We report the first case in Japan of paraneoplastic dermatomyositis with pure seminoma, a tumor that extremely rarely accompanies dermatomyositis. The patient presented to the hospital with muscle weakness and erythema and was diagnosed with dermatomyositis from skin biopsy. Routine radiological screening revealed testicular tumor and massive lymph node metastases. We initially performed orchiectomy along with conventional immunotherapy. However, muscle weakness gradually worsened, and he eventually showed dysphagia and forced respiration and became bedridden. Although he seemed close to being too unstable to tolerate further treatment, we started carefully adjusted chemotherapy comprising 4 courses of etoposide plus cisplatin, which proved highly successful. Lymph node metastases completely disappeared and swallowing and respiration fully normalized after completing chemotherapy. We believe that this clinical success was due to our decision to initiate chemotherapy even in such a weak patient. Dermatomyositis is a connective tissue disorder caused by perivascular inflammation in the skeletal muscles. Clinical manifestations include muscle weakness and skin lesions such as heliotrope rash and Gottron's papules. Approximately 20% of patients with dermatomyositis reportedly show concomitant malignancies, including breast, colon, ovarian, and lung cancers .We recently encountered a dermatomyositis patient with metastatic seminoma, which is extremely rare as an accompanying tumor. Of note was the finding that symptoms of dermatomyositis did not resolve with orchiectomy and steroids but resolved fully with chemotherapy started despite the unstable condition of the patient. We report here the unique clinical course of this patient with dermatomyositis accompanied by metastatic seminoma along with a review of the literature.A 70-year-old man presented to the Department of Internal Medicine at our hospital complaining of muscle weakness of the extremities, erythema, and impaired gait, which had rapidly deteriorated since 1 month earlier. Muscle weakness was assessed as MMT 2/5, and he was bedridden with PS grade 3. Based on these clinical findings, dermatomyositis was suspected. Skin biopsy confirmed the diagnosis of dermatomyositis.Whole-body CT was performed as a routine screening for neoplasm, revealing a lesion in the left testis and systemic enlargement of the lymph nodes . The patβ and AFP levels were within the normal ranges of <0.1 ng/mL and 4.6 ng/mL, respectively.Serum levels of CK and aldolase, both representing myogenic enzymes that serve as serum markers for dermatomyositis, were increased to 2621 U/L and 19.1 IU/L, respectively. In terms of markers for testicular tumor, serum LDH was elevated to 1033 U/L, whereas HCG-On the assumption that the presence of the testicular tumor had played a role in the development of muscle weakness, we performed left orchiectomy. The resected testicular tumor was pathologically diagnosed as seminoma (pT2N2M1aS1: stage IIIB) . However2 and cisplatin at 20 mg/m2 for 5 consecutive days every 3 weeks, excluding bleomycin from the standard regimen, because dermatomyositis could have been complicated with interstitial pneumonia. The treatment course is shown in Since removal of the tumor along with immunotherapy had not relieved his symptoms, we were left with no choice but to treat the patient with chemotherapy for seminoma. This had not been the initial option, as the patient was considered almost too weak to tolerate this therapy. We selected four courses of EP regimen, comprising daily doses of etoposide at 100 mg/mIn the first course of the EP regimen, abdominal lymph nodes showed a mild reduction in size and respiratory muscles seemed to regain some strength. Because of bone marrow suppression with this first course, the doses of both cisplatin and etoposide were reduced to 70% from the second course onward.By the time all four courses of EP had been completed, the metastatic lymph nodes had completely disappeared . In addiBreast, colon, lung, and ovarian cancers are frequently reported as associated cancers in paraneoplastic dermatomyositis, but association with testicular tumor has rarely been documented . Our revDermatomyositis has been reported in various stages from stage I to stage III –4. In ouAutoimmune reactions have been speculated to play a role in the mechanisms of paraneoplastic DM. Kaji et al. recently identified an autoantibody reactive with 155/140 kDa nuclear proteins, which could serve as a serological marker for the disease . They reIn the present case, symptoms of dermatomyositis progressed despite removal of the primary tumor along with immunotherapy. Although our patient appeared too weak to tolerate chemotherapy, which is not generally performed for such fragile patients with PS grade 4, we had no other choice but to apply this option to combat the metastatic lesions.Our regimen, which was carefully selected and dose-adjusted, proved successful. At the completion of chemotherapy, symptoms of dermatomyositis had fully resolved and lymph nodes had totally disappeared, indicating that our strategy was valid. Although administration of anticancer agents to highly unstable patients with poor PS is a challenge, we consider that such treatment could be feasible under an appropriate regimen with adjusted doses. The patient in this case is now being followed up on an outpatient basis. As of 2 years since being discharged from hospital, the patient has experienced no recurrence of the testicular tumor or DM.


Aim. To report on the endodontic and periodontal management of a root and alveolar processperforation in a maxillary front tooth. Summary. Perforation during access cavity preparation is an infrequent complication duringendodontic therapy, leading to potential periodontal tissue breakdown. The case described thetwo-stage management of a massive facial root perforation requiring a connective tissue graftto correct a mucosal fenestration persisting after orthograde repair of the root defect withMTA. Root canal perforation is defined as a communication between the root canal system and the periodontal attachment apparatus through the root canal walls. Iatrogenic root perforation is a procedural mishap but quite an uncommon event , which mFurthermore, perforation has been shown to negatively impact the outcome of nonsurgical endodontic treatment .Both management and prognosis are associated with the location of the tooth in the arch, the level of the perforation, the side of the root that is affected, the size of the defect, and the time. Several materials and technics have been used earlier to address this kind of problem either surgically , 7 or noPresented as a root end filling material in 1993  and firsDepending upon the tooth being affected and the localization of the perforation defect along the root surface, an esthetic defect can arise, especially in maxillary front teeth.This paper presents the multidisciplinary management of a buccal, midroot perforation on a maxillary central incisor complicated with a mucosal fenestration that needed a surgical correction after orthograde repair.A 20-year-old Chinese female patient presented to the Department of Conservative Dentistry and Endodontics with a history of a nonsurgical root canal treatment on the left maxillary central incisor  the week before. She mainly complained about the recent development of two fistulous tracts in the upper arch .The medical history was noncontributory; she had good oral hygiene and a normal healthy gingiva except at the buccal surface adjacent to the involved tooth 21.Teeth 11 and 21 presented with deep cavities; all teeth in the area responded within normal limits to cold test except for tooth 21 which showed an access cavity filled with a temporary dressing on the palatal surface of the crown. The probing of the gingival sulcus was normal for each tooth without pocketing, whereas tooth 21 was slightly responsive to percussion.A radiograph taken with a film holder  showed extensive coronal decay on both teeth 11 and 21 and a radiolucency superimposed on the canal outline altering it . AnotherCareful removal of carious tissues was performed under rubber dam isolation after disinfection of the operative field and the tooth . The cavities on teeth 11 and 21 were then filled with a direct composite restoration .An access cavity preparation was made on the palatal surface of tooth 21 under magnification with an operative microscope . Upon direct examination of the root defect, the diagnosis of buccal root perforation was confirmed . DecisioThe root canal was shaped manually according to the Schilder technic  with copAfter cone fitting, a gutta-percha cone devoid of sealer was seared off apical to the level of the perforation and the gutta-percha was warm molded to the dentinal walls in order to enclose the canal path and prevent inadvertent falling of the cement inside the canal.A mixture of MTA powder  with sterile water was prepared on a sterile glass slab according to the ratio recommended by the manufacturer (0.33 liquid/powder ratio). A first amount of the mixture was carried with a Messing Gun  inside the root canal and adapted to the defect with a specially designed spatula , the excess of moisture was blotted with the blunt tip of a sterile paper point in order to stabilize the cement in situ, and the defect was completely filled in successive increments of material . The tipTooth isolation and field disinfection were performed as described previously. The temporary dressing was removed as the paper points; the hard setting of the MTA cement was verified with an endodontic probe. After removal of the root canal plug of gutta-percha, the canal was irrigated with 1 mL of a 17% EDTA solution left in place for 1 minute  before a final flush with 6 mL of sodium hypochlorite  for 5 minutes. After cone fitting, the canal was filled according to the Schilder technic of warm vertical condensation  for the The access cavity was filled with a glass ionomer cement  covered with a resin composite restoration  .At the recall appointment three weeks later, tooth 21 was completely asymptomatic, the fistulous tracts had disappeared, but a scar corresponding to the coronal fistulous tract remained. At review after three months radiographic examination showed that the periapical lesion had reduced in size but a small fenestration of the attached gingiva was present communicating with the perforation site as shown by the exploration with a periodontal probe. Moreover, there was a mucosal discoloration resulting from the MTA placement Figures .The decision was made to perform a surgical correction of the defect and to increase the thickness of the gingiva in order to correct the discoloration. After local anesthesia with 1.6 mL of 4% articaine solution associated with 1/100.000 epinephrine , the margin of the fistulous tract was removed by a sharp dissection. A buccal intrasulcular incision, including one tooth mesial and distal of the lesion, was made. A vertical release incision was performed in the distal of tooth 12 extending beyond the mucogingival limit. A split thickness flap, using a 15 blade, was elevated to fully expose the buccal surface of the root. The fibrous, adhering tissues over the root were carefully removed. After raising the flap, it was possible to confirm that the periapical lesion did not communicate with the fenestration even if it had also perforated the facial cortical plate and that a small ridge of crestal bone remained coronally to the perforation. An apical curettage of the periapical lesion was performed in order to remove any extruding sealer from the previous root canal filling and to promote healing on both inflammatory sites. Neither apicoectomy nor retrofill was done since nonsurgical root canal treatment following root repair led to the disappearance of the most apical fistulous tract Figures . The sliThe six-month follow-up of the case did not reveal any recurrence of mucosal fenestration and gingival healing appeared complete. Probing depths on labial surface were within normal limit. The periapical lesion was in the course of healing . At thisA partial-thickness incision was performed under local anesthesia, using a 15 blade, to remove the prominent part of the graft. As in an external bevel gingivectomy, no suturing or dressing was used leaving a connective tissue exposure, and hemostasis was achieved by means of compression with a piece of sterile moist gauze. That patient was asked to use antiseptic mouthwash twice a day for 7 days (0.12% chlorhexidine). After secondary intention healing of the gingival wound, an intracoronal tooth whitening procedure was first performed on tooth 21 in two separate appointments according to the standard walking bleach technic , before At the one-year follow-up, the gingiva maintained its healthy state. The periapical lesion was still healing .At the two-year follow-up, the periapical lesion had completely healed and was asymptomatic ; the patThe case presented in this paper was a severely compromised central maxillary incisor. The site of the perforation was several millimeters beyond the crest; the wide area of the communication with the external surface of the root and thinning of the root canal wall made the prognosis poor. Treatment options were discussed with the patient, including immediate extraction and subsequent restoration with either a three-unit partial fixed prosthesis or a single-tooth implant. Even if an analytic framework could theoretically help in choosing the option the most likely to succeed, clinical decision can only be made at the patient level taking into account individual perception of success and financial considerations , 23. ConThe extensive mucosal and osseous damage the patient came in with incited us to first repair the root defect instead of placing an interappointment calcium hydroxide dressing to obtain the fistula's closure. Our aim in this procedure was to prevent further periodontal destruction, especially in a coronal direction to avoid downgrowth of gingival epithelium to the level of the perforation site. Furthermore, cases of gingival necrosis have been reported with calcium hydroxide extrusion into surrounding tissues , 25.Bargholz  describeDue to the localization of the osseous defect below the level of the mucogingival junction and the thinness of the free gingiva, the healing process resulted in a mucosal fenestration and discoloration of this soft tissue area. Discoloration caused by MTA placement has been previously reported in literature and was related to iron oxide , 28. WhiThe prognosis of perforations, which do communicate with the oral cavity, is considered to be questionable . Guided


