A 45-year-old female was diagnosed as having lung adenocarcinoma harboring an anaplastic lymphoma kinase (ALK) rearrangement, stage IV (T2bN3M1b). She was treated with crizotinib as second-line chemotherapy. The clinical stage after crizotinib treatment was ycT2aN0M0, stage IB. We performed a left lower lobectomy and lymph node dissection aimed at local control and pathological confirmation of the remaining tumor. The final pathological stage was ypT2aN2M0, stage IIIA with Ef 1b. To the best of our knowledge, this is the first case report of surgical resection in ALK rearrangement-positive lung adenocarcinoma after crizotinib treatment. Rearrangements of the ALK gene are present in 3% to 5% of non-small cell lung cancers (NSCLCs) ,2. They Crizotinib is an oral small-molecule tyrosine kinase inhibitor (TKI) of ALK, MET, and ROS1 kinases . CrizotiIn phase 1 and 2 studies, crizotinib treatment resulted in objective tumor responses in approximately 60% of patients with ALK-positive NSCLC and in progression-free survival of 7 to 10 months . In a raHowever, there has been no data about the preoperative crizotinib treatment in NSCLC patients. We report the case of a 45-year-old female who underwent surgical resection after crizotinib treatment in stage IV NSCLC.TNM Classification of the International Union Against Cancer (UICC), 7th edition, and the patient was considered to have inoperable T2bN3M1b. stage IV disease. We evaluated the responsiveness of tumor to chemotherapy, based on Response Evaluation Criteria in Solid Tumors (RECIST), version 1.1.A 45-year-old female, current smoker with no significant medical history, was found to have a left lung mass in July 2012. Chest computed tomography (CT) revealed a 58-mm-diameter mass in the left lower lobe and bilateral hilar and mediastinal lymphadenopathy. Positron emission tomography (PET)-CT showed FDG accumulation in the lesions mentioned above and in the left axillary lymph node. The serum carcinoembryonic antigen (CEA) level was high as 71.0 ng/ml. Transbronchial biopsy of the primary lesion revealed adenocarcinoma. Head magnetic resonance imaging (MRI) was negative for metastases. The clinical stage was determined based on the In August 2012, chemotherapy with cisplatin and pemetrexed was initiated, resulting in stable disease after four courses, followed by seven cycles of pemetrexed continuation maintenance therapy. Although she achieved stable disease for approximately 9 months, cancer regrowth occurred at the right mediastinal lymph nodes  revealed that the tumor had an ALK rearrangement  and fluorescent t Figure A,B. Crizt Figure B. A partRecently, several authors reported surgical treatment, which can be performed for local control and diagnostic intent after epidermal growth factor receptor (EGFR)-TKI gefitinib administration, showing long-term survival in some patients ,13. And Crizotinib is an oral small-molecule TKI targeting ALK. The present patient with ALK rearrangement-positive NSCLC showed pronounced and rapid regression of tumor only 6 months after starting crizotinib. Despite the remarkable downstaging after crizotinib treatment, the patient had a more advanced pathological stage than the preoperative clinical stage. Dramatic radiologic response does not necessarily correlate with cell death. The present result suggests initially expressed systemic disease was essentially unchanged even after dramatic radiologic response to crizotinib. Stage IV lung cancer is considered to be a systemic disease; therefore, surgical treatment is rarely viewed as a feasible option. Nevertheless, some reports indicate near-complete pathological response of advanced NSCLC after induction chemotherapy ,14. SystWe report a case of surgical resection of lung adenocarcinoma after crizotinib treatment. The present report provides insight into the efficacy of surgery after crizotinib therapy, but a prospective study is needed to verify this and the optimal duration of ALK-TKI treatment, the timing of surgery, and the role of adjuvant ALK-TKI treatment.Written informed consent was obtained from the patient for the publication of this case presentation and accompanying images. A copy of the written consent is available for the review by the Editor-in-Chief of this journal.


Segmental dilatation of the intestine in pediatric age group is a rare entity. Patients usually present with partial intestinal obstruction which may delay surgical decision. Our case was an 18-month-old girl, who presented with partial intestinal obstruction, provisionally diagnosed as a case of Hirschsprung’s disease. Diagnostic evaluation with contrast study gave a clue of small intestinal obstruction with a dilated segment. Segmental dilatation of the intestine (SDI) is a condition characterized by a sharply defined and markedly dilated segment of the intestine flanked by normal-caliber afferent and efferent bowels. Although segmental dilatation can involve the gastrointestinal tract anywhere from duodenum to distal colon, ileum is the most commonly affected site.[1] It is a rare cause of intestinal obstruction in neonates and children with a few cases described in the literature.[2] We report a case of segmental dilatation of ileum in a child.An 18-month-old girl presented with abdominal distension, bilious vomiting and failure to pass stool for 2 days. There was a history of off and on abdominal distention and episodes of vomiting with spontaneous resolution since birth. There was no history of delayed passage of meconium or failure to thrive. On examination, abdomen was hugely distended, soft, and with no tenderness. Per rectal examination was unremarkable. Abdominal x-ray showed a big air fluid level occupying the entire abdomen associated with multiple other air fluid levels. Blood investigations were normal. A nasogastric tube was inserted and IV fluids were started. The patient was kept under observation. She passed stool occasionally but abdomen remained distended with persistent bilious aspirate. Repeat radiograph showed same findings. Contrast enema showed a non distended colon localized in the left part of the abdomen with a hugely distended loop occupying the right flank and iliac fossa .Exploratory laparotomy showed a segmental dilatation of small bowl, 70cm proximal to ileocecal junction, which was about 13 cm x 10 cm in size, and the affected section of the intestine was dilated regionally to six times the normal value in diverticular fashion . The dilated segment was in continuity with normal caliber distal bowel and mildly dilated proximal bowel and was twisted 90 degrees but viable. There was associated malrotation present. The distended loop was resected and ileo-ileal anastomosis done. Ladd’s procedure was also added. Postoperatively, the patient did well. Histopathology showed chronic inflammation of both mucosa and submucosa, with superficial ulceration without evidence of abnormal heterotopic gastric mucosa and with the presence of normal ganglion cells. The resected appendix was normal.SDI is an uncommon entity described for the first time in the colon by Swenson and Rathauser in 1959. SDI can affect colon, ileum, and jejunum.[3]Criteria for diagnosis of this entity, as proposed by Swenson and Rathauser are (1) Limited bowel dilatation with 3- to 4-fold increased size.(2) Abrupt transition between dilated and normal bowel.(3) No intrinsic or extrinsic barrier distal to the dilatation.(4) Clinical picture of intestinal occlusion or sub-occlusion.(5) Normality of neuronal plexus, and(6) Complete recovery after resection of the affected segment.SDI can present as an isolated entity or may be associated with other congenital malformations. These associated malformations are found in more than 50% of the cases. Digestive malformations are essentially represented by disorders of rotation and fixation, as in our case where malrotation was found. Omphalocele is presumed to play a role in its etiogenesis.[2]Clinical presentation depends upon site of SDI and age of presentation. More than 50% of the SDIs occur in neonatal period. The diagnosis is most frequently made at surgical intervention. Equally, the SDI can be possibly revealed through a complication: mainly a bowel obstruction. Cases involving the colon have a clinical picture very similar to that of Hirschsprung’s disease. Beyond the neonatal period, the SDI can present with chronic abdominal pain, distention and anemia, which would follow the ulceration of the dilated loop.Radiologic findings are not specific and abdominal x-ray shows signs of intestinal obstruction with one big fixed distended loop with hydroaeric level. A contrast x-ray study can identify the dilated segment. Computed tomography may show a large saccular dilatation of a bowel loop, containing a mixture of oral contrast material and feces. The usual finding on laparotomy is a localized dilation of an isolated, well defined segment of bowel with apparently normal bowel proximal and distal to this segment. The obstruction in these cases is functional and non-mechanical because the lumen of the dilated segment is continuous with rest of the intestine as in our case.[6] In conclusion, SDI should be included among the differential diagnoses of intestinal obstruction in neonates and children especially if there is a hugely distended loop on abdominal x-ray.Source of Support: NilConflict of Interest: None declared


