease and currently the patient is in good conditions and with normal glycemic levels.Clinical hypoglycemia occurs when plasma glucose concentration is low enough to cause symptoms and/or signs, which include neurological alterations. The clinical feautures of hypoglycemia are non specific and it is not possible to establish a single plasma glucose concentration that definitively confirms clinical hypoglycemia. Therefore, hypoglycemia is confirmed by the documentation of the Whipple’s triad , e.g. syThe most common cause of hypoglycemia is represented by insulin, insulin secretagogues, alcohol abuse and drugs of different classes . HypoglyHypoglycemia may be caused by hyperinsulinism in the absence of prior gastric surgery or after Roux-en-Y gastric bypass for obesity . It can Although rare, insulinoma is the most common neuroendocrine tumor of the pancreas with an annual incidence of four in every 1 million persons. Malignancy is observed in only 10% of cases . The cli111In-DOTA-exendin-4 administration is another non-invasive diagnostic approach that may succesfully localize small insulinomas pre- and intraoperatively [68Ga-DOTA-somatostatin analog PET/CT, because it is largely superior to somatostatin receptor scintigraphy, and facilitates the diagnosis of most types of NET lesions [Non-invasive imaging procedures, such as CT and MRI, are used when a the biochemical diagnosis of primary hyperinsulinism has been made. Invasive modalities, such as endoscopic ultrasonography and arterial stimulation venous sampling, have frequently been shown to be superior to non-invasive localization techniques to preoperatively localize insulinomas . Numerouratively . Very re lesions . In our Surgery is the first choice therapy for resectable insulinomas. A pharmacological approach can be useful both during the preoperative period, and for preventing hypoglycaemia in insulinomas with unknown localization. Diazoxide can prevent hypoglycemia by suppressing the release of insulin from insulinoma cells via opening ATP-sensitive potassium channels . The useIt has to be said that the response to somatostatin analogs may differ according to the presence of various subtypes of sstR on insulinoma cells. Octreotide binds predominantly to sstR subtype 2. The absence of these receptors on insulinoma cells may aggravate hypoglycemia when a patient is treated with octreotide. This effect may be due to the inhibition of contra-insular hormones such as growth hormone and glucagon by somatostatin , 33. TheIn summary, we have described a rare case of malignant insulinoma in a patient with recurrent hypoglycemic episodes. The patient was successfully treated with short acting octreotide analogs before surgery, after testing its efficacy on glycemic control. No disease recurrence was observed at 6 month after surgery and the condition of the patient is currently very satisfactorily.


Carotid body tumors (CBT) are rare neoplasms with relatively; due to their malignant potential, the indicated treatment is surgical resection. There have been described some associations of carotid body tumors with other neoplasms, however, the concomitant presentation of CBT with multinodular goiter is rarely encountered.We herein present the case of a 43-year old woman with the aforementioned association. As a part of the diagnostic evaluation, an ultrasound was performed, revealing both conditions simultaneously. The surgery took place with the aim of resecting both lesions during the same intervention. The patient was discharged without complications.The concomitant presentation of goiter and carotid body tumor is rarely reported but an extended Kocher incision could be enough to an adequate exposure and same surgical procedure resection.This case reinforce the concomitant assessment in two different pathologies. Although there is one case reported before, this case lead to improvement in the treatment of these patients. Carotid body tumors are rare neuroendocrine tumors originating from the sympathoadrenal and parasympathetic paraganglia of the autonomic nervous system. The incidence of this tumors is estimated to be of 1:30,000 to 100,000 in the general population [Thyroid nodules represent a common clinical problem, nonetheless only 5% of them are palpable, while incidental diagnosis with a cervical ultrasound is thought to be over 70%, with a greater prevalence in elderly patients . While mThe association of multinodular goiter with carotid body tumors or paraganglioma, is a rare form of presentation; with very few cases reported in the literature . The treA 43-year-old woman was referred to our institution with a neck mass in the left submandibular region. She was studied in an outside hospital, where a neck ultrasound showed a multinodular goiter and a CBT on the left carotid bifurcation. A neck Computed tomography angiography (CTA) demonstrated a carotid body tumor (3.8 cm × 2.5 cm × 3.3 cm) classified as Shamblin II and an intrathoracic multinodular thyroid gland with a right lobe extension of 11.9 cm × 9.7 cm and the left lobe of 25.2 cm × 21.3 cm with caudal retrosternal growth and evidence of slightly trachea deviation .Fig. 1GoThyroid function test (TFT) were normal, serum antithyroglobulin antibodies and thyroperoxidase antibody (TPO) were undetectable. Thyroid gammagram was performed reporting a multinodular goiter. Fine needle aspiration was performed demonstrating nonmalignant cells. A total thyroidectomy was performed with a transverse lower neck incision (Kocher incision), posteriorly, CBT was resected by an extension of the previous Kocher incision to the anterior border of the sternocleidomastoid muscle using the retrocarotid reported previously in our group as an effective technique, also two surrounding lymph nodes were resected to rule out malignancy . The patThe patient recovered satisfactorily after the surgery and she was discharged in postoperative day number three, she currently continues her follow-up as our patient. Twelve months after the surgery, she does not present any complaints. She is currently on levothyroxine and calcium carbonate.Neck lymphadenopathy or tumors evaluation require a careful medical history and a thorough physical examination of the patient. Multiple causes of volume increase in the neck have been reported previously; within most common causes, we could divide into 4 different groups: 1) infections , 2) malignancies (hematologic and solid tumors), 3) immunologic disorders  and 4) miscellaneous (Lymph node hyperplasia) . MultinoMost patients with multinodular goiter have few or no symptoms except for those with large goiters. Many patients are referred to the hospital for cosmetic reasons or, more frequently, for compression of cervical structures causing tracheal deviation, shortness of breath, cough, voice changes, odynophagia and dysphagia ,16. WhilOn the other hand, the clinical presentation of carotid body tumors differs depending on their size. Carotid body tumors classically present as asymptomatic enlarging lateral neck masses  and mostIn conclusion, the association of paraganglioma with multinodular goiter is rare and has a good prognosis according to few cases reported in the literature. Surgery is the treatment of choice, and resection of both lesions in a single procedure is feasible in most cases without increasing morbidity. In this particular case, a left extended cervical incision allowed successful removal of both tumors without any complication.The authors have no conflict of interests.No sponsors involvedThis case is exempt from institutional ethnical approvalWritten 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".Ramon Garcia-Alva, Data collection, writing the manuscriptLuis Bobadilla Writing the manuscript.Luis H Arzola Writing the manuscript, data collection.Monserrat Escobar-Preciado, Data collection.Javier E. Anaya-Ayala, Critical review of the article.Carlos A. Hinojosa Conception and design. Critical review of the article.Not requiredDr. Carlos A. Hinojosa,Professor of Vascular SurgeryNot commissioned, externally peer reviewed.