Gastric dilatation may be an important complication in patients with eating disorders, particularly bulimia, which requires early diagnosis. We present a case in which it could be diagnosed using utrasonography in emergency department.19year old woman consulted our emergency department for abdominal pain within 3 hours of onset, without nausea, diarrhea or constipation, having presented a unique bilious vomit. He denies any personal history of interest.FC: 88 bpm, BP: 113/75 mmHg, SO2: 98%. Abdominal distention with diffuse signs of peritonism.The blood analysis showed no remarkable changes.The abdomen radiography showed a diffuse absence of air throughout all the abdomen. (image)Assessed by the surgeon, who after considering the episode as an acute abdomen, indicated the need for surgical intervention. It was decided to perform a bedside ultrasound previous to the surgery, detecting an intraluminal structure without peristalsis, extending from the epigastrium to hypogastrium, suggestive of gastric dilatation. Study was completed by abdominal CT scan which confirmed the diagnosis.Following the new findings anamnesis was performed again. The patient told a high intake of yogurt and large amounts of water in the hours before the onset of symptoms. She explained previous episodes that resolved after vomiting, which had been impossible this time.A nasogastric tube was placed, checking its position by ultrasound. 6 liters of gastric contents were extracted within the next 48 hours. At days 2 and 4 a bedside ultrasound was performed, demonstrating the progressive decrease of gastric size. The patient was discharged after 6 days.The gastric dilatation in patients with disorders of eating behavior presents a rare clinical manifestation, which may lead to gastric necrosis and perforation if not diagnosed early.In this case, the history and physical examination did not allow suspect this pathology. Laboratory tests and conventional radiology proved insufficient.Therefore, in case of abdominal pain of unclear etiology, we recommend an ultrasound at the bedside.The study was conducted in accordance with the ethical standards dictated by applicable law. Informed consent was obtained from each owner to enrolment in the study and to the inclusion in this article of information that could potentially lead to their identification.


It is essential to identify any variant anatomy prior to surgery as this could have a drastic effect on surgical planning. We describe a case in which two vascular irregularities, an Arc of Buhler and celiac stenosis, were identified on angiogram after completion of a pancreaticoduodenectomy. While there could have been catastrophic results from his surgery in the setting of celiac stenosis, the presence of the aberrant Arc of Buhler allowed this patient to emerge without any permanent morbidity.  The gastrointestinal system is supplied mainly by three vessels, the celiac trunk and the superior and inferior mesenteric arteries. There are numerous anatomical variants that exist between these arteries, many of which regress during embryological development. The embryological remnants of arterial communications between the celiac trunk and the superior mesenteric artery (SMA) collectively are derived from the persistence of the Tandler longitudinal anterior primitive anastomosis . The ArcMedian arcuate ligament syndrome (MALS) is defined as postprandial abdominal pain, nausea/vomiting, and weight loss due to celiac artery compression from the median arcuate ligament and surrounding structures . NormallA 58-year-old male with no significant medical or surgical history presented to the emergency department after his coworkers noticed scleral icterus. A 1-week history of darkened urine, vague abdominal pain, and acholic stools was noted upon further questioning. He reported a smoking history of 15 pack-years and substantial daily alcohol intake, though he reported no family history significant for malignant disease. Laboratory values on presentation demonstrated total bilirubin of 15.5 mg/dL, direct bilirubin of 11.7 mg/dL, and alkaline phosphatase of 951 U/L. Subsequent abdominal ultrasound revealed significant intra- and extrahepatic bile duct dilatation (CBD 2.0 cm) with concomitant pancreatic ductal dilatation. CT abdomen and pelvis demonstrated similar findings, without identifying a culprit mass .An endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic ultrasound (EUS) were performed the following day confirming ductal dilatation and identifying 32.5 mm by 31.8 mm ill-defined periampullary hypoechoic mass. EUS-guided fine needle aspiration was performed resulting in a diagnosis of pancreatic adenocarcinoma. There was no evidence of celiac lymphadenopathy or distant metastatic disease. The portal vein, superior mesenteric artery, and celiac axis were not involved with the lesion. Surgical oncology was consulted for resection. His CA 19-9 was found to be 793. Given findings on initial imaging and EUS/ERCP, the tumor appeared resectable and no further radiological studies were deemed necessary. At this time, after review by radiologists and surgeons, no other abnormalities were noted on the radiologic studies. After discussing treatment options with the patient and his family, the decision was to proceed with surgical resection.The patient was taken to the operating room where an initial diagnostic laparoscopy demonstrated no metastatic disease. An open pancreaticoduodenectomy was then performed with no complications. There was no noticeable vascular anomaly or aberrant anatomy during the case. Pathologically, the tumor measured 7 × 3 × 2.8 cm identified as moderately differentiated pancreatic ductal adenocarcinoma with 23 lymph nodes negative for malignancy. The tumor was staged at T3N0M0 (Stage IIA) per the American Joint Committee on Cancer guidelines .On postoperative day five, the patient experienced a significant decrease in his hematocrit level from 32.5 to 26.7% along with complaints of increased abdominal pain. After a poor transfusion response, a CT angiography was performed and revealed an intraluminal blush in the small bowel likely at the hepaticojejunostomy site. High-grade stenosis of the celiac artery was also demonstrated .A nasogastric tube was placed, returning 700 cc of blood and the patient was taken to the interventional radiology suite to achieve hemostasis. Attempts to cannulate the celiac artery were unsuccessful given the high-grade celiac stenosis seen on the CT angiography. A lateral aortogram was performed which failed to identify the origin of the celiac artery. The superior mesenteric artery was then cannulated and angiogram demonstrated extravasation from a distal branch in the right upper quadrant .Interestingly, contrast filling of the celiac artery was appreciated on this angiogram consistent with collateral circulation and communication between the superior mesenteric artery and celiac artery Figures  and 4. RFollowing IR intervention, the patient's hematocrit stabilized and slowly returned to normal limits. He experienced no further bleeding episodes. A follow-up CT showed concerns for a partial left hepatic lobe infarct. The remainder of his hospital course was complicated by bacteremia requiring antibiotic treatment. He was subsequently discharged on postoperative day 19.There are two main collaterals that occur between the celiac artery and the SMA: the gastroduodenal artery (GDA), a branch of the common hepatic artery, and the dorsal pancreatic artery, a branch of the splenic artery . The AOBMost reports in the literature describing the AOB are associated with finding a symptomatic aneurysm or describing the incidence of AOB after a retrospective review of angiographic films. There are reports describing celiac obstruction by an AOB aneurysm treated successfully with coil embolization , 13. TheThere is one case in the literature of an incidental AOB with associated celiac occlusion in preoperative workup for a pancreaticoduodenectomy . In thisThis case underlines the importance of preoperative planning and image review prior to surgical intervention as it could drastically change the patient's outcomes. Unfortunately, it was only after retrospective analysis that the AOB and celiac stenosis were seen on preoperative imaging, albeit the findings were not as impressive as the postoperative and IR imaging. It is important to educate both radiologists and surgeons, on the possible anatomical variants that may be encountered in the vascular supply of the foregut.