To explore the clinical characteristics and pathological features of epithelioid inflammatory myofibroblastic sarcoma (EIMS) with emphasis on the diagnostic spectrum.The clinical data and histological features in 5 additional cases of EIMS were retrospectively reviewed. Immunohistochemical study and interphase fluorescence in situ hybridization (FISH) analysis were carried out.There were 2 males and 3 females with age at presentation ranging from 15 to 58 years . All 5 tumors were intra-abdominal with 2 arising in the mesentery and 1 each in the omentum, rectum and transverse colon. The tumor size ranged from 5 to 20 cm in maximum diameter . Histologically, all 5 tumors were composed predominantly of large epithelioid cells possessing vesicular nuclei, prominent nucleoli, and amphophilic cytoplasm. Mitotic figures were easily identified . Tumor cells were arranged in clusters or sheets embedded in a myxoid stroma containing prominent neutrophils. A minor component of spindle cells was present in focal areas. By immunohistochemistry, all 5 cases were positive for anaplastic lymphoma kinase (ALK) with a nuclear membrane pattern in 4 and cytoplasmic staining with perinuclear accentuation in 1. Besides ALK, tumor cells stained variably for desmin (4/5), alpha smooth muscle actin (2/5), muscle-specific actin (1/2) and pan-cytokeratin (1/4). FISH analysis demonstrated the presence of ALK rearrangement in all 5 cases. Of 5 patients, 3 developed local recurrence, 1 died of disease 8 months after surgery.EIMS represents a highly aggressive variant of inflammatory myofibroblastic tumor characterized by epithelioid morphology, prominent neutrophilic infiltrate, and nuclear membrane staining of ALK with ALK rearrangement. As patients with ALK-rearrangement tumors may benefit from targeted therapy, accurate diagnosis of EIMS is very important. Familiar with the characteristic features of EIMS will help pathologists avoid misdiagnosing the tumor as other malignancies. IMT usuIn 2011, Mariño-Enríquez et al.  describeFive cases of EIMS were retrieved from the archive files of the Department of Pathology, Fudan University Shanghai Cancer Center. The cases were initially diagnosed between 2012 and 2015 and were all consultation cases. The clinical and follow-up data were obtained from the electronic medical records, hospital discharge summary or by telephone inquiry. All available hematoxylin-and-eosin (H&E) slides were reassessed for cytomorphology, mitotic activity, composition of inflammatory infiltrate, stromal change, and presence of necrosis.Immunohistochemical study was performed on paraffin-embedded sections on Ventana Benchmark XT autostainer (Roche). The primary antibodies used in the study included desmin , alpha smooth muscle actin , muscle-specific actin , H-caldesmon , ALK , CD30 , vimentin , S100 protein , pan-cytokeratin, epithelial membrane antigen , myogenin, CD117 , discovered on GIST-1 (DOG1) , CD34 and Ki-67. Appropriate positive and negative controls were run simultaneously for all antibodies tested.Interphase fluorescence in situ hybridization analysis was carried out on 5-μm-thick sections of formalin-fixed, paraffin-embedded tissue in 5 cases, according to the manufacturer’s protocol. The presence of ALK gene rearrangement at 2p23 was tested using the LSI ALK dual-color break-apart probe . The fluorescence signals were analyzed using an Olympus BX51 fluorescence microscope . A total of 100 nuclei were evaluated from each specimen.A 37-year-old woman went to the clinic of a local hospital because of abdominal pain and hematochezia for one week. Colonoscopy examination showed a polypoid mass protruding into the rectum cavity, measuring approximately 5 cm in maximum diameter, with a rough and uneven surface Fig. . Then thThe patient was a 55-year-old man who complained of abdominal pain and distension lasting for two days. Physical examination showed abdominal mass with tenderness. No obvious mass was found by colonoscopy. X-ray displayed intestinal obstruction. Computed tomography (CT) scan demonstrated a solid mass in the pelvic cavity Fig. . ClinicaA 22-year-old young man presented with abdominal mass with intermittent abdominal pain for eight days and fever for five days. Subsequent CT scan revealed a solid heterogeneous mass of the right abdominal cavity, suspicious of GIST. After the admission, the patient underwent a complete excision of the abdomen mass and partial transverse colectomy soon. Intraoperatively, an enormous mass measuring approximately 20 × 15 cm was identified, with multiple small omental or mesenteric nodules. Pathological examination of the submitted specimen showed that malignant tumor was suspected as GIST. To confirm the diagnosis, the pathological materials were sent to our department for further consideration. With the adjunctive study of immunohistochemistry and FISH, the lesion was re-diagnosed as EIMS. The patient developed local recurrence 2 months after surgery. The targeted therapy of ALK inhibitor (crizotinib) was administrated later. He achieved a partial response to the treatment. He was alive with disease at 14-month follow up.The patient was a 58-year-old woman who complained of a slowly enlarging and painless mass on the abdomen for two months. Physical examination showed an ill-defined solid mass of the right lower abdominal wall with no tenderness. Then she received a local resection. Intraoperative examination revealed that the mass was firm in consistency and infiltrated into the skeletal muscle of the abdomen wall. Pathologically, the tumor was considered as pleomorphic sarcoma by the referring pathologist. After surgery, the patient was treated with the additional chemotherapy. She developed local recurrence 2 months later and died of disease at 8-month follow-up.A 15-year-old young woman complained of recurrent abdominal pain for four months and abdominal mass for four days. Physical examination revealed a well-defined solid mass of the right abdomen with mild tenderness. Ultrasonography displayed an abdominal tumor with heterogeneous internal echoes, measuring about 10.2 × 7.5 cm. Subsequently, the patient received a complete excision of the abdomen mass and partial transverse colectomy. During the operation, the tumor was identified on the transverse colon and measured approximately 12 × 8 cm, with multiple enlarged omental or mesenteric lymph nodes. The lesion was considered as fibrosarcoma with inflammatory cell infiltrates by the referring pathologist. After surgery, the patient did not receive the adjunctive therapy. She was free of disease at 7-month follow up.Tumor size ranged from 5 to 20 cm in maximum diameter . On cut section, the tumors were grayish-white to pink-tan in color, moderate or fleshy in consistency, with myxoid appearance in some areas.On low power, the tumor displayed a polypoid appearance in case 1, which was mainly located on the submucosa and infiltrating the muscularis. In case 2 and 3, the masses invaded the intestinal wall Fig. . In caseImmunohistochemically, all 5 tumors were strongly positive for ALK, with nuclear membrane pattern in 4 cases Fig. , and cytBy FISH analysis, all 5 tumors showed ALK gene rearrangement, as indicated by the presence of isolated green (centromeric) and orange (telomeric) signals flanking the ALK locus at 2p23 Fig. .IMT is a well-described entity with a predilection for children and adolescents and occurs predominantly in the upper respiratory tract, lung and abdomen. Histologically, it is composed of spindled fibroblasts and myofibroblasts with occasional “ganglion-like” cells present in the tumor. IMT with prominent epithelioid cytomorphology is very rare. In a study with 73 cases of IMT, Cook et al. found 4 cases that exhibited round cell transformation characterized by large polygonal cells with large nuclei and prominent nucleoli, in a loose, pale staining background . In contAs EIMS has not been widely recognized, the incidence remains underestimated. To the best of our knowledge, only about 20 cases have been documented in the English literature –12. In tEIMS has distinctive morphological features. The tumor is typically characterized by loosely arrayed, round or epithelioid neoplastic cells with vesicular nuclei, prominent large nucleoli and amphophilic to eosinophilic cytoplasm distributed in a widespread myxoid stroma. The striking feature of EIMS is the presence of obvious inflammatory infiltrates frequently composed of neutrophils. All tumors almost contained a small amount of spindle cell component. Immunohistochemically, EIMS demonstrated a unique nuclear membrane staining pattern of ALK, which was observed in 80 % 20/25) of cases  is the most likely to be confused with EIMS, because both tumors have an overlapping immunophenotype and cytogenetic feature, including reactivity for CD30, ALK and SMA, and the presence of ALK rearrangement . DetectiWith respect to the biologic behavior of EIMS, 8 of 21 patients with follow-up information died of the disease , 7 was alive with disease, and the other 6 remained well with no evidence of disease , 11, 12.The optimal therapy of EIMS has not been well established. A surgical resection remains to be considered as the mainstay of treatment. In view of postoperative adjuvant therapy, available experiences are very limited. Most of reported cases are administrated by postoperative chemotherapy or radiotherapy, but it seems to have no obvious effects in the control of rapid recurrence , 11, 12.In summary, EIMS is a highly aggressive IMT variant characterized by epithelioid cytomorphology, often neutrophilic inflammatory infiltrate, loose or myxoid stroma and distinctive nuclear membrane or perinuclear ALK staining. It has a striking male predominance and mostly arises in the intra-abdominal locations. Familiarity with histologic features of this special tumor type with application of an appropriate immunohistochemical panel can arrive to a correct diagnosis. Detection of ALK rearrangement by FISH or RT-PCR will further confirm the diagnosis of EIMS and provide a reliable reference for ALK-targeted therapy.ALCL, anaplastic large cell lymphoma; ALK, anaplastic lymphoma kinase; CT, computed tomography; EIMS, epithelioid inflammatory myofibroblastic sarcoma; FISH, fluorescence in situ hybridization; GIST, gastrointestinal stromal tumor; H&E, hematoxylin-and-eosin; IMT, inflammatory myofibroblastic tumor (IMT); RT-PCR, reverse transcription-polymerase chain reaction