Histiocytic sarcoma (HS) is a very rare malignant histiocytic derivation cancer. The extranodal multisystemic HS has an aggressive clinical course and poor Disease-Free Survival (DFS) and Overall Survival (OS). There are no shared and effective therapeutic protocols; our approach aims to improve the prognosis for advanced diseases.53-year-old female patient admitted to hospital for intestinal obstruction in April 2016 with evidence of an ileal mass in the right iliac fossa and peritoneal metastases, undergoing urgent surgery of ileal resection, ileostomy and nodular peritoneal mass biopsy. Histological examination diagnosed HS. Staging exams excluded the involvement of other gastro-intestinal districts in the absence of concomitant lymphoproliferative disorders, and PET-CT revealed multiple abdominal and mediastinal nodes hyperplasia. The patient performed chemotherapy and, seeing the partial abdominal extranodal response, was performed cytoreductive surgery (CRS). Histological examination confirmed the diagnosis of HS with multiple peritoneal, ileal, colic and omental localisation. Adjuvant second-line chemotherapy treatment (4 DHAP cycles) was performed. Disease Free Survival at ten months from cytoreductive surgery, Overall Survival at 21 months from diagnosis.Despite a multimodal therapy with surgery and chemotherapy, extranodal multisystemic HS has a poor prognosis. Up to now the role of surgery is limited to biopsies or complications treatment. Our results of DFS and OS show that cytoreductive surgery may be a valid therapeutic choice.The surgical approach with major cytoreductive purposes could improve the prognosis in cases with prevalent abdominal extranodal localisation. According to data from the SEER database of U.S. National Cancer Institute, HS is more common in adults with a median age of 63 years despite having an extremely wide range (from 18 to 96 years) with a slight incidence in favour of males (1.5:1) . The patThe clinicopathological manifestation could be as primitive extranodal neoplastic disease  or assocUp to now, the role of surgery is almost confined to biopsies or complications treatment. Our results of DFS and OS show that cytoreductive surgery may be a valid therapeutic choice for improving the prognosis of advanced extranodal abdominal HS.253-year-old female patient with a history of latent tuberculosis infection in prophylactic treatment with isoniazid, previous surgery of tonsillectomy, appendectomy and cholecystectomy. Family history negative for neoplastic diseases. Hospitalised at another institute in April 2016 with clinical manifestation of intestinal obstruction with evidence for CT scan of a solid occluding mass of a distal ileus of 5 cm maximum diameter localised in the right iliac fossa. Multiple nodular neoformations at the peritoneal level, the largest of 2 cm maximum diameter with associated free fluid in the abdomen and multiple lymphadenopathies of the ileal mesentery and some enlarged lymph nodes of the right anterior heart-phrenic angle. The latest was the unique finding of extra-abdominal disease spread. So the patient underwent surgery for ileal resection and ileostomy, with a peritoneal nodule biopsy.The definitive histological examination of the ileal mass  characterization showed positive reactions for histiocytic markers (CD163 and CD68); negative reactions for lymphoid markers  and myeloperoxidase. The reaction for cNPM is negative. Negative reactions for cytokeratins , desmin, caldesmon, ML actin, CD34, CD117, DOG1, S100 protein, melanA, CD21, CD1a. Cytoproliferative activity (MIB1-LI) in 15–20% of cells. Biopsy histological examination on the major of the peritoneal nodularity confirms the diagnosis of HS peritoneal localisation. The patient evaluation was performed with Esophagogastroduodenoscopy and Colonoscopy that excluded the involvement of other gastrointestinal tracts, bone marrow biopsy to confirm the absence of concomitant lymphoproliferative diseases. PET-CT showed multiple uptakes at the ileal mesentery, right and left colon, lymph node uptake also at the thoracic level at the phrenic heart angle, a finding already known in previous exams. The patient then performed six cycles of CHOEP chemotherapy. After adjuvant therapy, the PET-CT described a partial response to treatment with regression of uptake areas at thoracic level and persistence of abdominal uptake of already knows localisations . After aPost-operative course was characterised by close monitoring in ICU up to the 3rd postoperative day and then transferred to our ordinary ward, the onset of infectious pneumonia resolved with antibiotic therapy. Discharge of the patient at home on the 20th postoperative day. Histological examination confirmed the diagnosis of HS with ileal relapse, multiple peritoneal, small bowel mesentery, colic and omental localisation, negative for HS localisation the other tissues removed. The postoperative CT scan showed a complete resection of abdominal HS localisation such as CT-scan at three and six mounths-follow-up. During the same period the patient underwent a second-line chemotherapy treatment with four cycles according to DHAP scheme . The subsequent CT-scan performed at ten months from cytoreductive surgery (17 months from diagnosis) highlighted a disease relapse with multiple abdominal and thoracic localisations. Exitus at 21 months after diagnosis due to progressive decay of the general performance status.3The main feature of this case is the relatively long disease-free survival of the patient obtained with an innovative surgical approach. HS is an extremely aggressive neoplastic disease for which, as a second-line treatment after partial response to adjuvant chemotherapy, it was chosen, after multidisciplinary discussion, to surgical cytoreductive treatment. There is no standardise therapy for HS, the rarity and consequently, the smallness data present in literature does not allow the formulation of a widely shared diagnostic and therapeutic management. After pathologic evaluation with the exclusion of other pathologies of histiocytic aetiology by immunohistochemistry, for the patient was suggested cytoreductive surgery. The correct procedure of CRS consists of being as radical as possible, so it was necessary to make the total colectomy, the oophorectomy and the splenectomy. The spleen was removed to guarantee the radicality of surgery. In literature, data on OS and DFS for HS with multisystemic abdominal localisation are few and fragmented. Pileri conducted one of the major reviews in 2002  which anHS is an extremely aggressive neoplastic disease for which in our centre, as a second-line treatment after partial response to adjuvant chemotherapy, it was chosen, after multidisciplinary discussion, to surgical cytoreductive treatment a noticeable result when compared to the poor prognosis of multisystem extranodal cases.The limited knowledge of the HS natural history makes every report precious. Development of clinical trials that allow the elaboration of a widely shared therapeutic, diagnostic procedure is needed. In such rare diseases, randomised or prospective studies are hard to achieve, and case reports will continue to be the foundation management of these patients.4Proper integration between oncologists and haematologists, radiologists and surgeons resulted in the correct management of a patient suffering from a rare disease. The authors suggest, in well-selected patients and after the tumour board discussion, to consider CRS as another therapeutical option in this aggressive disease, ever after adequate chemotherapy that, to date, must be considered the first line of therapy ever.Further studies are needed to validate the hypothesis and broaden the knowledge of this rare disease.No conflicts of interest.Non funding were used.The ethical approval for this case report is been exemptThe submitted case report was not a study, therefore no ethical approval or informed written consent was neededWritten 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 requestBarbato Giuseppe, MD - Data collection and author of case report and discussion.Tarantini Alessandro, MD - Data collection and author of case report and discussion.Serra Francesco, MD -Review of surgical technique literature and co-author of discussion.Cabry Francesca, MD - Review of surgical technique literature and author of introduction.Farinetti Alberto, MD - Review of literature and co-author.Sorrentino Lorena, MD - Review of surgical technique literature and author of introduction.The submitted case report is not a research study.Gelmini Roberta.The authors have nothing to disclose.Not commissioned, externally peer-reviewed.