Giant neurofibromas in patients with neurofibromatosis type 1 involve multiple regions and are often difficult to surgically extirpate. However, surgical intervention is the most effective means for improving quality of life. The case reported herein is unique in that it involves a giant neurofibroma, involving the patient’s peritoneal and pelvic cavities, retroperitoneal space, and buttock, which was causing compressive displacement of abdominal and pelvic organs. A challenging surgical intervention was required to accomplish near-total resection to relieve organ compression while preserving visceral and genitoanal function.The case reported is of a patient presenting with a solitary giant retroperitoneal neurofibroma that threatened to obliterate both peritoneal and pelvic cavities and protruded conspicuously into the right gluteal region. The enormous dumbbell-shaped mass was surgically removed in three parts. Postoperative pathology studies confirmed a diagnosis of neurofibroma. Follow-up computed tomography images taken three months postoperatively revealed residual tumor in the perianal region. The patient’s quality of life had measurably improved on follow-up at eight months.Surgical intervention in such extraordinary circumstances of a giant neurofibroma causing compressive displacement of critical organs reduces tumor burden, restores appearance and function of patient’s body and internal organs, and improves the patient’s quality of life. Neurofibromatosis type 1  is an autosomal-dominant inheritable syndrome attributable to genetic mutations in the coding of neurofibromin . IntrapeThis 45-year-old female patient was hospitalized for a sizeable tumor of the right buttock Fig.  that impContrast-enhanced computed tomography (CT) revealed an enormous dumbbell-shaped mass (68.3 cm superoinferiorly), occupying the mid-abdomen, pelvic cavity, and buttock. The rectum, uterus, and urinary bladder were compressed and shifted to the left Fig. . On CT aen bloc abdominopelvic tumor removal. The protuberant skin-covered gluteal component, still communicating with the pelvic tumor, was incised along its base to maximize excision and skin procurement.The patient was placed in lithotomy position for the two-part tumor resection. First, a 20-cm midline incision was made from the umbilicus to pubic region, exposing a bulky encapsulated tumor of the abdomen and pelvis. The mass displayed solid and cystic areas, reaching a diameter of 30 cm. It was soft to the touch and grossly intact, completely displacing the peritoneal and pelvic contents. The bowel, uterus, and bladder were compressed and shifted laterally. The ureters and bladder were secured while freeing the encapsulated edge of the mass and severing its pelvic floor attachment, thus enabling Having no clear boundary, the narrowest segment of the dumbbell-shaped mass also involved the vagina and anus, creating compressive displacement (to the left) and preventing complete excision without mutilation. The remainder of this segment was isolated along its border and removed. Ultimately, the excised mass measured 50 cm across and weighed ~25 kg Fig. . PostopePostoperatively, the patient became febrile. B-scan ultrasonography indicated a seroperitoneum of moderate degree that was evacuated on postoperative day 6 via B-scan ultrasound-guided paracentesis. The fever thereafter abated. Three months postoperatively, a residual perianal tumor was presumptively identified in follow-up CT images. The gluteal and perineal regions were otherwise satisfactory, and the patient’s quality of life had measurably improved Fig. .Fig. 5aInfiltrative neurofibromas (with ill-defined borders) cannot be treated radically . The neuThis report validates the use of surgical intervention to reduce tumor burden, restore appearance and function, and improve quality of life in such extraordinary circumstances.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Ossifying fibroma is a rare benign bone tumor that occurs mostly in the jaw, but also affects paranasal sinuses and fronto-ethmoidal complex. Occipital bone is an extremely rare location for these tumors; only two cases have been reported.We present the first case reported as psammomatoid subtype of ossifying fibroma, according to the 2005 WHO classification. An 18 years old male patient with infratentorial tumor, in the occipital bone, that produces mass effect over the cerebellum.This case may provide a guide to consider these lesions for a more rapid and precise diagnostic in future cases. Juvenile psammomatoid ossifying fibroma (JPOF) is a rare benign bone tumor that often affects facial bones, especially the mandible, it commonly occurs in young patients, and the treatment is indicated because of deformity and esthetical reasons mostly, en bloc resection is the treatment of choice. It is as subtype of juvenile ossifying fibroma in the WHO classification of odontogenic tumors  and with juvenile trabecular ossifying fibroma (JTOF). COF is an odontogenic neoplasm arising from the periodontal ligament and affecting the tooth bearing are as of the mandible, and maxilla, whereas the nasoethmoidal location is rare (Speight and Carlos Only two cases of ossifying fibroma in the occipital bone have been described in the literature. Yamashita et al. published the first case back in 1977; the second case is published by Ozcan et al. in 1995; both of them in asymptomatic patients and without a subtype classification. We present the first case of occipital JPOF with neurological manifestations (Table The treatment of choice for JPOF is complete surgical excision. Partial or incomplete resection leads to recurrences and is reported to range from 30 to 56 % (Bertrand et al. The JPOF is a benign tumor with aggressive behavior that rarely affects the cranial bones; this is the first reported case within this localization causing neurological deficits. Total resection is the treatment of choice. Prognosis is good, without any case of metastasis being reported.


Rhabdomyosarcomatous dedifferentiation of GIST following tyrosine kinase inhibitor (TKI) therapy is rare, with only a handful of cases previously reported in the literature. Herein we present a case of metastatic GIST initially treated with imatinib that developed radiographic evidence of progression after 8 months of standard dose therapy with continued progression despite attempts at using dose-escalated imatinib 400 mg bid. Due to the patient's worsening clinical symptoms and radiographic concerns for colonic thickening, abscess, and extraluminal air, the patient underwent a palliative resection of a large heterogeneous mass arising from the posterior stomach and several metastatic foci. Pathology revealed a dedifferentiated GIST with rhabdomyosarcomatous features. This report will highlight the unique features of this case and review the existing literature. Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the alimentary tract. GISTs most commonly affect adults over the age of 50 with a slight male predominance . These nImatinib, a tyrosine kinase inhibitor (TKI), is the current mainstay of treatment for individuals with unresectable or metastatic disease based on data from Demetri et al. showing sustained objective response in more than half of patients treated . This poHerein we report a case of metastatic GIST with rhabdomyosarcomatous transformation following treatment with imatinib.Surgical specimens were fixed in 10% neutral buffered formalin. Following fixation gross examination was performed and representative sections were embedded in paraffin. Five micron thick hematoxylin and eosin stained sections were created. Immunohistochemistry for CD117 , CD34 , DOG1 , desmin , smooth muscle actin , myoD1 , and myogenin  was performed. Molecular analysis for KIT mutation was performed at an outside laboratory  using DNA extraction and purification of paraffin embedded tumor tissue.A 47-year-old African American male presented to the emergency department with complaints of right lower quadrant abdominal pain and a 20-pound weight loss over the prior two months. The patient had no significant past medical history or any other symptomatology. Computerized tomography (CT) imaging revealed a 14 cm tumor with possible central necrosis that originated from the posterior gastric wall and extended superiorly to the diaphragm . AdditioEight months after initiation of imatinib CT imaging demonstrated tumor regrowth and heterogeneous enhancement at the primary tumor site while other metastatic sites remained stable. The dose of imatinib was subsequently escalated to 800 mg daily (400 mg bid).Approximately one year after his initial presentation, the patient presented with upper gastrointestinal bleeding and an associated microperforation due to tumor progression . Given cThe resection revealed a 10.4 × 6.4 × 6.3 cm tumor arising from the stomach and invading into the spleen and pancreas. The bulk of the tumor was composed of pleomorphic, eosinophilic polygonal cells with bizarre nuclei, abundant cytoplasm, and increased mitotic activity (up to 3 mitotic figures per 50 high power fields) . Also obTo our knowledge, only 6 cases of rhabdomyosarcomatous dedifferentiation in GISTs have been reported following treatment with TKIs , 5. HeteThe rare cases of dedifferentiated tumors have generally demonstrated a more aggressive clinical course, with recurrence and metastases. This stands in contrast to primary GISTs with rhabdoid morphology, which, although also uncommon, have not demonstrated malignant behavior , 9.Based on the small number of previously reported cases, dedifferentiated tumors, as in our case, demonstrate resistance to the currently available TKI therapy. Time to treatment failure in these rare tumors with RMS dedifferentiation ranged from 10 to 24 months . Our patIn general, when treatment failure occurs, a unique pattern of disease progression follows, termed a “resistant nodule.” These nodules are seen on imaging as new central or peripheral areas of enhancement within preexisting responding lesions. These imatinib “resistant nodules” appear in about 50% of patients after two years of therapy and are frequently found to have novel mutations in KIT or PDGFR (that were not present in the primary tumor). These findings support the hypothesis that “resistant nodules” arise through clonal evolution . HoweverCuriously, the initial biopsy in our case was negative for KIT and PDGFRA mutations. This possibly represents a false negative result as the tumor was initially objectively responsive to therapy, and tumors have not previously been reported to gain a primary-type (exon 9 or 11) mutation upon progression.With regard to therapy, sunitinib and regorafenib are approved therapies in the second and third lines, respectively, but both have demonstrated only limited success –13. NoveIn summary, we report a genetically unique case of rhabdomyosarcomatous dedifferentiation in a GIST following TKI therapy. Our case supports prior reports that these tumors behave in an aggressive fashion with early recurrence and resistance to TKI treatment. The molecular findings suggest that the resistance to TKI therapy in these rare tumors is driven more by alternative mechanisms than through secondary Kit/PDGFRA mutations. Further study is needed to clarify these mechanisms as well as determine optimal treatment strategies.