We present herein what we believe is the first reported case of massive upper gastrointestinal bleeding in pregnancy due to a pancreatic neuroendocrine tumour causing left sided portal hypertension. A 37-year-old 27-week pregnant female presented with massive haematemesis and melaena requiring transfusion of 10 units of red cell concentrate. Gastric varices were evident at endoscopy. An MRI revealed a large mass infiltrating the pancreatic tail and spleen with massive upper abdominal varix formation secondary to splenic vein invasion. A caesarean section was performed, followed by a radical en bloc partial pancreatectomy and splenectomy with resection of the fundus of the stomach and ligation of gastric and splenic varices. Her postoperative course was uncomplicated. Histology revealed a well differentiated grade 2 neuroendocrine tumour with final staging of T4N0. This case highlights an infrequently encountered cause of massive gastrointestinal bleeding. Diagnosis and management of pancreatic neuroendocrine tumours, due to their rarity and variable clinical presentation, can be challenging particularly in the setting of pregnancy where the wellbeing of a second patient must also be considered. A multidisciplinary approach with input from obstetricians and general surgeons is required when deciding optimum management, while also taking into account the patient's preferences. Pancreatic neuroendocrine tumours are rare tumours of the pancreas, associated with variable clinical presentations. Diagnosis antepartum results in significant additional therapeutic challenges and both risk of maternal disease progression and risk to the developing foetus must be considered when deciding optimum management. We present herein an unusual clinical presentation of a rare diagnosis and what we believe is the first reported case of massive upper gastrointestinal bleeding in pregnancy due to a pancreatic neuroendocrine tumour (NET) causing left sided portal hypertension.A 37-year-old woman, gravida 3, para 2+0, at 27-week gestation was transferred from a peripheral hospital with a three-day history of haematemesis and melaena requiring 10 units of red blood cells. An endoscopy prior to transfer showed numerous clots in the stomach but no bleeding source was identified. She had no significant past medical or family history and her pregnancy had been uncomplicated to date. Prenatal investigations had revealed a diagnosis of trisomy 21 and a congenital cardiac defect.On presentation, she was haemodynamically stable; her haemoglobin was 9.3 g/dL. Ultrasonography confirmed a viable pregnancy. Urgent upper gastrointestinal endoscopy revealed extensive gastric varices but no bleeding point and no oesophageal varices . ContrasFollowing consultation with the obstetrics team and the patient and her family, a plan was made to proceed with early surgical resection, given the high risk of rebleeding, with delivery of the foetus planned for the same day. She was given corticosteroids for 4 days and then underwent initial uncomplicated caesarean section followed by a radical en bloc partial pancreatectomy and splenectomy with resection of the fundus of the stomach and ligation of massive short gastric varices . Her posHistology revealed a well differentiated grade 2 neuroendocrine tumour with a Ki67 of 10% and a mitotic rate 6 per 10 HPF. The surgical margins were free of tumour and 0/3 lymph nodes were involved. Final staging was T4N0. Her case was discussed at the gastrointestinal cancer multidisciplinary meeting and no further treatment was recommended.Interval imaging with contrast enhanced CT thorax, abdomen, and pelvis at 3, 6, 9, and 18 months was performed with no evidence of disease recurrence or metastases. At 3 months postoperatively, an indium-111 octreotide single-photon emission CT scan (OctreoScan) demonstrated no evidence of metastases. At last follow-up and 24 months postoperatively the patient was well and free of disease.Pancreatic NETs are rare neoplasms that arise in the islet cells of the endocrine pancreas and account for less than 5% of all pancreatic tumours . The bio18FDG-PET/CT, and 68Gallium-PET/CT has further enhanced diagnostic accuracy.Endoscopic ultrasound, computed tomography, and magnetic resonance imaging are the primary modalities used to evaluate tumours of the pancreas, with MRI preferable in pregnancy due to high detection rate with the use of fat-suppressed T1 weighted images and lower foetal risk compared to computed tomography (CT) . In addiSurgical resection is the cornerstone of treatment for localised disease , 8. The Given the unpredictable and variable natural history of PNETs development of metastases must be anticipated, particularly in this case, due to the large size of the tumour and extent of local invasion. In general guidelines recommend follow-up surveillance imaging with MRI or multiphasic CT 3 months after resection and annually thereafter. However, frequency and duration of surveillance may vary and patients with advanced tumours or unfavourable histological parameters may require follow-up imaging at shorter intervals .Pancreatic disease is the most common aetiology of left sided portal hypertension. Splenic vein thrombosis is evident in up to 45% of patients with chronic pancreatitis, although most patients are asymptomatic , 16. In We believe this case is the first pancreatic NET reported in pregnancy presenting with upper gastrointestinal bleeding from varices consequent of splenic vein invasion. There have been three documented cases of PNETs in pregnancy , 18. The


We report here the case of the youngest patient with adenomyomatosis of the gallbladder in a female infant diagnosed at 4 months of age. This diagnosis was made based on characteristic ultrasonography findings in a patient that was undergoing routine surveillance for a suspected clinical diagnosis of Beckwith-Wiedemann syndrome. The patient remains asymptomatic and currently no surgical interventions have been needed. We review the pathophysiology and ultrasonographic findings of this rare condition and present a comparison with the only other four pediatric cases of adenomyomatosis of the gallbladder. Adenomyomatosis of the gallbladder (ADMG) is an acquired condition characterized by localized or diffuse epithelial proliferation and invagination of the mucosa through a hypertrophied muscularis, forming intramural diverticula or sinus tracts. ADMG is diagnosed mainly by imaging. The pathogenesis, pathology, and indications for surgery in this condition are not well understood, especially in children. Although ADMG is benign in nature, it is possible that lithiasis and chronic inflammation secondary to ADMG may lead to dysplastic changes and cancer .We report the case of a female infant diagnosed with ADMG at 4 months of age based on characteristic ultrasonography findings. This diagnosis was made incidentally on a patient that was undergoing routine surveillance for Beckwith-Wiedemann syndrome (BWS). This represents the first report of concurrent BWS and ADMG. Moreover, our case is an infant and to our knowledge is the youngest patient reported with ADMG.The female proband was the first child of nonconsanguineous biracial parents (Caucasian and Brazilian). As a product of a naturally conceived full term pregnancy, she was born by cesarean section and weighed 4.932 kg (>97th centile) with no prenatal or perinatal complications. She was first evaluated at 2 months of age for possible diagnosis of BWS given her macrosomia and positive family history. Her weight was 7.2 kg (>97th centile), length was 61 cm (93rd centile), and head circumference was 42.2 cm (98th centile). There was no history of omphalocele or neonatal hypoglycemia and, on physical exam, no hemihyperplasia, ear lobe creases, or macroglossia. Dysmorphic features included narrow palpebral fissures, epicanthal folds, and tented vermilion of the upper lip.She was subsequently evaluated at 7 months of age with persistent macrosomia. She is otherwise healthy and with normal developmental history. To complete the genetic workup, we also performed a whole genome array  that was normal. The infant received the clinical diagnosis of BWS and was started on the recommended surveillance protocol. Her alpha-fetoprotein levels (AFP) were 71.2 IU/mL and 22.6 IU/mL at five and eight months of age, respectively . Liver enzymes and renal ultrasounds have been normal.Of particular interest, however, was her initial abdominal ultrasound at 4 months of age . The gal CDKN1C sequencing which was also normal.Her 24-year-old mother presented to genetics clinic 34 weeks into the pregnancy. Given the history of macroglossia, hemihyperplasia, and Wilms tumor, we made a clinical diagnosis of BWS and ordered methylation analysis from peripheral blood  which was normal. We then orderedHyperplastic cholecystosis is a term used to differentiate from inflammatory conditions such as acute cholecystitis, since it lacks inflammatory features but exhibits features of hyperplasia. It includes two types of mucosal abnormalities of the gallbladder which are usually clinically incidental findings at the time of cholecystectomy: cholesterolosis and adenomyomatosis. Cholesterolosis is characterized by mucosal villous hyperplasia with excessive accumulation of cholesterol esters within epithelial macrophages, while ADMG describes an acquired, hyperplastic lesion of the gallbladder characterized by overgrowth of the mucosa, thickening of the muscle wall, and intramural diverticula .Rokitansky-Aschoff sinuses (RAS) can appear on ultrasound as echo-poor intramural cystic structures. If they contain sludge, they become echogenic. RASs can also produce distal sonographic shadowing or ring-down (comet tail) artifacts. Such ring-down has been proposed to result from reverberation between the near and far surfaces of the sinuses themselves, closely adjacent intrasinus papillary projections, or contained cholesterol crystals. Sonographic findings of ring-down and polypoid projections of <10 mm suggest ADMG –5.Although gallbladder diseases in children are reported with increasing prevalence because of the widespread use of ultrasonography as a diagnostic tool, ADMG in children was first reported in the 1990s and only in four cases to date  1, 6–8]–86–8]. AADMG is currently divided into three types: diffuse, localized, and segmental (annular type). In the localized type , a cystic structure forms a nodule, usually in the fundus, that projects into the lumen showing a polyp on ultrasonography , 9. Our Although ADMG is benign in nature, stones and chronic inflammation secondary to ADMG may lead to dysplastic changes and cancer, but the causal relationship remains questioned , 10. SurWe present the case of the youngest patient with ultrasonographic findings highly suggestive of ADMG with the context of possible BWS. Ultimately, we need better knowledge to determine the long term consequences of ADMG and its potential relationship with gallbladder cancer.