The evaluation of the temporal bones by imaging methods is challenging andthought-provoking, requiring that the radiologist have specific knowledge of the anatomyof the many small, delicate local structures. One interesting aspect is that the imagingfindings can be symmetric and bilateral, making recognition of the normal anatomy evenmore important. The correlation with clinical data is also essential.(1) presents a review of theimaging findings of the main hearing disorders, all of which have a great impact onpatient quality of life, together with a brief summary of the clinical presentation ofeach. The article in question, authored by Salata et al.(1), divides hearing disordersby etiology-neoplastic, infectious/inflammatory, congenital, traumatic/post-surgical, orother-which is the ideal didactic approach for a pictorial essay. Another way ofanalyzing the temporal bone is by anatomical division. It is important for radiologiststo create a logical sequence in their routine evaluation, so that they do not overlookany of the structures. The typical approach is to start from the outside and workinward, evaluating the external ear, external auditory canal, mastoid, middle ear, innerear, petrous apex, facial nerves, vestibulocochlear nerves, and extra-axial regions ofthe brain, such an approach being crucial for formulating a diagnostic hypothesis andmaking the differential diagnosis(2).An article published in the previous issue of Radiologia Brasileira(3).Among the imaging methods currently available, the most widely used are computedtomography (CT) and magnetic resonance imaging (MRI), both of which are represented inthis article, as the means of illustrating the various diseases mentioned. It isimportant that radiologists know the main indications for the use of each modality, aswell as its limitations(3).The recently developed modality known as cone-beam CT provides higher resolution intemporal bone examinations and exposes patients to less radiation, although itsindications are quite limited, as is its availability in Brazil. However, it has beenused for monitoring the evolution of alterations already identified on multidetector CTand for the diagnosis of pathologies in children, as well as for the evaluation ofossicular prostheses, cochlear implants, malformations, andotosclerosis.In brief, CT allows excellent detection of calcifications, cortical bone, air, and fat,particularly in the study of the external and middle ears, as well as the oticcapsule(3). In addition, new MRI techniques, such asdiffusion-weighted and perfusion-weighted imaging, are already part of the routinediagnostic protocol at various centers, especially diffusion-weighted imaging for thepreoperative  or postoperative (follow-up) evaluation ofcholesteatoma, which classically presents restricted diffusion(6), although lesions smallerthan 3 mm might not be detected, even in 3.0 T scanners.MRI offers excellent characterization of the soft tissues, being indicated for theinvestigation of masses in the middle ear and of alterations that affect nervestructures such as those in the inner ear, middle cerebral fossa, posterior cerebralfossa, and base of the skull, has cast new light on the diagnosis ofMénière's disease (MD). It consists in performing MRI in a 3.0 T scanner 4h after intravenous administration of paramagnetic contrast medium, employing athree-dimensional fluid-attenuated inversion recovery sequence for the diagnosis andgrading of endolymphatic hydrops, which is present in up to 73% of patients withclinically possible MD, in 100% of those with clinically probable MD, and in 95% ofthose with clinically definite MD. Although not yet a diagnostic criterion, as a futureperspective and with optimization of the technique, it could contribute to thediagnosis, influence therapeutic decision making, and play a role in follow-upevaluations(6-8).Another recently developed MRI technique, currently used at only a few centers around theworld(1) article presents a comprehensive review of the hearingdisorders that have a great impact on patient quality of life. It is fundamental thatradiologists have knowledge of the anatomical structures of the temporal bones andcreate a routine for reporting their analysis of an examination, as well as being awareof the newest techniques available, in order to select the imaging method best suited toeach clinical situation.In conclusion, the Salata et al.