Manipulation of vagal nerve rootlets, whether surgical or through mass effect of a neoplasm, can result in asystole and hypotension, accompanied by ST depression and right bundle branch block. There are few case reports of a neoplasm causing these effects, and this case describes a patient with such a mass presenting with syncopal episodes. A 43-year-old man with a past medical history of HIV, bipolar disorder, and epilepsy was admitted to the neurology service for a video electroencephalogram (vEEG) to characterize syncopal episodes that were felt to be epileptic in origin. During the study, he experienced symptoms of his typical aura, which correlated with a transient symptomatic high degree AV block on telemetry, and an absence of epileptic findings on vEEG. Magnetic Resonance Imaging (MRI) of the brain showed a mass in the left posterior carotid space at the skull base. The patient underwent permanent dual chamber MRI-compatible pacemaker placement for his heart block. His syncopal episodes resolved, but presyncopal symptoms persisted. We discuss the presentation and treatment of vagal neoplasms. The vagus nerve provides parasympathetic innervation to the sinoatrial node, atria, and ventricles of the heart. Manipulation of vagal nerve rootlets, whether surgical or through mass effect of a neoplasm, can result in asystole and hypotension, accompanied by ST depression and right bundle branch block –3. This A 43-year-old man with a past medical history of HIV, bipolar disorder, and epilepsy was admitted to the neurology service for a video electroencephalogram (vEEG) to characterize syncopal episodes that were felt to be epileptic in origin. These episodes started about ten years priorly and were associated with a preceding aura followed by loss of consciousness. He described this aura as a numbness that spread from head to toe and left him unresponsive for several minutes. He denied posturing, incontinence, eye deviation, tongue biting, or clonic activity during the events. He reported postevent disorientation and lethargy lasting from several hours to a full day. He had been on multiple antiepileptic medications without resolution of his episodes.On examination, the patient was an anxious appearing well-built gentleman with normal vital signs and normal cardiac and neurologic exams.During the vEEG, he experienced symptoms of his typical aura. Concurrently, his vEEG activity was normal, but his cardiac telemetry demonstrated pauses of up to six seconds , reflectNeurosurgery declined surgical intervention, recommending Lamotrigine as seizure prophylaxis and serial MRIs to examine progression of the mass. The patient underwent permanent dual chamber MRI-compatible pacemaker placement for his heart block during the admission and was discharged with plans for outpatient follow-up.Follow-up MRI after 4 months revealed a stable left carotid space mass. The patient denied further syncopal episodes since pacemaker implantation. He did, however, endorse episodes of numbness, decreased vision, and change in alertness occurring nearly daily, for which he was advised to consult his cardiologist.The vagus nerve provides parasympathetic innervation to the sinoatrial node, atria, and ventricles of the heart. It is also involved in the baroreceptor reflex, which responds to elevated blood pressure by increasing parasympathetic tone via decreased heart rate. Its path begins in the medulla oblongata, passes through the jugular foramen, and extends into the carotid sheath between the internal carotid artery and the internal jugular vein. The nerve then continues down to the neck, chest, and abdomen, where it helps to innervate the viscera. Cardiovascular effects of vagal disturbance similar to those seen in our patient have been described during surgical procedures involving manipulation of the vagal nerve rootlets , 4. NagaNeoplasms of, or affecting, the vagus nerve include schwannomas, paragangliomas, branchial cleft cysts, malignant lymphomas, and metastatic cervical lymphadenopathy. Most schwannomas of the 9th, 10th, and 11th cranial nerves are slow-growing and histologically benign and do not invade surrounding structures. Their main effect is due to pressure exerted on the surrounding cranial nerves, cerebellum, and brainstem. If symptomatic, they commonly present with hoarseness, vertigo, tinnitus, cervical pain, or paroxysmal cough . Vagal pSeveral case reports have described a presentation similar to that of our patient, in which a vagal neoplasm caused episodes of asystole and syncope , 6. OkmeAlthough rare, carotid mass should be included in the differential of a patient with syncopal or epileptic events. Imaging is central to the diagnosis of a vagal nerve neoplasm, with MRI being the study of choice . This im


Primary pulmonary synovial sarcoma (PPSS) is rare. We describe a case of PPSS complicated by tibial adamantinoma that required differentiation from lung metastasis.synovial sarcoma, X breakpoint 1 gene-fusion transcript was detected by reverse transcription-polymerase chain reaction. It is diagnostic of PPSS. Resection margins were negative. The patient was well without evidence of recurrence or metastasis of the PPSS or adamantinoma at the 30-month and 15-year follow-ups.A 39-year-old Japanese woman presented with hemoptysis, dyspnea, and a well-defined tumor measuring 3.0 cm in greatest diameter in the right lower lobe on chest computed tomography (CT). Positron emission tomography/CT with fluorodeoxyglucose (FDG-PET/CT) showed mild uptake of FDG . Her past history included surgery for adamantinoma of the right tibia at age 25 years. We considered the possibility of pulmonary metastasis from the adamantinoma and performed fluoroscopy-assisted thoracoscopic resection of the tumor after CT-guided Lipiodol marking. Histologically, the tumor was composed mainly of a dense proliferation of spindle cells. Immunohistochemical studies were positive for epithelial membrane antigen, B cell lymphoma 2, and transducing-like enhancer of split 1. They were negative for CD34. The Clinical and radiological manifestations of PPSS overlap with those of other lung tumors. The solitary pulmonary nodule in this case was indistinguishable from pulmonary metastases of the adamantinoma based on clinical symptoms, epidemiology, chest radiography, CT, and FDG-PET/CT. PPSS was diagnosed only after evaluating gross pathology, histology, immunohistochemistry, and cytogenetics. PPSS should be included in the differential diagnosis of a well-defined homogeneous round or oval lung mass. To our knowledge, this is the first report of PPSS complicated by adamantinoma. Most lung tumors are malignant, carcinomatous metastases from other tissue cancers, such as bone tumors. Primary lung sarcoma is rare, accounting for less than 0.5% of all lung tumors, 2. The A 39-year-old Japanese woman, who was a household helper, presented to a community hospital with hemoptysis and dyspnea. Other symptoms, such as cough and chest pain, were not presented. She reported a family history of stomach cancer (her father). She had smoked one or two cigarettes daily for the last 19 years. There was no history of asbestos exposure or tuberculosis. She had undergone wide resection of an adamantinoma of the right tibia and limb salvage with an uncemented megaprosthesis  15 years previously Figure a. BronchBecause the tumor was suspected to be a pulmonary metastasis from the earlier adamantinoma, we surgically resected it without prior biopsy. The procedure was as follows. The pulmonary nodule was marked with 0.4 mL of Lipiodol®  under CT guidance. The visceral pleura near the nodule was marked with 1 mL of a mixture of atelocollagen and methylene blue. During the thoracoscopic surgery performed 1 day later, the marked nodule was grasped with ring-shaped forceps under C-arm fluoroscopic guidance and resected, 7.sarcoma, X breakpoint 1 (SYT-SSX1) fusion gene was detected by reverse transcription-polymerase chain reaction using ribonucleic acid samples from the tumor tissue. Hence, the final diagnosis was PPSS. Resection margins were negative.Histopathologically, the tumor showed densely proliferated spindle cells with a high mitosis rate Figure a. The imSYT-SSX1 variant gene, we recommended chemotherapy with a combination of ifosfamide and doxorubicin. She refused further treatment, however. Follow-up visits were undertaken every 3 months after the surgery. She has remained well without evidence of recurrence or metastasis of the PPSS or the adamantinoma at the 30-month follow-up.The patient’s hemoptysis and dyspnea resolved after the surgical procedure. Because the patient had factors indicating a poor PPSS prognosis, including her age (>20 years), sex, and the presence of the et al.[Soft tissue synovial sarcoma is a clinicopathologically and cytogenetically distinctive neoplasm that mainly affects deep soft tissues of the extremities in adolescents and young adults. Synovial sarcoma is also recognized as a primary pulmonary neoplasm. PPSS iset al. reportedet al., 8, 11. et al., 10. Radet al., 13. Theet al.. Similaret al.. Becauseet al..Adamantinoma, in contrast, is a low-grade neoplasm that affects long bones and accounts for less than 1% of all primary bone tumors. These lesions occur most commonly in patients 10–50 years of age with slight male preponderance, 17. Theet al.[et al.[We considered pulmonary metastasis of adamantinoma in the differential diagnosis of our case because of the CT and FDG-PET/CT findings, the past history of adamantinoma, and the lack of reports of PPSS in conjunction with another tumor in the literature. Metastatectomy to remove pulmonary adamantinoma lesions with good results has been reported. There aet al. concludeet al., 22. Theet al., 23. To et al., 15. Acc[et al., the diaSYT-SSX1 variant gene[Because of its rarity and the paucity of data regarding its natural history, there are no guidelines regarding the optimal treatment for PPSS. Current treatment thus includes surgical resection (lobectomy or pneumonectomy). In our ant gene. AlthougWe had recommended that our patient undergo chemotherapy with ifosfamide and doxorubicin, but she refused further treatment. We also suggested pazopanib, which interferes with vascular endothelial growth factor and platelet-derived growth factor pathways. Pazopanib is the first nonchemotherapeutic anticancer agent approved by regulatory authorities for soft tissue sarcoma and to which pulmonary adamantinoma metastasis responds, 27. LonThis is the first report of PPSS complicated by tibial adamantinoma, which is a low-grade malignant primary bone tumor. PPSS is a rare tumor without specific symptoms, signs, or radiological features. The role of functional imaging has not yet been evaluated, and the diagnosis is usually based on histopathological examination or cytogenetic testing of the resected tumor. The diagnosis of PPSS before surgery also may not be possible, as in our patient. Along with pulmonary metastasis from a bone tumor, PPSS should be included in the differential diagnosis of a well-defined homogeneous round or oval lung mass.Written informed consent was obtained from the patient for publication of this Case Report and any accompanying images. A copy of the written consent form from our university hospital is available for review by the Editor-in-Chief of this journal.