Charcot arthropathy is a chronic, degenerative condition and is associated with decreased sensorial innervation. Numerous causes of this arthropathy have been described. Here we report a case of neuropathic arthropathy secondary to syringomyelia which was misdiagnosed as a soft tissue tumor and treated surgically and additionally with radiotherapy at another institution. The patient had clinical and radiological signs of syringomyelia, associated with a limited range of motion, swelling, and pain in the affected joint. Neuropathic arthropathy, although less common, should be considered in cases of unexplained joint swelling, pain, and limited range of motion of the affected joint. Neuropathic arthropathy, also known as Charcot joint, is a destructive situation resulting from decreasing or loss of proprioception, pain, and temperature sensation . Many diA 44-year-old female patient was admitted to our clinic in 2012 with left shoulder pain which had been present for 1 year. Past medical history revealed that she had been examined at a state hospital in her hometown 9 months previously with complaints of shoulder pain. After magnetic resonance imaging (MRI) examination, a preliminary diagnosis was made of malignant mass in the shoulder joint. A tru-cut biopsy was performed, after which she underwent surgery for aggressive fibromatosis. Despite radiotherapy treatment for 6 months after surgery, her condition gradually worsened. During follow-up, an MRI study was carried out for suspected recurrences and, as a result, she was referred to our hospital with a diagnosis of recurrence of soft tissue tumor.On physical examination, a deltopectoral incision scar, swelling, and superficial skin lesions, which were probably secondary to radiotherapy, were observed . NeuroloAfter evaluation of all the findings, the patient was diagnosed with neuropathic shoulder arthropathy and was referred to the physical therapy and rehabilitation department for conservative treatment. Nonsteroidal anti-inflammatory drugs were started for swelling and pain of the shoulder. In addition, the patient was managed conservatively for prevention of joint trauma with physical therapy and a protective orthosis was applied. At the two-year follow-up, the shoulder pain had subsided and mobility had improved. On physical examination, passive forward flexion and abduction were 90°, external rotation was 25°, and internal rotation was at the infrascapular region. Muscle strength around the shoulder was 3-4/5. At the time of writing this report, the patient was doing well on this conservative mode of treatment, performing daily activities and fully capable of independent self-care.The diagnosis of neuropathic arthropathy is often delayed. In this case study, a patient with Charcot arthropathy of the shoulder joint caused by syringomyelia was initially misdiagnosed and treated for a soft tissue tumor. Syringomyelia is a chronic and slowly progressive disease of the spinal cord in which there is a fluid-containing cavity (syrinx) inside the spinal cord. It can be congenital or can occur due to trauma, vascular problems, tumor, degeneration, or infection . NeuropaThere are many causes of this arthropathy and up to 25% of cases of neuropathic arthropathy are caused by syringomyelia . A neuroDiabetic patients tend to have involvement of the joints of the foot and ankle, whereas larger joints such as the knee are commonly affected in patients with syphilis . ShouldeSome authors have reported unsatisfactory results with arthrodesis, resurfacing operations, and hemiarthroplasty, which have been applied for the treatment of neuropathic shoulder arthropathy , 13. In In conclusion, Charcot's shoulder is a rare disorder described in fewer than 60 patients in world literature, and correct diagnosis is possible by careful physical and neurological examination and pathological evaluation when needed. Once a neuropathic joint has been diagnosed, its etiology should be ruled out with aseptic joint aspiration to look for infection or tumor, and an MRI to evaluate for syringomyelia if the etiology remains in doubt. In order not to miss the diagnosis, the orthopedic surgeon should be aware of the characteristic clinical and radiological signs of this rare condition.


The radiological finding of a calcified intracranial lesion commonly represents a slow growing benign mass. Brain metastases originating from colorectal cancers are rare, occurring in approximately 2-3% of patients. Therefore the presence of a calcified brain lesion in a patient with a positive oncological history requires a high index of suspicion for brain metastases. Presented herein is a case of a frontoparietal calcified lesion initially overlooked as a benign tumour. Subsequent imaging following a neurological episode revealed a significant increase in size of the lesion with surrounding tissue oedema, prompting further investigation for suspicion of a calcified metastatic colorectal adenocarcinoma. The radiological findings of calcified intracranial lesions generally represent slow growing, benign masses . Most coWe present a case of a frontoparietal calcified brain lesion initially diagnosed as either a meningioma or a granuloma. Subsequently, with a delay of just under a year, the lesion was correctly diagnosed as metastatic colorectal adenocarcinoma.The patient is a 61-year-old female who was diagnosed with colorectal carcinoma (CRC) in 2011. The diagnosis was made after colonoscopic examination revealed an obstructing mass. The patient underwent an anterior resection with subsequent investigations revealing high grade metastatic adenocarcinoma. The patient was started on palliative chemotherapy consisting of FOLFOX and Avastin.Two years after diagnosis (04/2013) a CT brain with contrast was performed revealing an incidental finding of a calcified lesion . The calOne year later (March 2014) the patient had an unwitnessed episode consisting of uncontrolled neuromotor disturbances with opthalmoplegia. The event was suspicious for an acute dystonic reaction due to frequent Maxolon (metoclopramide) administration. The episode subsequently self-resolved; however residual left arm weakness was apparent for the following two weeks. A CT brain was organised to exclude metastases and other organic pathologies. The previous calcified lesion on CT was noted to have increased significantly in size to 3 cm with prominent surrounding tissue oedema .This was increasingly suspicious of a metastatic brain lesion. MRI scan was organised for confirmation, revealing a nonenhancing intra-axial mass lesion with surrounding vasogenic oedema consistent with the calcified lesion seen on CT . The appStereotactic craniotomy was performed with successful excision of the tumour. Histological examination of the surgical specimen revealed an adenocarcinoma with abundant tumour necrosis; the tumour was composed of broad palisading glands, papillary formations, and dystrophic calcification . The immThe calcification of brain metastases is extremely rare and can easily be misdiagnosed as a benign tumour . GeneralThe most common primary sites reported to produce calcified metastatic cerebral lesions include breast, lungs, ovaries, and colon . The detOur case demonstrates a solitary calcified frontal lobe nodule, which metastasised from CRC. Using simple arithmetic and known statistics we can appreciate why such a diagnosis can be difficult to determine. Assuming that 2.3% of CRC metastases to the brain and only 3.5% calcify, a rough estimate of only 0.08% of patients with CRC would have such radiological findings , 8.Unlike other metastatic lesions that can be localised using positron emission tomography (PET) scan, brain metastasis proves to be difficult . MagnetiDifferentiating between metastatic cerebral lesions and benign intracranial neoplasms, such as a meningioma for example, is imperative to appropriate treatment planning. The best therapeutic approach to an asymptomatic benign lesion is conservative treatment with close follow-up to avoid surgical morbidity . Our casThe differential diagnoses for solitary calcified lesions depending on location include angiomas, meningiomas, and gliomas . In compMcCutcheon reported a median survival of 1–3 months for patients with untreated CRC brain metastases . The medIn conclusion, it is recommended that oncology patients should receive early brain scans even if asymptomatic, as it may lead to the early discovery of silent metastases leading to appropriate treatment, improved survival rates, and a heightened quality of life. Furthermore, the discovery of a single subcentimetric, nonenhancing calcific focus with no perilesional oedema in patients with a known oncology history must include metastases as a differential diagnosis, as although it is uncommon it is clinically significant.