Hidradenoma papilliferum, also known as papillary hidradenoma, is a rare tumor of the sudoral glands, that occurs almost exclusively in women, on the vulvar and perineal region. Non-genital papillary hydradenoma is an even more rare occurrence, though some cases have been described in literature.This is a report of a 56 years old patient who presented with unilateral hearing loss secondary to a mass of the external auditory canal (EAC). A biopsy allowed us to make the diagnosis of a papillary hidradenoma of the EAC. The treatment was based on surgery; the tumor was removed while respecting the adjacent structures. Follow-up of the patient shows no actual recurrence.Ectopic Hidradenoma papilliferum located in the EAC is a rare occurrence, and should be considered as a possible diagnosis when investigating a mass of the EAC. •Hidradenoma papilliferum is typically a benign genital tumour, there have been reported cases of an ectopic localization in the head and neck region.•The principal differential diagnosis of a mass of the EAC should be a malignant tumor, particularly a squamous cell carcinoma.•Surgery remains the first-line treatment of benigntumours of the EAC. The extent of the the excision will depend on the size of the tumour and its histological type. It mainIn the case of the external auditory canal (EAC), the tumor develops from the ceruminous glands, which are modified apocrine sweat glands . The mai2A 56 years old north african male patient, with no prior medical condition, presented in our department with a 5-month history of hearing loss. There was no notion of facial palsy or otorrhea.Clinical examination found a red burgeoning mass in the right EAC, measuring approximatively 1,5 cm in diameter . Pure ToComputed tomography showed images pertaining to a tumor of tissular density , limitedA biopsy realised under local anesthesia allowed us to diagnose a papillary hidradenoma of the EAC , showingThe patient received surgical treatment a month after the first consultation.Surgery was performed under general anesthesia, using a combined retroauricular and endaural approach.The intervention consisted of the complete resection of the mass, while preserving the tympanic membrane and the auricle.A 3-month follow up found the patient in good health, with no clinical sign of a relapse.3Ceruminous glands are modified sudoral glands, localized deep in the skin of the EAC . Tumors Hidradenoma papilliferum is a benign, cystic tumor, which characteristically occurs in the anogenital region, mostly in white women . HoweverEctopic hidradenoma papilliferum localized in the EAC usually presents as a unilateral conductive hearing loss, sometimes associated with otalgia, otorrhea or facial palsy ,8,10,12.Computed tomography is especially useful when a malignant tumor is suspected, to assess the type of tumor and its spreading to the local structures , as well as to determine the anatomical relationships of the tumor while preparing for surgery . In the Confirmation of the diagnosis relies solely on histology , though It is recommended that the type of surgery should be adapted to the histological type of the tumor ,10. The 4Although rare, the possibility of sweat gland tumors, particularly malignant tumors, should be considered when investigating a mass of the EAC, to enable early diagnosis and treatment. Radiological evaluation and pathological examination are vital to make the diagnosis. The treatment relies mainly on surgical excision, which should be wide to prevent a local reccurrence.Written informed consent for publication of their clinical details and/or clinical images was obtained from the patient.Ethical approval has been exempted by our institution.None.Rabii LAABABSI: Corresponding author writing the paper.Zineb ELKRIMI: writing the paper.Boouzbouz anass: writing the paper.Lekhbal adil: writing the paper.Reda Abada: study concept.Sami Rouadi: study concept.Mohamed Roubal: correction of the paper.Mohamed Mahtar: correction of the paper.Researchregistry5198.Laababsi rabii.Not commissioned, externally peer reviewed.The authors declare having no conflicts of interest for this article.


Papillary thyroid cancer with desmoid-type fibromatosis (PTC-DTF) is an uncommon tumor characterized by extensive stromal proliferation of fibroblasts and myofibroblasts with a small component of PTC. We report a case of PTC-DTF with infiltration of the mesenchymal component of tumor into perithyroidal muscle and early recurrence of desmoid after thyroidectomy, an outcome previously not reported.A 20-year-old man underwent left hemithyroidectomy for a thyroid nodule. Pathology demonstrated a 4.2 cm tumor with PTC-DTF with the PTC comprising <10% of the tumor. The stromal component extended into adjacent skeletal muscle. After completion thyroidectomy, histopathology of the right thyroid lobe revealed no malignancy or fibromatosis. Neck MRI 16 months after the initial operation revealed a 10.5 cm tumor in the left thyroid bed. Core biopsy and open excisional biopsy showed desmoid-type fibromatosis without PTC. The patient is undergoing chemotherapy of his recurrent desmoid-type fibromatosis.In patients with PTC-DTF there is a risk of recurrence of the benign component of the tumor. In recent reports, the role of less aggressive surgery, or even non-surgical management, of patients with recurrent DTF has been emphasized, in particular when extensive surgery may be associated with high risk of functional loss. The management of our patient adheres to modern recommendations for the treatment of DTF.Patients with PTC-DTF should be carefully monitored after thyroidectomy for both recurrent PTC and local recurrence of the fibrous component of the tumor. The tumAberrant nuclear expression of β-catenin in the mesenchymal cells is present in most cases of PTC with fibromatosis-like stroma ,7. BecauWe report a case of PTC-DTF with infiltration of desmoid-type fibromatosis into perithyroidal muscle and an early recurrence of the mesenchymal tumor component after thyroidectomy, an outcome previously not reported in the literature. The case report adheres to the Surgical CAse REport (SCARE) guidelines .2A 20-year-old man without personal or family medical history presented to his primary care physician with a left-sided thyroid nodule. Ultrasound showed a 3.2 × 1.9 × 2.7 cm heterogeneous nodule in the left thyroid lobe without calcifications or increased vascularity A. Fine nThe patient subsequently underwent completion thyroidectomy. Histopathology of the right thyroid lobe revealed no malignancy or fibromatosis. The patient had an uneventful recovery after completion thyroidectomy and was doing well 3 and 6 months postoperatively with well-healed neck incision. However, the ventral neck was firmer than typically seen after a thyroidectomy. Because of this, a neck CT was performed 3 months after completion thyroidectomy, revealing a 2 cm fluid accumulation in the left surgical bed as well as obscuration of adjacent fat planes, consistent with postoperative changes C. There Because of risk for local recurrence of the desmoid-type fibromatosis, the patient was scheduled for neck MRI but was lost for follow up. He returned 16 months after the initial operation with a palpable mass in his left neck and an MRI revealing a 10.5 cm tumor in the left thyroid bed D. Core b3On gross examination, cut section of the left thyroid lobe showed a circumscribed yellow to white 4.2 × 3.9 × 3.1 cm fibrotic nodule with areas of focal hemorrhage A. MicrosImmunohistochemistry showed aberrant nuclear staining for β-catenin in stromal cells whereas the epithelial component showed membranous staining for β-catenin without aberrant nuclear expression A and B. 4PTC-DTF may present as a firm enlarging thyroid mass ,7. BecauCTNNB1 gene, as reported in the stromal cells of