The annular pancreas is a rare congenital anomaly in which a ring of the pancreas parenchyma surrounds the second part of the duodenum. Malignant tumors are extremely rare in patients with an annular pancreas. A 64-year-old man presented with appetite loss and vomiting. Abdominal contrast-enhanced computed tomography (CT) indicated pancreas parenchyma surrounding the second part of the duodenum, and a hypovascular area occupying lesion in the annular pancreas. Subtotal stomach-preserving pancreaticoduodenectomy was performed. Histopathology showed pancreatic carcinoma occurring in the complete annular pancreas. An annular pancreas is a rare congenital anomaly in which the second part of the duodenum is surrounded by a ring of pancreas parenchyma. It was first described by Tiedemann  in 1818 A 64-year-old man was admitted to University of Yamanashi Hospital for complaint of abdominal pain and vomiting. On admission, physical examination revealed no anemia and jaundice in the conjunctiva, and no tenderness and no mass in the abdomen. Laboratory findings showed elevated alkaline phosphatases, 898 U/l (100–310), aspartate aminotransferase, 67 U/l (<30 U/l), alanine aminotransferase, 144 U/l (<35U/l) and hemoglobin A1c, 7.7 % (<6.2 %). Furthermore, carbohydrate antigen 19-9 was elevated to 158 U/ml (<37 U/ml); however, carcinoembryonic antigen was within the normal range.Upper gastrointestinal radiology and esophagogastroduodenoscopy showed stenosis at the descending part of the duodenum. Endoscopic ultrasonography showed a hypoechogenic mass measuring 21 × 13 mm in the pancreas head. In addition, abdominal enhanced-CT revealed the pancreatic parenchyma encircling the descending part of the duodenum, and a hypovascular mass lesion was observed in the annular pancreas (Fig. The patient underwent subtotal stomach preserving pancreaticoduodenectomy with dissection of the regional lymph nodes. At laparotomy, the pancreas parenchymal tissue was surrounding the descending part of the duodenum, and the tumor was located in the annular pancreas Fig. . NeitherThe annular pancreas is a rare anatomical anomaly, in which the pancreas parenchyma surrounds the descending part of the duodenum. Most of adult cases become symptomatic at ages between 30 and 50 years . The prePreviously, two hypotheses have been proposed to explain the etiology of the annular pancreas: Lecco’s theory attributes it to adhesion of the right ventral angle to the duodenal wall , and BalThe presence of the annular pancreas was reported in three of 20,000 autopsies  and threThe efficacy of endoscopic ultrasonography in the annular pancreas has also been reported, as for less-invasive imaging devices , 17. In Yogi et al. classified six variants of the ductal anatomy in patients with annular pancreas . The mosAmong the cases reported in the literature, only five have been reported to involve the annular pancreas , 20–22. In conclusion, to our knowledge, the present report is the first clinical case of pancreatic carcinoma occurring from the annular pancreas. In patients with the annular pancreas, the possibility of coexistent malignancy in the pancreato-biliary system should be considered. EUS-FNA is most likely effective for the definitive diagnosis of pancreatic carcinoma occurring in the annular pancreas. For further elucidation of the annular pancreas, MRCP and the resected specimen cholangiopancreatography would be beneficial.


Osteoid osteoma is type of benign bone tumor, characterized by a well-demarcated core with a typical size of <1 cm and by a distinctive surrounding zone of reactive bone formation. The tumor can occur anywhere in the cortex or medulla of the skeleton. However, the lesion usually affects the long bones of the lower extremities. The present study describes two cases of osteoid osteomas located in the rib. Osteoid osteoma is a type of benign bone tumor, which was first described by Jaffe in 1935 . The maiA 22 year-old male presented with back pain in the right side that had been apparent for one year. The pain was intermittent and more severe at night, while it was reduced by taking aspirin. A local hospital was unable to establish a diagnosis and, thus, the patient was referred to the Beijing Jishuitan Hospital  in December 2011. A fixed tenderness point was identified on the right side of the back. An X-ray image revealed a radiolucent lesion located in the tenth rib of the right side, which exhibited prominent sclerosis surrounding a central radiolucent nidus . A compuIn February 2011, a 16 year-old female was admitted to the Beijing Jishuitan Hospital. The patient presented with back pain that had been apparent for six months. A slight curve towards the left side of the patient’s back was observed. The patient complained of pain at night that was reduced by taking aspirin. A fixed tenderness point was identified in close proximity to the spine. A CT scan revealed a lesion that was located at the top of the sixth right rib with calcification present in the center , while aTumors of the rib are uncommon, constituting only 5–10% of all bone neoplasms. Osteoid osteomas are benign osteoblastic neoplasms, which are characterized by a well-demarcated core with a typical size of <1 cm and by a distinctive surrounding zone of reactive bone formation . An osteThe most common types of tumors that affect the ribs are metastases and myelomas. Primary tumors of the ribs are uncommon. Therefore, the location of a tumor within the rib may help establish a differential diagnosis . CartilaIn total, <1% of osteoid osteomas affect the ribs . The defThe differential diagnosis of painful lesions located close to the ribs should consider tumors of the bone. Aneurysmal bone cysts of the posterior vertebral elements, eosinophilic granulomas and osteoid osteomas are the most commonly occurring lesions . Due to Complete surgical excision is the standard treatment method for osteoid osteoma and is usually offered to patients experiencing chronic and substantial pain that is not relieved by conservative treatment. In circumstances where excision is challenging, curettage is a good option. Removal of the nidus is the main aim of the treatment . Recent In conclusion, osteoid osteoma of the rib is an extremely rare condition. The typical clinical symptoms and characteristic imaging features often allow for a clear diagnosis. The method used for the treatment of osteoid osteoma depends on the tumor location.


Osteoblastoma is a rare benign osteogenic bone neoplasm; it represents 1 to 3% of all bone tumors and usually seen in the second decade of life with a predilection in males. Classically it develops in the vertebral column. The skull is rarely involved and the orbital roof was reported to be less frequently involved. We report a new case of a benign osteoblastoma arising from the orbital roof with an intracranial and intra orbital extension. A 28-year-old woman presented with a recent history of rapidly progressive left exophthalmos, unilateral vision loss and headache. Her medical history was unremarkable. At examination there was a downward proptosis of the left eye with complete ophthalmoplegia. Visual acuity was 20/200 in the left eye with an increased intraocular pressure. Funduscopy revealed subtotal optic nerve cupping. On computed tomography there was a well sumscribed hyperdense mass arising from the horizontal part of the frontal bone with intraorbital and anterior cranial fossa extension. Magnetic Resonance Imaging showed a homogeneous extraconal mass with low signal intensity on T1-weighted imaging, there were an anterior displacement of the left eyeball which was compressed by the tumor and a superior displacement of the left frontal lobe. This was consistent with a frontal bone osteoblastoma. A complete surgical removal was decided. This was performed within a left-sided subfrontal approach with extradural exposure of the anterior skull base. Histopathological examination revealed a mature benign osteoblastoma. Postoperatively the patient resolved her proptosis. However the visual acuity remained unchanged. The main differential diagnosis of osteoblastoma is osteoid osteoma. Osteablastoma typically involves the axial skeleton and exhibits a high growth potential, while the osteoid osteoma occurs in the long bones and is much less aggressive. On computed tomography osteoblastoma presents typically as a dense, sclerotic, homogenous mass with well-defined margins and thin cortical margin. On MRI the tumor generally shows low-signal intensity on both T1 and T2 weighted studies without gadolinium enhancement. Regardless of tumor location a total resection should be attempted to avoid local recurrences; which is pretty difficult when there is a cranial base extension. Adjuvant radiotherapy for residual tumor may then be discussed.


Ceruminomas are rare tumours arising from the apocrine sweat glands of the ear canal. We present a case of a malignant ceruminoma, which was managed with local surgical excision only rather than the wider clearance more commonly undertaken with these invasive neoplasms. We present the clinical case, histological analysis, and clinical progression for this patient and review the literature on this uncommon pathology. Ceruminoma is a rare tumour arising from the apocrine sweat glands of the ear canal. We present a case of a malignant ceruminoma, which was managed with local surgical excision only rather than the wider clearance more commonly undertaken. We discuss the histological analysis and clinical progression for this patient and review the literature on this uncommon pathology.A 39-year-old man presented in 1998 with an 18-month history of left-sided nonprogressive hearing loss. He had no symptoms of discharge, pain, tinnitus, or disequilibrium and was otherwise fit and well. On examination the left ear canal was occluded with a tumour arising from the superior aspect of the external auditory canal. The lesion did not blanch on compression or appear to pulsate on examination. Pure tone audiogram showed a mild to moderate conductive hearing loss in the left ear and normal hearing in the right ear.A Computed tomography (CT) scan of the temporal bones showed a well-defined soft-tissue mass in the external auditory canal extending into the middle ear. There was no distortion of the ossicles or facial nerve involvement. A magnetic resonance imaging (MRI) scan with gadolinium suggested that the lesion was not vascular in nature.Surgical exploration was carried out via an endaural approach and the tumour was excised leaving a complex perforation of the tympanic membrane. There was further tumour excised from the middle ear with preservation of the ossicular chain and the tympanic membrane was grafted with a temporalis fascia underlay graft.Postoperatively the patient's hearing improved such that he had normal hearing in the left ear with no conductive deficit. The surgically removed specimens were a 1 × 0.5 × 0.5 cm nodule from the ear canal and further fragments excised from the middle ear. All were analysed histologically. All specimens contained poorly differentiated, most probably ceruminous carcinoma.The patient was discussed at the joint clinicopathological multidisciplinary meeting and it was decided that the best clinical course of action would be a more radical surgical resection to clear the middle ear. This approach would likely cause further hearing loss.The patient declined any further surgical treatment and requested active observation (1999). He was followed up at regular intervals both clinically and radiologically. At the last review he was asymptomatic and disease free clinically and radiologically on MRI scan.The tumour showed a discohesive growth pattern comprising pleomorphic epithelioid cells possessing eccentrically located round or angulated nuclei with plentiful eosinophilic cytoplasm. “Indian file” configuration with nuclear moulding was present as were primitive trabecula and sparse tubuloglandular structures, in areas with associated stromal induction. Infrequent monomorphous solid islands exhibiting a few cribriform gaps indicate a possible in situ component.The cytoplasm was faintly positive with diastase periodic acid-Schiff (DPAS) staining and negative with Perl's stain. There was intense cytokeratin immunoreactivity with negative epithelial membrane antigen (EMA), S100 protein, and carcinoembryonic antigen (CEA). Oestrogen and progesterone receptor studies were equivocal.This profile supports apocrinoid differentiation and, arising in the ear canal, a primary ceruminous gland tumour is most likely.These tumours are uncommon and as such there is limited literature available on the topic and it is accepted that there is no extensive reporting by surgical pathologists available . These tCases of ceruminoma are rare; they have been seen to represent 0.00025% of all cancer cases and in one series just 2.4% of ear neoplasms that were referred , 2. A prCeruminous gland tumours present in a wide range of ages  from 21 The clinical course of the growth is most accurately determined by histological diagnosis as the signs and symptoms are not always correlated with the level of invasiveness . GeneralIn cases of invasive ceruminomas, aggressive surgery with postoperative radiotherapy is considered to be the best management due to the ability of these tumours to invade locally and destroy middle ear structures and the surrounding skull base , 6, 7. CThe importance of performing the right type of excision is notable, with recurrence of disease increasing the risk of mortality from 9% with no recurrence to 83% with recurrence , 2, 11.These tumours have the potential to metastasise to the lungs and to other glandular structures such as the parotid , 12, 13,Ceruminoma is an outmoded description that encompasses tumours of the ceruminous glands in the external auditory meatus. The ceruminous glands are modified apocrine sweat glands found in the deep dermis located in the cartilaginous outer two-thirds of the external auditory canal. The glands secrete a watery fluid that combines with the output of the sebaceous glands to form cerumen.There are various case reports and case series in the literature dating back a number of years, although large series are limited. In general the guidance is to regard all of these tumours as malignant, as the only marker of malignancy seems to be bony invasion. Clinical presentation in other cases is most commonly a mass in the external ear canal followed by hearing changes or facial nerve involvement. The sizes at diagnosis are extremely variable—up to 3 cm in one reported case.Mortality and morbidity for these tumours are variable and are worsened by extensive local invasion and systemic spread. In one case series the patients who died as a result of the disease were treated patients who experienced local recurrence. There are cases where there have been pulmonary metastasis—blood-borne spread has been seen to be a feature of adenoid cystic carcinoma.As mentioned above, hidradenomata is an accepted classification that encompasses these tumours. There are four histological subtypes—ceruminous adenoma, pleomorphic adenoma, adenoid cystic carcinoma, and ceruminous adenocarcinoma , 9. See Immunohistochemical staining typically shows cytokeratin (AE1/AE3 and LP34) 8/8 (100%), epithelial membrane antigen (EMA) 8/8 (100%), CK7  6/8 (75%), CK5/6  4/8 (50%), and S100 protein. Some cases have reported expression of tyrosine crystals, similar to what is seen in salivary gland tumours .This is a case of a rare invasive carcinoma of the external ear canal. This particular tumour was surgically managed with a close excision margin without any radiotherapy. This is contrary to the more conventional approach, which would be a radical excision with lateral temporal bone resection and blind pit closure of the ear canal. Other case reports have advised that this excision should be followed by postoperative radiotherapy  to preveThere are case reports that report progression of high-grade ceruminous adenocarcinomas showing metastatic progression to local lymph nodes and to the parotid. However distant metastases are rare and local invasion is the most common form of progression.Ceruminoma is a rare diagnosis and these tumours display varying differentiation and aggressiveness. Most authors promote wide-local excision for all lesions and further extensive excision with or without radiotherapy for the more aggressive lesions. This patient was treated with a local resection and a more conservative approach at the patients' request. This has proven successful; with over 10 years of follow-up there is no local or distant recurrence and he has conserved hearing in the ear affected by the tumour.This is surprising given the fact that the histology was poorly differentiated; one would expect this to be a more aggressive lesion with a high chance of local recurrence. It is therefore possible that some of these lesions even with evidence of invasion are not as aggressive as poorly differentiated carcinomas occurring elsewhere in the body are.Despite this good result we would still advocate a more radical approach to treatment for these lesions than was taken in this case.