Extramedullary hematopoiesis (EMH) is a rare disease, where hematological disorder drives extramedullary hematopoietic tumor formation in multiple regions of the body. The present study reports a case of EMH presenting as multiple tumor-like lesions of mediastinum in a 61-year-old male with α-thalassemia, which was subjected to a video-assisted thoracoscopic surgery tissue biopsy to differentiate it from other mediastinal tumors. To date, only three cases of EMH in patients with α-thalassemia have been described in the literature. Patients with EMH typically exhibit no hematological disorder preoperatively and therefore EMH is frequently misdiagnosed. In the present study, along with a literature review of the clinicopathological features of EMH, the diagnosis and treatment of this rare case was discussed, in order to differentiate diagnosis, and particularly to distinguish EHM from extramedullary myeloid sarcoma. Extramedullary hematopoiesis (EMH), which refers to hematopoiesis occurring outside the medulla of the bone, is a rare hematological disease, secondary to insufficient bone marrow function ,2. EMH oAlpha-thalassaemia is inherited as an autosomal recessive disorder characterized by a microcytic hypochromic anaemia, and is a result of impaired production of 1,2,3, or 4 alpha globin chains, leading to a relative excess of beta globin chains of haemoglobin (Hb) . It is oThe present study describes a case of EMH presenting as multiple tumor-like lesions in the mediastinum of a patient with α-thalassemia. The patient was diagnosed with mediastinal EMH following a tissue biopsy via video-assisted thoracoscopic surgery (VATS). The study protocol was approved by the Research Ethics Committee of The Third Affiliated Hospital, Sun Yat-Sen University .The present case report discusses the major diagnostic problems of mediastinal EMH, distinct characteristics for differentiating EMH from other mediastinal tumors and the treatment strategy applied, as well as a literature review of this rare clinical entity.A 61-year-old Chinese male presented with a 3-year history of intermittent chest pain and tightness. Physical examination revealed elevated blood pressure (149/96 mm Hg), but no other remarkable findings. A chest X-ray and computed tomography (CT) scan were performed and multiple paravertebral masses were identified . Subsequ9/l; hemoglobin (Hb), 119 g/l; platelets, 176×109/l; mean corpuscular volume, 76.4 fl; mean corpuscular Hb concentration, 258 g/l; segmental neutrophils, 48.1%; lymphocytes, 44.5%; monocytes, 5% and eosinophils, 1.9%. No pathological changes were detected in erythrocyte sedimentation rate or C-reactive protein.The complete blood count revealed the following results: White blood cells, 11.44×10To determine the diagnosis of the mediastinal masses, a surgical tissue biopsy via VATS was performed following the provision of consent of the patient and family. A section of the dark-red mass in the left thoracic cavity was excised and electrocoagulation was used for hemostasis. Frozen section examination revealed EMH. A conventional permanent histopathological examination was also performed; the biopsied tissue was stained (hematoxylin and eosin) and examined under a BX43 microscope , revealing hyperplasia with activity of two types of hematopoietic cell, namely erythroid and megakaryocytic cells . FurtherPostoperatively, various laboratory tests were performed, which demonstrated low Hb (90 g/l). Subsequently, thalassemia-associated tests were performed. A diagnosis of α-thalassemia was made based on the increased glucose-6-phosphate dehydrogenase activity (15919.000 mU/g), decreased HbA2 (0.9%) and RBC-BT , as well as positive hemoglobin H staining with hemoglobin electrophoretic studies .The patient was transferred to the Department of Hematology for further treatment, where bone marrow aspiration cytology confirmed the findings of anemia and erythroid hyperplasia . In addition, the relative proportion of immature red blood cells was also significantly increased, but was not accompanied by malignant cell invasion .max) was 5.2. The remaining two paravertebral masses, at the posterior-inferior mediastinum at the T10 level, demonstrated no increased glucose metabolism, with SUVmax values of 3.8  positron emission tomography-computed tomography (PET/CT) was performed to evaluate the mediastinal masses and determine the presence of any additional extramedullary masses. PET/CT identified remnant tissue in the left thoracic T3-T4 paravertebral space following biopsy, and the maximum standardized uptake value  is also a rare extra medullary tumor mass consisting of myeloid blasts. EMS is also known as granulocytic sarcoma or chloroma, since it is diagnosed based on the presence of a green color caused by myeloperoxidase. EMS occurs in ~5% of patients with acute myeloid leukemia (AML) . Rarely,max values and normal appearance of the tissue. The concurrent presence of an underlying hematopoietic disorder may suggest a diagnosis of EMH. In EMS, a PET/CT scan identifies intense FDG uptake (A PET/CT scan may be diagnostically useful in such cases. To date, only a few cases regarding the diagnosis of EMH using PET/CT have been reported ,18, withG uptake .As a result of its rarity, there are no evidence-based guidelines for the treatment of thoracic pseudotumors induced by EMH. Essentially, EMH is a compensatory mechanism for chronic anemia; therefore, recurrent blood transfusions to correct such anemia may result in shrinkage of the mass. However, blood transfusions are not entirely harmless or risk-free, and this therapeutic effect is temporary, slow to occur and may be insufficient –21. HemaBased on the literature review, it was hypothesized that close follow-up of the patient was important. Considering the excellent results obtained with low-dose radiotherapy for EMH masses, the patient will be advised to undergo radiation therapy in the future.EMH should be considered in the differential diagnosis of an intrathoracic mass. Particularly, if a history of thalassemia or chronic anemia is discovered, based on evaluation of the patient's medical history, as well as laboratory studies. Surgical resection and radiation therapy should be considered as treatment options for EMH.