PTC-DTF [Aberrant nuclear expression of β-catenin in the spindle cells was observed in several previous cases of PTC-DTF ,7,18 rep PTC-DTF , suggestDTF may be associated with mutations of the adenomatous polyposis coli (APC) gene and familial adenomatous polyposis (FAP) ,10. Our The pathogenesis of stromal proliferation in PTC-DTF is incompletely understood. While reactive and neoplastic etiologies have been proposed , changesDespite the small size of PTC in PTC-DTF, cases with extrathyroidal extension of the PTC component and lymph node metastases have been described ,7. InterIn contrast, the mesenchymal component of the tumor presented challenges regarding management and risk for recurrence. Extrathyroidal extension of DTF with infiltration into surrounding skeletal muscle has been reported previously ,6 but loBecause of the rarity of PTC-DTF, guidance for initial management and treatment of recurrence must be sought in the literature on primary DTF in other tissues. Although earlier reports emphasized the importance of negative surgical margins to prevent local recurrence , recent The shift towards conservative surgical, and even non-surgical, management of DTF is supported by the fact that some of these tumors are “self-limiting” and may regress spontaneously . Many DT5Although the patient is still undergoing treatment for his recurrent DTF, precluding statement about the long-term outcome, the present report is important for raising awareness of an extremely rare condition and highlighting recent management options for PTC-DTF that shows aggressive features with local recurrence of the mesenchymal tumor component. Patients with this unusual tumor should be carefully monitored after thyroidectomy for both recurrent PTC and local recurrence of the fibrous component of the tumor. In patients with recurrent DTF, a multidisciplinary approach is essential.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.The study has been reviewed by the Committee on Clinical Investigations (CCI) at the Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA. The IRB protocol is 2019D000498. “The proposed activity as described DOES NOT constitute human subjects research. Therefore, no further CCI review and approval is required.” The date of Review and Determination was 6/3/2019.The individual described in the case report cannot be identified by any of the images or through any part of the text.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.Study concept and design: Eve Roth, Courtney Barrows, Per-Olof Hasselgren, Benjamin James.Data collection: Eve Roth, Michiya Nishino, Barry Sacks.Interpretation of data: Eve Roth, Courtney Barrows, Michiya Nishino, Barry Sacks, Per-Olof Hasselgren, Benjamin James.Initial drafting of paper: Eve Roth, Per-Olof Hasselgren, Benjamin James.Research Registry UIN 4950.Per-Olof Hasselgren.Benjamin James.Not commissioned, externally peer-reviewedNone of the authors have any conflict of interest to declare.


We describe a case of intramuscular vascular malformation with extensive ossification within the posterior compartment muscles of leg which grossly mimicked long bone leading to equinus deformity of foot. Phleboliths and calcifications are characteristic of vascular malformations, while massive ossification is rare. To our knowledge, no extensive ossified vascular malformation within the leg muscle has been reported. Intramuscular vascular lesions occur with an incidence of 0.8%, most frequent in upper and lower extremities. Most are venous malformations. The diagnosis is rarely made before surgery and requires a definitive histological analysis, as there are no pathognomonic clinical or radiographic findings, especially with extensive ossification. Spontaneous regression does not occur, and excision of lesion is due to aesthetic and functional disturbances.  It is a new and rapidly developing discipline that demands teamwork between various specialties. For centuries, vascular birth marks were called by vernacular names derived from folk beliefs. It was believed that these were imprinted on the unborn child by a mother’s emotions or diet. Adjectives such as “cherry,” “strawberry” and “port-wine” have their roots in these traditional beliefs.2 in 1982 cleared the terminological confusion of vascular anomalies and was accepted by the International Society for Vascular Anomalies in 1996. This scheme evolved from studies that correlated physical findings, natural history, and cellular features.3 There are two major categories of vascular anomalies, tumors and malformations. Vascular tumors are endothelial neoplasm characterized by increased endothelial turnover; malformations are the result of abnormal development of vascular elements during embryogenesis. Calcification within vascular malformation is a common finding; but ossification within the malformation of the extremities is rarely reported.4-8A patient with vascular anomalies becomes a wanderer because of the improper diagnosis, complexity of treatment, limited outcome and lack of expertise available with individual doctors. Introduction of a biological classification system9 While ossified intramuscular vascular lesions have been reported in the extremities,10 to the best of our knowledge, ossified intramuscular vascular malformation resulting in painless equinus deformity of ankle joint has not been previously reported in the literature. The clinical, radiologic, magnetic resonance angiographic and histological features are discussed here. We believe that massive ossification is a rare phenomenon in vascular lesions and deserves recognition because it can pose diagnostic challenges.Surgical excision is the treatment of choice for symptomatic lesions.A previously healthy 42 years old male presented to our institute with history of gradually progressive and painless swelling over left calf since two months. He was a non-smoker, laborer by occupation. The patient noticed a firm swelling in calf region of left leg 5 years ago. No history of trauma or infection prior to the appearance of the mass was reported. No family history of any such swelling in the past. Patient was operated for swelling over calf region 20 years back but no records were available. There was a history of gradual increase in deformity of left foot since 3 years and the patient had started walking on toes on left side with no dorsiflexion at ankle joint. On examination, there was a single, non-tender, hyperpigmented scar of size 8×3 cm over mid-calf region fixed to underlying structures. A large, well defined non-tender, firm, swelling was palpable in posterior aspect of left leg measuring about 28×8 cm extending from tendoachilles region up to 5 cm distal to popliteal fossa and medially and laterally up to border of tibia and fibula respectively. The overlying skin was normal with no discoloration and local raise of temperature. Movement of knee joint was normal. There was fixed equinus deformity of left foot . No inflBecause of patient symptoms and with clinical diagnosis of a vascular malformation, a wide surgical excision of the lesion was done. Through a 25 cm longitudinal incision across the calf, posterior compartment muscles were exposed. The mass was found completely involving superficial group of posterior compartment muscles sparing the deep compartment with no attachment to periosteum or bone . PeroneaThe lesion was completely removed along with overlying cutaneous scar with wide surgical margins leaving posterior tibial artery in continuity. Intraoperative, complete dorsiflexion of foot was achieved with intact vascularity of leg. The excised specimen was very hard like bone and had to be cut longitudinally with saw . GrosslyMany of the vessels were thin walled with anastomosing and a sinusoidal appearance. Some of them showed fresh and organized thrombi within this