Sacrococcygeal teratoma (SCT) is the most common congenital tumor in neonates. SCT may be benign (mature and immature) or malignant. Malignancy is rare in SCT diagnosed before 2 months of age. Here, we describe a neonate having a mature teratoma with a focus of malignant yolk sac tumor within and the difficulties faced in its management. SCT may be benign (mature and immature) or malignant. Malignancy is very rare in infants less than two months of age (7% in boys and 10% in girls) [1].Yolk sac tumors (YST) are rare and highly malignant tumors, occurring in children as well as in young adults. Although most germ cell tumors in children originate in the gonads, the most common primary site for YST is the sacrococcygeal region [2]. Here we describe a rare case of a mature teratoma with a focus of malignant yolk sac tumor within in a neonate and the difficulties faced in its management.A 2.2kg baby girl, born of a full-term vaginal delivery, presented to us on day 1 of life with a sacrococcygeal swelling. The neonate had passed urine and meconium and was breast feeding well. Local examination revealed 5 x 8 cm cystic, lobulated, trans-illuminant swelling extending up to anus, with the tip of coccyx protruding outwards [Figure.1]. There was no evidence of any neurological deficit.Antenatal ultrasounds in mother were unable to diagnose the tumor. Ultrasound of the sacrococcygeal region in baby showed evidence of a cystic lesion of 5.9 x 2.7cm size with spina bifida in L5-S1. Her alfa-feto protein level (40606ng/mL) were in the normal range for her age group  [4]. Non-contrast computed tomography (CT) scan of pelvis revealed a large hypodense cystic lesion in the subcutaneous tissue of lower back in close approximation with the lower sacral vertebra with evidence of dural ectasia [Figure 2].Intra-operatively, a 10x8x8 cm cystic swelling with a 0.5x0.5cm solid nodular component within was seen. There was no intra-pelvic extension. Surgical excision of the SCT with coccygectomy was done. Histopathological report revealed a mature teratoma with a small focus of yolk sac component measuring 0.5cm diameter, surrounded by a thin rim of fibrous tissue. Her metastatic work up was done, which included CT chest . AFP and beta Human Chorionic Gonadotropin levels after surgery were 1947ng/ml and less than 0.1mIU/ml .There was no consensus on the management in the tumor board; the pediatric oncologists favored administration of adjunct chemotherapy, whereas few pediatric surgeons and histopathologists favored ‘wait and watch’ policy. We opted in favor of chemotherapy as our patient came from a far distance and close surveillance was difficult. Before starting chemotherapy, her baseline hemogram, kidney and liver function were done. The baby was given three cycles of chemotherapy. After the first cycle of chemotherapy, the baby developed diarrhea, mucositis, electrolyte disturbances, febrile neutropenia and severe thrombocytopenia, for which appropriate therapy was given. The subsequent cycles she tolerated well with no side – effects. At 6 months follow-up, she is doing well.The imaging of sacral tumors in children is valuable to look for foci of malignancy. YST, consisting of fat-free soft tissue, is often complicated with hemorrhage, necrosis and cystic degeneration. Honeycomb-like change is an imaging characteristic of YST [4]. Loss of planes between tumor and surrounding tissue, sacral invasion and metastases are suggestive of malignancy. YST element was just a small focus in our case; therefore, such changes were not seen.AFP is a useful tumor marker for diagnosis and monitoring of therapy. However, AFP levels are normally increased in neonates and reach adult values by 8 months of age. Thus normal AFP levels for age should be taken into consideration while interpreting the results [3].The management of SCT is mainly surgical with complete excision of the tumor. The treatment of malignant SCTs, such as primary yolk sac tumor is dependent on the extent of disease. Local disease is best managed surgically, while advanced tumor stages benefit best from neoadjuvant platinum based chemotherapy followed by surgery. Survival rates using these strategies are higher than 80% [5].As per NCI guidelines, surgery and chemotherapy with four to six cycles of standard cisplatin, etoposide, and bleomycin (PEB) gives an overall survival (OS) outcome greater than 90% [6]. However, recent studies show that the JEB regime  gives a superior response as compared to PEB in the treatment of malignant SCT [7]; it is to be given for 4 courses or to be continued for 2 courses beyond documented Complete Response. Bleomycin is to be omitted in children less than 1 year age as per the UKCCSG GCII guidelines [8]. Another school of thought is to keep a regular follow up of patients having complete excision of the malignant focus with clear resection margins. In such cases, chemotherapy is only begun if there is evidence of recurrence. The rationale for this approach is to avoid cytotoxic chemotherapy in the neonatal range. However, the recurrence rate of malignant SCT is 33 percent [1]. Also, a CCG-POG study reported that four of six children with malignant recurrence after neonatal resection of mature or immature teratomas were noted to have microscopic foci of YST in the original specimen [9]. In the developing countries, where majority of the patients belong to poor families with low socioeconomic status and come from remote and far off places, it is very difficult for them to adhere to the strict and regular follow up required in such cases. As there is a high risk that these patients may be lost to follow up and present later with a large recurrence, we prefer to give four cycles of upfront chemotherapy (JEB regime) to our patient.Source of Support: NilConflict of Interest: The author is editor of the journal but he is not involved in decision making of the manuscript.