Supraglottic adenoid cystic carcinoma (ACC) is extremely rare and may be misdiagnosed as laryngeal amyloidosis. The present report describes a case of supraglottic ACC, which went unrecognized until histopathological examination of the neoplasm 18 months after the first presentation. The present patient presented with progressive hoarseness for half a year and initially required partial resection. Following quick regional recurrence, the patient received a total laryngectomy while refusing radiotherapy. Adjuvant post-operational traditional Chinese medicine was accepted. Over 3 years’ follow-up, there was no evidence of regional relapse or distant metastases. The present case is compared with a second case of supraglottic submucosal mass in which the signs, symptoms and examinations were similar to the first case, but that was diagnosed as laryngeal amyloidosis. Attention should be paid to submucosal masses in the larynx to prevent underlying malignancy and subsequent disease progression. Immunocytochemistry, such as p63 staining, is mandatory for making an early differential diagnosis of supraglottic ACC. Traditional Chinese medicine may be a useful adjuvant therapy for this rare disease. Adenoid cystic carcinoma (ACC) of the larynx is an extremely rare disease that arises from the laryngeal glandular elements . ApproxiA 44-year-old woman with a six-month history of progressive hoarseness and an abnormal sensation in the throat was referred to the Nanjing Drum Tower Hospital . The patient had no history of smoking, coughing, dyspnea or dysphagia; however, it is worth noting that the patient was exposed to iron ore and coal dust for several years in the work place. In addition, the patient had a history of over five years of second-hand smoke inhalation.Fiberoptic laryngoscopy revealed swelling of the left ventricular fold and laryngeal ventricle that extended backwards to the left aryepiglottic fold. The bilateral vocal cords appeared normal and there was no vocal cord paralysis . The patThe preoperative diagnosis that was deemed most likely was that of a laryngeal neoplasm with laryngeal amyloidosis. The subsequent frozen section biopsy of the lesion taken during laryngofissure revealed the nature of the neoplasm. As the patient refused to undergo an expanded total laryngectomy, which is indicated for a locally advanced laryngeal tumor , local rACC of the head and neck typically arises in the major and minor salivary glands . As acceGrade I ACC is commonly associated with early recurrence and an earlier risk of distant metastases . In the Laryngeal ACC may be easily misdiagnosed as laryngeal amyloidosis, whose manifestation is similar . LaryngeIn the present case, the patient was misdiagnosed with laryngeal amyloidosis due to the following: i) The medical history of the patient revealed a female non-smoker with a two-year slow progression of symptoms; ii) the symptoms included hoarseness and an uncomfortable laryngeal sensation, but not dyspnea, dysphagia or stridor; and iii) the diagnostic tests revealed normal chest X-ray results and submucosal masses involving the left ventricular fold, aryepiglottic fold and pyriform sinus without vocal cord fixation or paralysis upon fiberoptic laryngoscopy and CT. Therefore, no clear malignancy was indicated.In comparison, one month prior to the present case, a 33-year-old female attended an appointment at the Nanjing Drum Tower Hospital and was diagnosed with laryngeal amyloidosis. The patient presented with an abnormal sensation in the throat. The findings of the laryngoscopy and CT scan are shown in Laryngeal ACC recurs quickly and requires sufficient management during the first treatment modality. Thus, we suggest a biopsy with specific immunocytochemical staining as the first treatment strategy. With developing molecular biology, novel therapeutic regimens may be identified from traditional Chinese medicine.Supraglottic ACC of the larynx is a rare entity and is often disregarded. This diagnosis should be considered in patients presenting with progressive hoarseness and an abnormal sensation in the throat, even if there are no other presenting symptoms and with/without a negative chest X-ray. Pre-operative histopathological analysis is essential for an earlier differential diagnosis of laryngeal amyloidosis. In our opinion, prospective randomized multicenter studies are required to determine an optimal treatment. Traditional Chinese medicine may be a post-operative treatment regimen instead of radiotherapy; however, it requires further investigation.


Severe traumatic injuries to immature teeth often cause damage to periodontal ligament as well as dental pulp; pulp necrosis, root resorption and subsequent apical lesion are common consequences. This article reports the surgical management of an infected immature maxillary central incisor associated with a gigantic periradicular lesion and severe root resorption. The tooth had a history of trauma and the patient suffered from purulent sinus tract and tooth mobility. After unsuccessful multi-session disinfection with calcium hydroxide, root end surgery was planned. During flap surgery and lesion enucleation, the root end was cleaned and filled with calcium-enriched mixture (CEM) cement. After one year, the radiographic examination revealed that the lesion was almost completely replaced with newly formed bone. In addition, clinical examination showed favorable outcomes; the tooth was symptom-free and in function. Due to chemical, physical and biological properties of CEM cement, this biomaterial might be considered as the root-end filling material of choice. The general objective of endodontic treatment is to retain the treated tooth in normal function, and the specific goal is to prevent/cure apical periodontitis (AP) . AP is dTraumatic dental injuries (TDI) are among the causes of AP . Two maiCalcium hydroxide (CH) has been used as the intracanal medicament of choice to eliminate pathogenic intracanal bacteria due to its high pH level . The hydThe differentiation of cysts and granulomas is not possible by traditional radiographic techniques . It is sThe main aim of surgical endodontics is to achieve an apical seal . Many prThe present case represents the trial and error treatment of a traumatized immature upper central incisor with root resorption and the least acceptable root length that was surrounded with an excessively large AP; treatment protocol started from orthograde endodontic intervention and ended up in periradicular surgery using CEM cement. A young man in his early twenties came to Mehr dental clinic complaining of a dull ache sensation in the anterior segment of the maxilla and mobility of the maxillary left central incisor. His medical history was not contributory and he reported an impact trauma at primary school. On clinical inspection, the tooth had a grayish hue and had grade II mobility. A draining sinus tract was evident 3 mm far from the gingival margin. The primary periapical radiography showed a very extensive periapical lesion surrounding the short/resorbed root of the tooth. In addition, the large size of pulp canal space was suggestive of tooth becoming necrotic before its maturation, which seemed accordant with the time of trauma. The distal margin of the lesion could not be defined after inserting the full length of a #30 gutta-percha cone into the sinus tract. An occlusal radiography confirmed the extremely large diameter of the lesion .After informing the patient of the hopeless condition of the tooth and possible treatment plans including saving the tooth via endodontic intervention with questionable prognosis, he insisted on giving a chance to the tooth and signed an informed consent.Subsequent to tooth isolation and preparation of a classic access cavity; the pus and exudate flew through the access. After almost 15 min, the exudate ceased and canal was constantly irrigated with sterile normal saline and then 5% NaOCl that was kept in the canal for 5 min. Then the irrigation solution was dried and the canal was filled with thick paste of calcium hydroxide (CH)  mixed with saline; the access cavity was temporarily sealed. After taking the postoperative radiography, the patient was dismissed. One week later, the intracanal medicament had washed away  and the The situation was discussed with the patient and he insisted on maintaining the tooth as long as possible. Endodontic surgery, curettage of the lesion and CEM root-end was offered as the final chance of saving the tooth. In the same session after disinfection with 0.2% chlorhexidine mouth rinse  and profound local anesthesia, a full rectangular mucoperiosteal flap was raised. As it could be foretold, the buccal bone plate was destroyed but the osseous crest was still present. After enlarging the fenestration with engine-driven saline-cooled round drill, the underneath large cavity was curetted and cleaned. Multiple tissue segments were kept in 10% formalin for histopathological evaluation. The cavity was cleaned to the extent that the iatrogenic perforation of the nasal floor as well as maxillary sinus did not occur. Then the root canal was retrogradely cleaned with an ultrasonic device . Then the canal was dried with sterile cotton pellets. CEM cement  was prepared according to the manufacturer’s instructions. The biomaterial was carefully inserted into the canal with a plastic instrument and incrementally condensed with a tiny condenser.After filling of the canal with CEM cement and taking a confirmation radiography , the ligHe did not attend the appointments set for final restoration of the access cavity and follow-up. One year later the patient accidentally appeared in the clinic and follow-up radiography was taken . SurprisThis report represented the combined ortho/retrograde intervention for an infected/traumatized and extremely root resorbed upper central incisor associated with a very large periradicular lesion. The retrograde canal filling was done with CEM biomaterial and after one year, although the tooth had extremely short root length, it was completely functional/asymptomatic and the lesion had healed with newly formed bone. We did not employ triple antibiotic, because of discoloration potential of minocycline. Currently, placement of CH as intracanal medicament, for achieving successful outcome, is a controversial issue ; howeverherodontics) was chosen instead of tooth extraction/implant placement. Implant treatment planning may involve separate examinations by the surgeon and restorative dentist and a variety of imaging techniques. A cost-benefit comparison between endodontic treatment and a single-tooth implant shows that the former is less expensive, entails fewer office visits and functionalizes the tooth more quickly than the implant . In many circumstances, surgical endodontics becomes a necessary step in eradication of AP . After sThere is a long tradition of long-term follow-up assessment of treatment outcome in endodontics. As a dynamic process, healing of AP requires sufficient time to evaluate its progression and completion . Short fThe last but not the least is the quality of apical seal provided by CEM biomaterial. Slight expansion, reasonable flow/film thickness, good adaptation as well as precipitation of hydroxyapatite over its surface, can ensure an effective seal. In addition, the high alkalinity of CEM cement provides a bactericidal environment that prepares the situations required for normal bone healing [15]. The present case confirms the profound abilities of CEM cement to induce healing with normal bone formation in seemingly impossible/hopeless cases with a combined ortho/retrograde approach.