vascular background, extensive osseous metaplasia characterized by mature lamellar bone formation was seen. . The dia11 The first description of a case of intramuscular hemangioma is attributed to Liston in 1843, which called this as “erectile tumor”.12 They are most frequent in the upper and lower extremities and trunk, occurring in the head and neck region in 15% of cases.12 Intramuscular hemangiomas can be either asymptomatic or symptomatic including pain, increased girth of the extremity or swelling and increased temperature or discoloration of the overlying skin. A palpable mass is seen in the vast majority of the cases. Larger hemangiomas may be associated with a bruit or thrill. It usually occurs before the age of 30 with equal sex distribution.13 The aetiology of intramuscular haemangioma still remains unknown.14 In addition to their vascular components, vascular malformations can show calcification, fat, smooth muscle and fibrous tissue, with fat being the most common component. Calcification and ischemic changes are rare, but recognized in venous malformation in brain  which are best appreciated on contrast enhanced MR.15,16Intramuscular vascular lesions have an incidence of 0.8%.11 Few cases have been reported of an extensively ossified venous malformation within the mylohyoid muscle11 and thigh.13,17,18 Ossification in the skeletal muscle vascular lesions is a rare phenomenon and less commonly described. The first case was reported as an orange-sized swelling in the right posterior lower leg of a 19 year old patient by Bishop in 1963.13Most intramuscular vascular lesions described in the literature as haemangiomas are not haemangiomas but venous malformations.13 X-Rays of ossified intramuscular hemangiomas showed irregularly ossified lesions in the soft tissues, the so-called “Swiss cheese” appearance described by Engelstadet et al.19 This appearance reflects the architecture of the mature bone interspersed with large cavernous vascular channels. Unlike appearance described by Engelstad et al., Swiss-cheese-like appearance was not detected in our case but linear and streak-like calcification was seen radiologically in our case.Differential diagnoses of ossified intramuscular vascular malformation radiologically include myositis ossificans, ossifying fibromyxoid tumor, fibrodysplasia ossificans, and extra skeletal osteosarcoma.13 The location and extent of the lesion must be determined for treatment planning and surgical resection remains the treatment of choice. Complete surgical excision with a surrounding margin of normal muscle is mandatory, using a minimally traumatic surgical technique and coagulation of all of the feeding vessels. Local recurrence rate could be up to 50%.17Asymptomatic intramuscular vascular malformation is observed closely. Indications for surgery include persistent pain, progressive increase in size of the mass, functional impairment and patient anxiety.Malignant transformation is rare. Lesions arising in muscle may create significant diagnostic uncertainty, as on clinical examination they are little different from soft tissue neoplasms and often imaging features may not be fully appreciated or easy to interpret. Skeletal muscle vascular malformations are uncommon soft tissue tumors that are completely treatable, the knowledge of their natural history, clinical findings, and imaging appearances are of great importance for proper diagnosis. Although rare, awareness of extensive osseous metaplasia is important for establishing diagnosis in view of its benign nature and also in excluding it from other bone forming lesions. To summarise, we are reporting a case of ossified intramuscular vascular malformation with its peculiar clinical, radiologic and histological features which shows a rare presentation of a relatively common tumor.The aim of reporting this case is to emphasize that, in cases with chronic or recurrent painful or painless swelling in a muscle or adjacent to a joint, associated with loss of function and deformity with or without an episode of trauma should alert the surgeon to this diagnosis. Investigations such as ultrasound can diagnose the lesion but may not be able to delineate its extent; MRI is preferred for identifying the exact location, extent, and size of the lesion for planning surgical excision, which isthe most preferred treatment for these lesions. Histological diagnosis is confirmatory. Patient should also be counselled for the possibility of local recurrence before surgery.The authors declare no conflict of interest.


To describe a rare case of a paraganglioma arising from the nasal septum and review the diagnosis and management of paragangliomas in the nasal cavity and paranasal sinuses.  We present a case of a 70-year-old female presenting with persistent nasal congestion and obstruction. Nasal endoscopy revealed a posterior septal mass approaching the sphenoid sinuses and partially obstructing the nasopharynx. A biopsy of the mass was taken, and histologic analysis confirmed a diagnosis of paraganglioma.  The patient underwent an endoscopic resection of the tumor. There has been no evidence of disease recurrence after 3 months of follow‐up.  Paragangliomas arising from the nasal septum are exceedingly rare, but should be considered in the differential diagnosis in patients presenting with nasal septal masses. These tumors are typically benign, although few cases of malignant sinonasal paragangliomas have been reported. Treatment requires surgical excision with close follow-up as several cases of tumor recurrence have been reported. Paragangliomas are rare, slow-growing neuroendocrine tumors arising from cells of neural crest origin. They typically originate from the adrenal gland, but occur extra-adrenally in 5–10% of cases . Though A 70-year-old female complaining of persistent nasal congestion and obstruction presented to our clinic for evaluation. She denied any headache or epistaxis. Nasal endoscopy was performed which showed a posterior septal mass approaching the sphenoid sinuses bilaterally and partially obstructing the view of the nasopharynx. The overlying mucosa was intact except for a small area superiorly which showed a soft granulomatous mass protruding into the left nasal cavity.The patient was taken to the operating room for septoplasty with biopsy of the mass at an outside institution. Microscopic examination of the biopsy specimen demonstrated clusters of epithelioid-appearing cells separated by bands of fibrillary stroma. The epithelioid cells were noted to have abundant amphophilic cytoplasm, uniform, rounded nuclei with “salt and pepper” chromatin, and small nucleoli. No mitotic activity, invasion, necrosis, or calcification was seen. Immunohistochemical staining demonstrated positivity for neuron-specific enolase (NSE), chromogranin A, synaptophysin, and CD56 cell markers within the epithelioid cells. Fibrillary cells were positive for NSE, chromogranin A, S-100, glial fibrillary acid protein (GFAP), and CD56 cell markers. Based on the histological appearance and immunohistochemical staining, a diagnosis of paraganglioma was made.The patient was referred to our institution for further management. Preoperative CT imaging showed a smoothly marginated, soft tissue density mass centered at the posterior nasal septum with extension into the nasopharynx and bulging into the right sphenoid sinus . Severe An endoscopic resection of the mass was performed. Intraoperatively, a large mass was seen in the posterior aspect of the septum, bulging into the bilateral nasal cavities and extending into the sphenoid sinuses . ErosionThe patient has been symptom free without local recurrence 3 months following tumor resection.Paragangliomas arising from the nasal cavity and nasal sinuses are extremely rare. Only 48 total cases of paragangliomas occurring