Gastrointestinal autonomic nerve tumor (GANT) is extremely rare and considered a variant of gastrointestinal stromal tumors (GISTs). GANT originates from the intestinal autonomic nervous system mostly of small intestine or the stomach. We report a colonic GANT diagnosed in a 5-year-old child who presented with abdominal pain and fever for a long period. Colonic resection and end to end anastomosis proved curative without the need of chemo-radiotherapy. Given the rarity of the tumor, the patient is on our long term follow-up. GANT is a variant GISTs with ultrastructural features of neural differentiation, and it is also known as plexosarcoma.[1] Clinically it presents with vague abdominal pain, a palpable abdominal mass, nausea or vomiting, hematochezia, anemia, and fever. Immunohistochemistry is required for the diagnosis demonstrating neuron like cells with axonal cytoplasmic processes and neurosecretory granules. Most of the GANTs arise from the small intestine or stomach. Here we report the management of a colonic GANT diagnosed in a young child.A 5-year-old girl, suffering from persistent abdominal pain and fever for a month, was transferred from another hospital for further management. She complained of severe recurrent diffuse abdominal pain associated with fever. A firm mass was palpable in the left iliac region. Laboratory tests showed neutrophilic leucocytosis and moderate elevation of C-reactive protein, compatible with an infective etiology. Ultrasound abdomen did not provide much information about the nature of the pathology therefore CT scan with contrast was performed which showed a hyperdense mass occupying the lumen of the descending colon. At laparotomy, a 6 cm long intraluminal firm mass was found in the descending colon. The colonic serosa was intact. The colon with the mass was adherent with the peritoneum, omentum, and the kidney capsule. After adhesionolysis, the segmental resection of the colonic portion containing the mass was performed with 2 cm tumor free margins on either side. An end-to-end colo-colonic anastomosis was performed and the resected colon and a small portion of the perirenal fat were sent for histopathological examination. The postoperative course was uneventful and the child was discharged on postoperative day five. The 7-month follow-up was also uneventful.Histopathological examination showed a diffuse tumour within the muscularis propria, made of either pleomorphic cells with eosinophilic cytoplasm and irregular nuclei or fusiform cells with wavy nuclei. Mitotic activity was very low and surgical margins were negative for tumor cells. At immunohistochemistry, the lesion was positive for Vimentin, S 100, CD 34, Caldesmone; weakly positive for CD 30 and Actin; negative for EMA, CD 117 (c-kit), CD 68, HMB 45, ALK, Cytokeratin, AE1 / AE3, Desmin, CD 21, Neu N . These findings were consistent with a mesenchymal neoplasm originating from the nerve sheaths .GANTs usually arise in the small intestine or stomach but other reported sites are in the mesentery and the retroperitoneum. These tumors are malignant in the adults especially occurring in stomach with the size more than 5 cm. Six pediatric cases have been described  with the primary tumor sited in the stomach in all cases except for the mesenteric involvement in a girl. Clinical features at presentation are non-specific: the most common presentation is abdominal pain and fullness, fever, gastrointestinal bleeding, chronic iron deficiency anaemia, weight loss, and malaise. In our case, high grade persistent fever and recurrent diffuse abdominal pain were the dominant presenting features.At histology the lesion may be spindled or epithelioid and is characterized by complex long thin interdigitating cell processes that are joined together by rudimentary cell junctions. Immunohistochemical features are essentially the same as GIST. Some reports have described chromogranin and synaptophysin staining; Neuron Specific Enolase (NSE) is always positive but is not considered a specific marker. The presence of CD34 is reported in all cases, while the S100 protein is present in half of the patients.[2] Muscle markers and vimentin are occasionally reported and genetic studies revealed mutations in KIT gene.[4]Radical surgical resection with tumor free margins seems curative approach, and long-term survival without recurrence is possible even with large sized tumors.[2] In all pediatric cases, the initial treatment was surgical resection; chemotherapy was administered to one patient on recurrence.[2] However various chemotherapy regimens have been utilized as first-line therapy in adult patients with metastatic/recurrent disease.[5] Radiation therapy has also been utilized in patients with recurrent and/or metastatic disease.[2]GANTs are extremely rare tumours; in children these are more common in females having smaller lesions and good prognosis with surgical excision compared with adult counterparts. Tumor excision is considered the optimal therapy in children, but a long term follow-up is required to pick the recurrence.Source of Support: NilConflict of Interest: None declared


Endometriosis causes rare complications in pregnancy, such as obstetrical bleeding, preterm birth, spontaneous haemoperitoneum, and intestinal perforation. The prevalence of spontaneous perforation due to intestinal endometriosis is unknown in pregnancy. A recent review of the literature indicated 15 bowel complications caused by endometriosis during pregnancy or at the immediate postpartum period. The diagnosis of a bowel perforation can be difficult and in all of the cases reported necessitates an exploratory laparotomy. Anyway, in the majority of cases bowel perforation is not diagnosed during this laparotomy, and a repeat laparotomy is needed. Laparoscopy is being used increasingly in the diagnosis and operative management of acute abdomen. Laparoscopy can be a useful means of diagnosis and in addition a therapeutic tool in selected pregnant patients with abdominal pain. We report the first case of a pregnant woman with spontaneous double sigmoid and rectal perforation from decidualized endometriosis diagnosed by laparoscopy. Endometriosis is characterized by benign proliferation of ectopic endometrial glands and stroma in the peritoneal cavity, resulting in inflammation and scarring, often leading to pelvic pain and infertility . It affe Escherichia coli, Enterobacter cloacae, and Enterococcus faecalis were detected in the intraperitoneal fluid culture. Patient was treated with Meropenem 1 gr i.v. every 8 hours for 10 days and she was discharged on postoperative day 12 without any other complications. Physiological fetal growth and an uneventful antenatal period were reported until 41 weeks of gestation when a cesarean section was performed for unsuccessful vaginal delivery induction. The patient delivered a healthy female baby weighing 3750 gr with Apgar scores of 8 and 9 at one and five minutes, respectively. Colostomy was closed after 3 months without any complications.A healthy 32-year-old Caucasian woman was admitted to emergency room with acute diffuse abdominal and pelvic pain, started 4 hours previously, at 25 weeks of pregnancy. She had an uncomplicated pregnancy. She had no reported history of endometriosis and previous abdominal surgery. Physical examination led to a diagnosis of pelvic peritonitis. Upon admission, temperature was 37.7°C and blood pressure was 110/62 mmHg. Laboratory tests revealed a white blood cell count of 18.500/mL, hemoglobin of 10.4 g/dL, and C-reactive protein (CRP) of 4.02 mg/dL . Abdominal ultrasound revealed minimal fluid around the liver, but neither air nor gross fluid was visible in the abdominal cavity. The obstetric evaluation ruled out an obstetric aetiology of patient's symptoms. The patient underwent an emergency operation for a presumptive diagnosis of acute diffuse peritonitis. Explorative laparoscopy was useful to confirm the clinical suspicion and revealed diffuse severe purulent peritonitis that originated from a double rectal and sigmoid perforation <1 cm in diameter related to an endometriotic nodule, that was confirmed in histopathological findings. A previous adherence, caused by endometriotic nodule, and the progressive traction of the enlarged uterus on the strictly adherent sigmoid colon could be a possible aetiopathogenetic mechanism. The surgical approach, for reduced intraperitoneal operative space, was continued through a median laparotomy. Purulent fluid was found in the abdominal cavity, and with further investigation a left sided sigmoid perforation was found associated with another perforation in rectosigmoid junction that was closed with linear stapler and sigmoid in site of perforation was chosen as stoma in left lateral abdominal wall. The patient tolerated the procedure well . The fetThe incidence of acute abdomen during pregnancy is 1 in 500–635 pregnancies . DespiteBowel perforation linked to endometriosis is a rare cause of the acute abdomen during pregnancy; there are only small number of reported cases: 12 cases of intestinal perforation due to endometriosis that was not associated with pregnancy and 13 cases occurred in pregnancy or at the immediate postpartum period –19.Previous surgery for deep pelvic endometriosis or IVF treatment seems to be associated with this complication –19.In our case there are not risk factors that could predict bowel complications.Perforations must be round or ovoid >1 cm in diameter, and must be antimesenteric in location.Fecal masses must be present within either the colon or abdominal cavity.Pressure necrosis or ulcer and chronic inflammatory reaction around the perforation site must be present microscopically.All of the above must be featured in the absence of any other active colonic pathology.The pathophysiology of endometriosis induced bowel perforation is not clear. Perforation of the bowel  is often due to pressure necrosis from fecal masses. In July 2000, Maurer et al. proposed a set of diagnostic criteria to differentiate stercoral perforation from other causes of bowel perforation [In our case one of possible mechanisms for large bowel perforation was due to increased abdominal pressure and also was facilitated by the progressive traction of the enlarged uterus on the strictly adherent between sigmoid-colon-recto and endometriotic nodule and consequently evolved in double rectosigmoid perforations.In addiction in pregnancy endometriotic nodule becomes decidualized with progressive reduction in size that can lead to perforation by weakening intestinal wall.Although endometriosis improves during pregnancy the current report shows the potential occurrence of serious and unexpected complications of the disease.Both the rareness of the perforation and the symptoms that are suggestive of pyelonephritis or diverticulitis may be misleading and delay the diagnosis.The diagnosis of a bowel perforation in all of cases reported necessitates an exploratory laparotomy but in the majority of cases bowel perforation is not diagnosed during this laparotomy and a new laparotomy is needed .Laparoscopy can be useful means of diagnosis and a therapeutic tool and should be considered instead of laparotomy in selected pregnant patients with abdominal pain.We report the first case of a pregnant woman with spontaneous double sigmoid and rectal perforation from decidualized endometriosis diagnosed by laparoscopy without cesarean section delivery at that time.Indeed, the appropriate management of these patients may be challenging and a good outcome is absolutely dependent on a multidisciplinary approach.