Duplication cyst of the stomach is a rare congenital malformation, typically diagnosed in the first year of life. In most adult cases the cyst remains asymptomatic, but patients may present with abdominal symptoms including epigastric discomfort or pain. We present a case of a 65-year-old male with an asymptomatic gastric tumor diagnosed incidentally during initial workup of his esophageal adenocarcinoma. Computed tomography revealed a low density soft tissue tumor near the gastroesophageal junction. Endoscopic ultrasonography demonstrated a cystic lesion as a hypoechoic round mass with well-defined borders. Following complete laparoscopic resection, microscopic review revealed a cyst lined with respiratory pseudostratified ciliated columnar epithelium and layers of smooth muscle with an outermost thin fibrous capsule consistent with a foregut duplication cyst. Foregut duplication cyst (FDC) is a rare congenital malformation which may occur at any level of the alimentary tract, with most cases diagnosed during the first year of life . DuplicaA 65-year-old male with known history of gastroesophageal reflux disease (GERD) and newly diagnosed esophageal adenocarcinoma was referred to our institution for further management. The patient stated progressive dysphagia with associated significant weight loss without hematemesis, melena, or hematochezia. At the outside institution, computed tomography (CT) and endoscopic ultrasonography (EUS) were performed as part of staging. The results demonstrated a T3 N1 Mx disease which prompted neoadjuvant chemotherapy.In addition to the adenocarcinoma, CT demonstrated a 4.3 × 4.2 cm low density soft tissue mass at the epigastric region, near the hiatus and the gastroesophageal (GE) junction . EUS shoNo significant reduction of this mass was noted on comparative imaging (CT and EUS) following neoadjuvant chemotherapy. The patient underwent transhiatal esophagectomy and partial gastrectomy, which were followed by an uneventful recovery.The specimen consisted of an 11.0 cm segment of esophagus and an 8.0 cm portion of stomach. Macroscopically, 1.0 cm inferior to the GE-junction, a well-circumscribed, white tan, saccular structure measuring 4.0 × 3.4 × 1.2 cm was identified. The cystic lesion was located in the serosa and did not communicate with the gastric lumen. Histologically, it consisted of mucosa lined with respiratory pseudostratified ciliated columnar epithelium (PCCE), which stained positive for thyroid transcription factor 1 (TTF-1) by immunohistochemistry . These fCystic lesions of the stomach are rare and, as a result of embryologic anomalies, are of posttraumatic, infectious, or neoplastic origin. They may occur as single or multiple lesions and may appear at any histologic layer of the stomach.Gastrointestinal malformations occur in the early embryonal stages, and a duplication of the alimentary tract is considered to be a result of abnormal canalization. It may form into a communicating or a noncommunicating cystic or tubular structure, which is lined by a mucosal membrane . These dIn the case presented here, the location of the duplication cyst implies the term “gastric duplication cyst,” but the term “foregut duplication cyst” is preferred, as it is lined exclusively with PCCE rather than gastric or intestinal mucosa , 8. To oThe histogenesis of FDC lined with PCCE remains unclear. Gensler et al. suggested that an FDC lined with PCCE may represent the caudal-most portion of the laryngotracheal outgrowth, which has remained attached to the portion of the primitive foregut destined to become the stomach . This suEUS, CT, and magnetic resonance imaging (MRI) are popular diagnostic methods for the examination of lesions along the gastrointestinal tract. Although FDC are uncommon, EUS findings of gastric duplication cysts have accumulated, and, thus, EUS has proven to be very useful for preoperative diagnosis along with CT or MRI .The clinical presentation of FDC includes abdominal symptoms with a palpable abdominal mass, pain, vomiting, hematemesis, and weight loss , 4. OtheEven though FDC are rare, they are potentially dangerous; therefore, complete surgical resection is advised whenever possible as either open surgery or minimally invasive laparoscopic or endoscopic surgery  , 10. SurIn summary, FDC of the stomach constitutes a congenital disease whose diagnosis is rendered by microscopic examination and which should be included in the differential diagnosis of a gastric mass . Because


The diagnosis of acute appendicitis can be somewhat obscure in a patient that presents with right lower quadrant abdominal pain. The advancement and ease of imaging have made CT scanning readily available in the emergency department. Management can be challenging when the patient has a high likelihood of appendicitis based on clinical suspicion and negative CT scan. The purpose of this case report is to demonstrate how an obstructing bezoar caused an appendicitis-like syndrome in a patient with negative CT scan and clinical diagnosis of acute appendicitis. This case report will discuss the appendicitis-like syndrome of an obstructing bezoar and an approach at management. The diagnosis of acute appendicitis can be somewhat obscure in a patient that presents with right lower quadrant abdominal pain. Pyrexia, pain localized to McBurney's point, and nausea can be a common occurrence in the emergency department. A good history and physical exam often make the diagnosis and negate the need for radiologic imaging. However, the advancement and ease of imaging have made CT scanning readily available in the emergency department. The sensitivity of CT scans for identifying acute appendicitis reaches approximately 97% and the negative predictive value (NPV) reaches 99% by some reports , 2. ManaA 32-year-old otherwise healthy male presents to the emergency department with a history of acute onset right lower quadrant abdominal pain which is 10 hours in duration. He describes the pain as throbbing in nature, 7 out of 10 on the pain scale with no radiation. No alleviating or exacerbating factors were described. The patient also complains of nausea and vomiting for this time period as well. In addition, he reports subjective fever and chills. He has no sick contacts. The patient states that his last meal was the past night and he reports having broccoli for dinner. He reports no difficulty in chewing food. He denies any significant past medical or surgical history including any gastric surgery or gastrointestinal resection. There is no history of Crohn's disease or ulcerative colitis in his family.General Appearance. He is alert and oriented times 3, in mild distress.Vital Signs. Temperature was 98.2 F. Blood pressure was 112/65 mmHg. Pulse was 96 bpm. Respirations were 16. SPO2 was 99% on room air.Skin. Skin was warm and dry. HEENT indicated the following: normocephalic/atraumatic, PERRL, moist oral mucosa, and healthy dentition. Lungs were clear to auscultation bilaterally with no wheezes, rales, or rhonchi. Heart had regular rate and rhythm and no murmurs, rubs, or gallops.Abdomen. Abdomen was soft. He is not distended. He is tender to palpation in the right lower quadrant with positive rebound at McBurney's point, positive Rovsing sign, and positive Obturator sign. No hernias were identified. Rectal exam revealed good anal sphincter tone, no external hemorrhoids/skin tags, and hemoccult negative stool.See See Figures Needle was inserted at the umbilicus and pneumoinsufflation was obtained. Using OptiView guidance, the laparoscope was inserted through a trocar into the abdominal cavity. Next, the area was inspected for injury, failing to reveal any. Additional ports were placed. The diagnostic laparoscopy immediately revealed exudative fluid in the abdominal cavity. This was yellow-green. It was suctioned and the specimen was sent for Gram stain culture and sensitivity. The fluid was washed out. The appendix was identified and found to be relatively normal with minimal serositis at the tip. The small bowel had serositis and inflammation throughout the majority of the small bowel affecting the proximal bowel more than the distal bowel. The terminal ileum was then identified from the ileocecal valve proximally and identification of a mass in the small bowel was made. The mass was firm and impacted and not able to be significantly milked in either direction. The stomach was inspected along with the duodenum and the gallbladder. The gallbladder was distended, but no signs of acute cholecystitis were appreciated. Next, anesthesia administered air to the stomach as well as methylene blue and neither of these tests revealed a leak in the stomach or duodenum. Therefore, the small bowel was then addressed just distally to the impaction and enterotomy was made. This was done by scoring the small bowel laparoscopically. The decision was made to open the fascia slightly at the umbilical port site and withdraw the small bowel. In doing so, the impacted foreign body had been milked away from the site. The bowel holding the foreign body was unable to be extracted through the enlarged port site, and, therefore, towel clamps were applied. Pneumoinsufflation was reobtained. The bowel was grasped with the grasper and extracted at the umbilical port site. The small bowel was opened with monopolar cautery via the Bovie cautery extracorporeally and then dropped back into the abdominal cavity. Towel clamps were applied to the skin and the impacted foreign body was milked towards the enterotomy . This teForeign body ingestion is a common problem experienced in the emergency department. The ingestion of foreign objects is most commonly seen in children, while, on the other hand, adult presentations of foreign body ingestion are more likely to be due to bezoars. A bezoar is defined as a swallowed mass of undigested material found within the gastrointestinal tract . The mosAccurate preoperative diagnosis of bezoars as the cause of a patient's acute abdomen symptoms is difficult . Due to Although clinical presentations of bezoars are unusual, there have been reports of foreign body ingestion mimicking other diseases such as renal stones and irritable bowel syndrome . FurtherHowever, to our knowledge, this is one of the first case reports of acute appendicitis-like syndrome caused by an intestinal obstructing bezoar. While bezoars are rare, they need to be part of a working diagnosis when dealing with atypical presentations, especially negative CT imaging, of intestinal obstruction and acute appendicitis.Foreign body ingestion in children and acute appendicitis are both common problems experienced in the emergency department, while bezoars in adults are uncommon and estimated to occur in about 0.4% of the general population. Complications of bezoars include intestinal obstruction and, in this case report, an acute appendicitis-like syndrome. Accurate preoperative diagnosis of bezoars as the cause of a patient's acute abdomen is difficult. CT examination is a valuable tool for diagnosing acute appendicitis or intestinal obstruction and revealing the etiology of a patient's clinical presentation. However, even with CT imaging, bezoars can be radiolucent or unidentifiable, as seen in this case report. Clinical judgment is therefore required to determine the next step in management. This case report describes the rare clinical presentation of an obstructing bezoar causing an acute appendicitis-like syndrome.