in the nasal cavity or paranasal sinuses have been reported to date, occurring in patients with ages ranging between 8 and 89 years old, with an average age of 49 years old. These tumors are slightly more prevalent amongst females (60.4%). Twelve were reported to be malignant in nature, demonstrating either intracranial extension or metastasis to the cervical lymph nodes, brain, and bone. These masses were most frequently reported to originate from the ethmoid sinuses, middle turbinate, and maxillary sinuses. Other less common locations of origin reported include the superior and inferior turbinates, and the sphenoid and frontal sinuses. To the best of our knowledge, there has only been one other reported case of a paraganglioma originating from the nasal septum  [8].The most commonly reported symptoms included nasal obstruction, headache, and recurrent epistaxis. The vast majority of reported cases were functionally inactive, with no evidence of catecholamine or adrenocorticotropic hormone (ACTH) secretion. However, there have been a few reported cases of metabolically active tumors secreting ACTH and catecholamines, causing hypertension and other cushingoid features in the affected patient , 13, 30.The origin of paragangliomas in the nasal cavity and paranasal sinuses remains unclear. Although paraganglionic tissue has never been demonstrated in the nasal cavity, a wider distribution of paraganglionic tissue is thought to exist in fetuses and neonates, degenerating after birth , 43. SomIn contrast, chondrocytes of the cartilaginous septum develop from neural crest cells between the nasal cavities by the ninth week of embryonic development , 47. ThiMasses arising from the nasal septum represent a broad spectrum of diagnoses including polyps, chondromas, hematomas, and hemangiomas. Less common septal lesions include sarcomas, melanomas, and other neoplasms , 50. PatThe classical histologic appearance of a paraganglioma features an alveolar pattern of well-defined clusters of epithelioid chief cells with round nuclei and eosinophilic cytoplasm. These cells form a “zellballen” pattern of nests separated by vascular stroma and spindle-shaped sustentacular cells , 43. ImmAlthough the majority of reported nasal paragangliomas are benign, 12 of the cases presented in this review (25.0%) were reported to be malignant, demonstrating intracranial invasion and/or metastasis to the brain, cervical lymph nodes, and bones. It is widely accepted that malignancy cannot be diagnosed by histology alone, but requires evidence of bony invasion or distant metastasis , 19, 22.Almost all of the cases presented in this review were treated surgically. Suggested management of sinonasal paragangliomas involves surgical excision with close follow-up, as several cases of recurrence have been reported , 22, 29.We present a rare case of a paraganglioma presenting as a septal mass. To the best of the authors' knowledge, only one other case of nasal septal paragangliomas has been reported. Clinically, these lesions cause symptoms of obstruction, congestion, headache, and epistaxis. Recognizing that paragangliomas can arise from the nasal septum is crucial to accurately diagnose these tumors when evaluating septal lesions. A diagnosis of a paragangliomas can be confirmed based on the histopathologic imaging. These tumors are usually benign but require additional imaging as malignancy cannot reliably be ruled out on the basis of histology alone. Treatment of these tumors requires surgical resection, though radiation therapy has been to show slow growth and decrease recurrence.


Oral focal mucinosis, the oral counterpart of cutaneous focal mucinosis, is a rare disease. As it has no characteristic clinical or radiological features, diagnosis is established by histopathological and immunohistological examination. We present three cases of oral focal mucinosis occurring in the retromolar (which is extremely rare) and gingival regions.Case 1 involved a 26-year-old Japanese man with radiolucency in the right retromolar region on panoramic radiograph and computed tomography; no obvious protrusion was observed in the region. This finding was clinically diagnosed as a tumor of the retromolar region. Case 2 involved a 60-year-old Japanese woman. A tumor-like mass of tissue was identified on the buccal gingiva at the maxillary right canine and first premolar region. The lesion measured 7 × 6 mm and exhibited elastic hardness and healthy-colored mucosa. The lesion was diagnosed as an epulis. Case 3 involved a 47-year-old Japanese woman. A tumor-like mass of tissue was identified on the buccal gingiva at the maxillary right canine and first premolar region. The lesion measured 10 × 10 mm and exhibited elastic hardness and redness of the surface mucosa. This lesion was also diagnosed as an epulis. Resection was performed in all three cases, and the lesions were histopathologically diagnosed as oral focal mucinosis. Postoperative courses were uneventful and, thus far, there have been no recurrences.Although it is difficult to diagnose oral focal mucinosis based on clinical symptoms and imaging findings, the disease should be considered a possibility when diagnosing benign oral tumors. We believe that an emphasis on histopathologic study is essential to confirm the clinical suspicion. Oral focal mucinosis (OFM) is a rare disease; in 1974, Tomich . While tA 26-year-old Japanese man was referred to our hospital in February 2008 with a chief complaint of swelling in the alveolar region of a maxillary anterior tooth, which had been present for the prior month. An intraoral examination revealed alveolar swelling on the labial side of the maxillary anterior tooth region. The mucosa of the retromolar region exhibited a normal color and no evident swelling Fig. a. A panoHistopathological examination identified stellate-shaped and spindle-shaped fibroblasts interspersed in an abundant myxoid matrix. Sparsely intercalated fibrous connective tissue was also observed Fig. a, b. AlcA 60-year-old Japanese woman visited our department in January 2015 with a chief complaint of a mass at the maxillary right canine and first premolar region, which had been identified during a visit to a private dental clinic in April 2014 for dental treatment and was still present at follow-up in January 2015. An intraoral examination revealed a 7 × 6-mm mass with elastic hardness and no mobility on the buccal gingiva at the maxillary right canine and first premolar region. The surface mucosa was a normal color, and the mass was painless and non-pedunculated Fig. a. DentalHistopathological examination identified a myxomatous stroma with well-delineated borders and few fibers Fig. a, b. TheA 47-year-old Japanese woman presented to our department in October 2016 with a chief complaint of a mass on the buccal gingiva at the maxillary right canine and first premolar region, which she had been aware of since September 2015. An intraoral examination revealed a 10 × 10-mm mass with elastic hardness and no mobility on the buccal gingiva at the maxillary right canine and first premolar region. There was partial redness of the surface mucosa, and the mass was painless and non-pedunculated  have been reported , 7.The clinical diagnosis of OFM is particularly difficult, and the diagnoses in previous reports have included fibroma (32.8%), epulis 10.4%), papilloma (3.0%), mucocele (3.0%), benign tumor (1.5%), periodontal abscess (1.5%), and giant cell granuloma (1.5%), as well as pleomorphic adenomas in cases of lesions of palatal origin   due to incomplete resection ; progresHere we present three cases of OFM along with a review of the literature. Although OFM is difficult to diagnose on the basis of clinical findings and its frequency is low, the disease should be considered when diagnosing benign oral tumors.