We report on a case of pancreatic arteriovenous malformation (PAVM) that obliterated shortly after diagnostic angiography (DSA). PAVM is a rare anomaly that presents with upper abdominal pain, signs of acute pancreatitis and massive gastrointestinal bleeding. The management of PAVM is rather complex, with complete treatment usually accomplished only by a total extirpation of the affected organ or at least its involved portion. DSA prior to treatment decisions is helpful for characterizing symptomatic PAVM, since it can clearly depict the related vascular networks. In addition, interventional therapy can be performed immediately after diagnosis.A 39-old male was admitted due to recurring upper abdominal pain that lasted several weeks. Initial examination revealed the absence of fever or jaundice, and the laboratory tests, including that for pancreatic enzymes, were unremarkable. An abdominal ultrasound (US) showed morphological and Doppler anomalies in the pancreas that were consistent with a vascular formation. A subsequent DSA depicted a medium-sized nidus, receiving blood supply from multiple origins but with no dominant artery. Coil embolization was not possible due to the small caliber of the feeding vessels. In addition, sclerotherapy was not performed so as to avoid an unnecessary wash out to the non-targeted duodenum. Consequently, the patient received no specific treatment for his symptomatic PAVM. A large increase in pancreatic enzymes was noticed shortly after the DSA procedure. Imaging follow-up by means of CT and MRI showed small amounts of peripancreatic fluid along with a limited area of intra-parenchymal necrosis, indicating necrotizing pancreatitis. In the post-angiography follow-up the patient was hemodynamically stable the entire time and was treated conservatively. The symptoms of pancreatitis improved over a few days, and the laboratory findings returned to normal ranges. Long-term follow-up by way of a contrast-enhanced CT revealed no recanalization of the thrombosed PAVM.The factors associated with the obliteration of PAVM during or after DSA are poorly understood. In our case it may be attributed to the low flow dynamics of PAVM, as well as to the local administration of a contrast agent. Asymptomatic PAVM, as diagnosed with non-invasive imaging techniques, should not be evaluated with DSA due to the potential risk of severe complications, such as acute pancreatitis. Pancreatic arteriovenous malformation is a rare anomaly, which can be congenital or acquired by way of inflammation, tumor or trauma. Most patients with PAVM remain asymptomatic and have progressive portal hypertension in the absence of primary liver pathology. Symptomatic PAVM commonly presents with upper abdominal pain, signs of acute pancreatitis and gastrointestinal bleeding , 2. The Treatment of PAVM is indicated only in symptomatic patients, who are the minority. The management of symptomatic PAVM is complex and involves a multidisciplinary approach with complete treatment usually accomplished only by a total extirpation of the affected organ or at least the portion involved. Although radical pancreatic surgery treats PAVM, it usually significantly affects the metabolic characteristics of the radically or totally pancreatectomized patient, in whom persistent malabsorption, deficiencies in fat-soluble vitamin and pancreatogenic diabetes are observed. Post-operative adverse chronic sequelae may also occur, including liver disease with unusual frequency, characterized by accelerated fatty infiltration and osteopenia, along with a reduction in radial bone mineral content in pancreatectomized patients within no more than 5 years after surgery .In recent years, transcatheter arterial embolization (TAE), an alternative to surgical treatment, has been performed as a conservative approach. Its use is rather limited due to the etiopathogenesis of PAVM as well as the adverse effects of embolization procedures , 6. DiagIn this paper we report a case of PAVM in a patient who underwent DSA with subsequent obliteration and thrombosis of the lesion and resulting acute pancreatitis.A 39-old male was admitted to our hospital due to several weeks of recurring upper abdominal pain. Initial examination revealed the absence of fever or jaundice, and the patient reported no major involuntary weight loss. No relevant family or past disease history or bleeding tendency was found. The initial laboratory tests, including that for pancreatic enzymes, were unremarkable. An esophagogastroduodenoscopy, performed few days earlier, revealed small mucosal breaks consistent with grade A esophagitis according to the Los Angeles Classification .B-mode ultrasound (US) showed a structurally heterogeneous and thickened neck of the pancreas, which was poorly differentiated from the surrounding fat Fig. . No dilaVascular access was obtained by a retrograde puncture of the femoral artery along with the introduction of a 5Fr access sheath. Selective catheterization and angiography of the celiac trunk and the superior mesenteric artery were performed by using a 5Fr Simmons sidewinder catheter Sim 1 . Superselective catheterization and angiography of the feeder arteries’ microcatheter were performed by a Progreat 2.4Fr  with 0.016˝ Radifocus Guide wire GT . The non-ionic, iso-osmolar contrast agent iodixanol 320 mg/ml  was used for the angiography in the total amount of 100 ml. The selective angiography of the celiac trunk and the superior mesenteric artery were performed with 20 ml at 5 ml/s, while super-selective angiography of feeder arteries was performed with 5–10 ml at 1 ml/s.The celiacography and selective angiography of the upper mesenteric artery revealed that the PAVM located in the neck of the pancreas was receiving arterial blood supply from four origins: a medium-sized feeding artery branching from a common hepatic artery , or no treatment at all –12. SurgIn the presented case, the PAVM, characterized by way of DSA, was relatively small in size, however, branching from numerous small-caliber feeding arterioles. No dominant arteries were found; therefore, coil embolization was not possible. Additionally, TAE by means of ethylene-vinyl alcohol copolymer  was discussed as the only treatment option. In keeping with a conservative treatment approach, it was not performed so as to avoid any undesired wash out of the infused agent to the non-targeted duodenum, which could possibly cause necrosis. Sclerotherapy was also not performed, since we lacked precise initial diagnostic imaging of the vascular malformation, which would have allowed a characterization of its flow dynamics, i.e. high-flow from low-flow. In the end, the patient received no specific treatment at all.Laboratory testing as well as imaging follow-up studies suggested that an obliteration of the PAVM appeared shortly after DSA and was followed by acute pancreatitis in accord with diagnostic criteria . SeveralIn our case, DSA was most likely responsible for the obliteration of the PAVM, however, it also induced pancreatitis, evidenced by subsequent laboratory and imaging findings. A diagnosis of acute pancreatitis is usually made on the basis of clinical and laboratory findings, therefore our follow-up imaging (US and MRI) was primarily used to monitor possible early complications, e.g. revascularization of the thrombosed PAVM. However, clinically this phenomenon seems to be exceedingly rare. In the post-angiography follow-up the patient received no additional treatment for the symptomatic PAVM. The patient was hemodynamically stable at all times and was treated conservatively. The emphasis of our conservative treatment was on supportive measures and on the prevention of the infection of necrosis and other complications. Supportive nonsurgical management was indicated mainly because of the improved symptoms of pancreatitis over a few days and the fact that laboratory findings returned to normal ranges.The widespread use of imaging techniques seems to be the exact reason for the increased number of reported PAVM cases in recent years. Besides PAVM detection, discrimination of flow characteristics of vascular malformations is also thought to play an essential role in determining appropriate treatment , i.e. suDiagnostic angiography prior to treatment decisions is beneficial for patients with symptomatic PAVM, since it can clearly depict a network of vascular malformation. In addition, subsequent interventional therapy can be performed immediately after DSA. Follow-up after DSA is necessary due to complications that may occur as a result of hardware manipulation or vascular spasm during the angiographic procedure. In our case, the factors associated with the obliteration of PAVM after DSA are not entirely understood, though the obliteration may be attributed to the low flow dynamics of the small caliber PAVM as well as to the local administration of the contrast agent. We therefore conclude that when PAVM is asymptomatic and diagnosed with non-invasive imaging techniques it should not be evaluated with DSA, in light of the potential risk of the severe complications of acute pancreatitis.CT, computed tomography; DSA, diagnostic angiography; MRI, magnetic resonance imaging; PAVM, pancreatic arteriovenous malformation; TAE, transcatheter arterial embolization; US, ultrasound


Primary gastric actinomycosis is extremely rare, the appendix and ileocecal region being the most commonly involved sites in abdominopelvic actinomycosis. Herein, we report a case of primary gastric actinomycosis. The diagnosis was made on microscopic evaluation of gastroscopic biopsy specimens. To the best of our knowledge, this is the third case to be reported in the literature, in which the diagnosis was made in a gastroscopic biopsy rather than a resection specimen.Actinomyces.An 87-year-old Saudi male on medication for cardiomyopathy, premature ventricular contractions, renal impairment, hypertension, and dyslipidemia, presented to the emergency department with acute diffuse abdominal pain, abdominal distension, constipation and vomiting for two days, with no history of fever, abdominal surgery or trauma. The patient was admitted to the hospital with an impression of gastric outlet obstruction. Based on radiologic and gastroscopic findings, a non-infectious etiology was suspected, possibly adenocarcinoma or lymphoma. Gastroscopic biopsies showed an actively inflamed, focally ulcerated atrophic fundic mucosa along with fragments of a fibrinopurulent exudate containing brownish, iron negative pigment and abundant filamentous bacteria, morphologically consistent with Althuogh extremely rare, primary gastric actinomycosis should be considered in the differential diagnosis of radiologic and gastroscopic diffuse gastric wall thickening and submucosal tumor-like or infiltrative lesions, particularly in patients with history of abdominal surgery or trauma, or those receiving extensive medication. A high level of suspicion is required by the pathologist to achieve diagnosis in gastroscopic biopsies. Subtle changes such as the presence of a pigmented inflammatory exudate should alert the pathologist to perform appropriate special stains to reveal the causative organism. Actinomyces species, most often Actinomyces israelii. There are three main forms of actinomycosis, namely, cervicofacial (31%-65%), abdominopelvic (20%-36%) and thoracic (15%-30%) [Actinomycosis is a chronic suppurative granulomatous inflammation caused by anaerobic, filamentous, Gram-positive bacteria of ofacial 3%-65%, ab15%-30%) . In abdonopelvic 0%-36% an15%-30%) . Primary15%-30%) -20. Here15%-30%) .Actinomyces , mild normocytic normochromic anemia , elevated serum lipase (1123 U/L), amylase (269 U/L), and creatinine (1.4 mg/dl), and low potassium (3.1 mEq/L). Plain abdominal X-ray showed a markedly dilated stomach  alerted us to the possibility of actinomycosis which was established by appropriate special staining that revealed the microorganisms. It is well known that the main sources of natural pigments are plants and microorganisms, including Actinomycets [Because of the submucosal localization of the inflammatory process, gastroscopic biopsy specimens usually reveal nonspecific inflammatory changes ,14,18,19nomycets .Streptococcus viridans, another endogenous aerobic/anaerobic facultative commensal present in the oral and GI-tract flora [Actinomyces infection with no necessity for culture confirmation.Culturing is negative in most cases of gastric actinomycosis (>76%) ,24,25. Ict flora . DespiteStreptococcus viridans, known to be an organism of low virulence, had contributed to the gastritis in our case remains unclear.Most anaerobic bacteria recovered from clinical infections are found mixed with other anaerobic organisms . PolymicPrimary gastric actinomycosis is an indolent infection. If the disease is recognized, the prognosis is good because antibiotic treatment, particularly penecillin is very effective ,19. Our Althuogh extremely rare, primary gastric actinomycosis should be considered in the differential diagnosis of radiologic and gastroscopic diffuse gastric wall thickening and submucosal tumor-like or infiltrative lesions, particularly in patients with history of abdominal surgery or trauma or those receiving extensive medication. A high level of suspicion is required by the pathologist to achieve diagnosis in gastroscopic biopsies. Subtle changes such as the presence of a pigmented inflammatory exudate should alert the pathologist to perform appropriate special stains to reveal the causative organism.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.