Salmonella. He underwent explorative laparotomy in emergency setting, and cholecystectomy was performed due to his toxic clinical condition. Empyema of gallbladder was revealed and cultures were collected. A combination of antibiotics (ciprofloxacin and metronidazole) was set, and the patient was discharged 8 days after the surgical operation in good condition. It has to be highlighted that acalculous cholecystitis is a rare entity, mostly at critically ill patients, and treatment options depend on clinical condition, risk factors, and etiology. To our knowledge, this is the first case report dealing with acalculous cholecystitis with empyema due to salmonellosis up to date. Although it is extremely rare, high index of suspicion is needed by the operating surgeon in order to avoid unfavorable outcomes.Empyema of acalculous gallbladder in the setting of salmonellosis represents an extremely rare and life-threatening clinical condition in adults. In this unique case report, we deal with a previously healthy patient who developed acalculous cholecystitis and empyema due to infection by Acalculous cholecystitis is an acute necroinflammatory disease of the gallbladder with a multifactorial pathogenesis. It accounts for approximately 5-10% of all cases of acute cholecystitis, is more fulminant than calculous cholecystitis, and is associated with high morbidity and mortality rates [ E. coli, Klebsiella pneumoniae, and Salmonella species, which then cause inflammatory response in the gallbladder wall [Pathogenetic factors are stasis, ischemia, cystic duct obstruction, and systemic illnesses, for example,der wall . Acalculder wall , 4.Empyema of the gallbladder is one possible complication in the natural course of acute cholecystitis, it is a surgical emergency and demands a surgical intervention. To our knowledge, this is the first case report up to date dealing with empyema of the gallbladder due to salmonellosis in adults.A 32-year-old previously healthy male was admitted to our hospital with generalized abdominal pain, diarrhea, nausea, vomiting, and fever up to 39°. During assessment he was febrile without chills at 39°, BP at 135/70, pulse rate of 95 bpm, and respiratory rate at 20 breaths per minute. By his physical examination the abdomen was mildly tender to palpation with guarding in his epigastric and umbilical region.μL with 92% neutrophils, 3.5% lymphocytes, normal red blood cell count (5x10∧6/μL), haemoglobin (14.5g/dL), and platelets (160x1000/μL). The biochemical studies including liver, renal, and coagulation profile were normal. There were a mild hyponatremia and hypokalemia and CRP was 8.56. Cultures were obtained from blood, stool samples were obtained, and ceftriaxone and metronidazole were empirically administered.Laboratory tests disclosed a white cell count of 14.4x1000/Furthermore, no abnormalities were detected on chest and abdomen X-ray whereas an urgent abdominal ultrasound revealed thickening of the gallbladder wall, gallbladder contraction, and a minor pericholecystic fluid collection, without dilation of common bile duct or intrahepatic biliary system. In view of the clinical and ultrasonographical findings, the patient was diagnosed with acute acalculous cholecystitis and he was hospitalized initially for fluids, antibiotics, and observation.μL with 67.4% neutrophils and 18.9% lymphocytes. Red blood cells were at 4.2x10∧6/μL, haemoglobin at 12.6g/dL, and platelets at 125.5x1000/μL  that causes typhoid and enteric fever and the much broader group of nontyphoidal Salmonella which causes gastroenteritis [ Salmonella gastroenteritis typically occur within 8 to 72 hours following ingestion usually of contaminated food or water, and the patient is presented with diarrhea, nausea, vomiting, fatigue, fever, chills, and abdominal pain [ Salmonellainfection cases develop bacteremia, which can lead to a variety of extraintestinal manifestations such as endocarditis, myocarditis, pneumonia, pleural empyema, osteomyelitis, acalculous cholecystitis, hepatitis, hepatic abscesses, splenic abscess, peritonitis, paralytic ileus, and urinary tract infection [Salmonellae are motile gram-negative bacilli that colonize a wide range of mammalian hosts. They cause a broad range of infections, including gastroenteritis, enteric fever, bacteremia, and endovascular and focal infections such as abscesses. They can be divided into two broad categories: the typhoidalnteritis . Symptomnal pain , 7. Fewenfection .Lothrop, in 1915, first reported a case of acute acalculous cholecystitis as a complication of salmonellosis . NowadayThe clinical manifestations of acute acalculous cholecystitis depend on the clinical status of the patient at the time of presentation. Fever, nausea, vomiting, diarrhea, abdominal pain, right upper quadrant pain, and leukocytosis are the initial symptoms, until the late complications such as sepsis and peritonitis . When thAbdominal ultrasonography is considered the investigation of choice, and the criteria of acute acalculous cholecystitis include gallbladder wall thickness of >3mm, ultrasonographical Murphy's sigh, enlarged tense gallbladder, pericholecystic fluid, and absence of gall stones. Computed tomography and HIDA-scan are considered a good adjunct to ultrasound , 14.Therapeutic management is controversial. Generally, if there are not any complications, such as empyema, gangrene, or perforation of the gallbladder, conservative treatment is sufficient. In case of complications the surgical treatment is mandatory. This can be performed with a simple percutaneous drainage, cholecystostomy (percutaneous or open), or cholecystectomy (laparoscopic or open) . In our In summary, detention of acute acalculous cholecystitis, suspicion of its rare complications such as empyema, and early surgical intervention are crucial. The management of acalculous cholecystitis without complications is usually conservative, but when it is enchanting with empyema, gangrene, or perforation of gallbladder, the surgical treatment, as in our reported case, is absolutely necessary in order to avoid unfavorable outcomes.