Focal fibrocartilaginous dysplasia (FFCD) is a rare, paraneoplastic disease that often presents in children and teenagers. Previous studies have reported cases of lesions in the proximal tibia and distal femur, as well as lesions in the upper extremities. The present study describes a case of FFCD on the transverse process and the rib. The imaging findings were found to correspond with the typical observations of FFCD and a biopsy from the nidus revealed pathological results similar to those of previous reports. Thus, the present study demonstrated that FFCD affects tubular bones as well as flat bones. Further studies are required to investigate the underlying mechanism and treatment of FFCD. Less than 100 cases have been reported in the English literature since the original description by Bell A 38-year-old male  presented at The First Affiliated Hospital of Nanchang University  with pneumonia and lesions on the left first thoracic vertebra, which were detected using chest computed tomography (CT). The patient reported that he had experienced a tolerable level of pain in the area three times during the previous ten years, with no history of trauma. No other family members had suffered from a similar condition. The initial consultation was performed on 28/05/2013. Informed consent was obtained from the patient.Physical examinations revealed that the patient was healthy and ambulated without difficulty. The patient exhibited a full range of motion of the neck and upper extremities. Furthermore, no masses were palpable. X-ray  and chesCurettage of the focal lesion and an incisional biopsy were performed due to the possibility of the lesion being an aneurysmal bone cyst. The surgery lasted 120 min and the estimated blood loss was 200 ml. A frozen section obtained during surgery did not provide a specific diagnosis. After one week, the pathologic results from a specimen of the lesion  demonstrTwo months after surgery, the patient was followed up. Chest CT  demonstret al . Radiologically, FFCD is similar to conventional FD, in that the lesion is well demarcated and exhibits cortical expansion, but with an intact cortex. In order to diagnose FFCD it must be differentiated from dyschondrosteosis, Ollier’s disease, neurofibromatosis and trauma . In contet al (It has been hypothesized that each of the deformities associated with FFCD may spontaneously correct or progressively improve and studies have reported an incidence of spontaneous correction ≤45% (et al  demonstret al ,14 and oet al . SurgicaIn lesions in any of the four limbs, FFCD is consistently associated with angular deformities in children. The associated deformities commonly result in limb-length inequalities, with disparities of ≤30 mm ,4,9. FurIn conclusion, the present case proposes that FFCD may affect tubular bones as well as flat bones. However, the underlying mechanism and treatment of FFCD requires further investigation.


Urethral leiomyoma is an extremely rare condition that arises from the smooth muscle of the urethra. To the best of our knowledge, there is only a single reported recurrence treated by a repeat excision in the literature to date. The present study reports an exceptionally rare case of a recurrent huge leiomyoma of the female urethra. The 47-year-old female was diagnosed with a huge mass located between the urethra and vagina during a gynecological examination. The patient had no symptoms and was successfully treated with transabdominal excision. Pathological examinations revealed a leiomyoma of the urethra. The patient was followed up for one year without any sign of recurrence. Urethral leiomyomas are rare mesenchymal benign tumors of smooth muscle origin that occur almost exclusively in females . To the A 47-year-old Chinese female was admitted to the Department of Urology , due to a solid mass detected during a gynecological examination. The patient was asymptomatic and had undergone two surgeries for urethral leiomyoma, which occurred six and nine years ago, respectively. Computed tomography demonstrated that the bladder and uterine were compressed by a 7.5×7.0-cm mass with well-defined outlines . MagnetiThe urethral tumor was completely excised and removed as close to the bladder neck as possible by transabdominal surgery. The detachment in the paraurethral region was meticulous to reduce injury to the urethra. The subsequent pathological diagnosis was of a leiomyoma. Immunohistological analysis demonstrated that the tumor cells were positive for desmin, cluster of differentiation 10 (CD10), smooth muscle actin and caldesmon, and negative for CD117 . The patLeiomyomas are benign tumors of smooth muscle origin that occur throughout the genitourinary system, most commonly in the renal capsule . UrethraUrethral leiomyomas are usually asymptomatic when they are small. As they grow in size, patients may complain of urinary tract infection, dyspareunia, urinary retention or irritative lower urinary tract symptoms . PhysicaIn the present study, the huge pelvic tumor should be differentiated from the urethral carcinoma or masses that have originated from other tissues, although the patient had a past history of urethral leiomyoma present six and nine years ago, respectively. In all previously published cases, the urethral leiomyomas have been treated surgically, with surgical excision as the first choice of treatment. The present patient was treated by transabdominal excision and the tumor was found to originate from the proximal segment of the urethra, which is close to the bladder neck.All previously reported vesical and urethral leiomyomas have followed a benign biological course , with on


Case. A right-handed 8-year-old female patient presented with a conventional, high-grade osteosarcoma involving her right humerus; through-shoulder amputation was recommended. After consultation, total humerus resection with expandable, total humeral endoprosthesis reconstruction was performed with a sleeve to encourage soft-tissue ingrowth. At three-year follow-up she has received one lengthening procedure and her functional scores are excellent. Conclusion. Total humeral resection and replacement in the pediatric population are rare and although early reports of expandable total humeral endoprosthesis outcomes demonstrate high failure rates, this patient's success indicates that expandable total humeral replacement is a viable option. Osteosarcoma is the most common primary malignant tumor of bone and most commonly affects children and young adults in the second decade of life. Adjuvant chemotherapy has improved the 5-year survival rate of nonmetastatic osteosarcoma to approximately 70%. Subsequent advances in three-dimensional radiological imaging and improved implant technology have facilitated limb-preserving surgery for osteosarcoma. Limb-preserving surgery in children presents the additional challenge of accommodating growth.We report the case of a pediatric patient diagnosed with osteosarcoma of the right humerus. Prior to presentation to our clinic, upper extremity amputation was recommended for surgical management. After review of her radiographic and staging studies and completion of her neoadjuvant treatment, an expandable proximal humeral replacement was performed. The purpose of this paper is to describe her surgical management and three-year functional and oncological outcome. The patient and her parents provided consent for this study and her rights were protected.A right-handed eight-year-old girl presented to her primary care physician in Iowa with 2-3 weeks of right arm pain at rest and with activity. There was no history of trauma and she localized the pain to the proximal and midshaft region of the right humerus. Radiographs revealed a blastic-appearing lesion and referral to an orthopaedic oncology surgeon was undertaken. A core needle biopsy was performed which confirmed a diagnosis of high-grade conventional osteosarcoma. Staging studies were performed including a computed tomography (CT) scan of the chest, magnetic resonance imaging (MRI) of the humerus, and whole-body Technetium-99 bone scan, which revealed no evidence of metastatic disease or Enneking Stage IIB disease. Our patient then began neoadjuvant chemotherapy and surgical planning was undertaken for a through-shoulder amputation of the right upper extremity. At this point her parents contacted our clinic to discuss limb-preserving surgery.Upon presentation to our clinic the patient had resolution of her arm pain. She had no relevant medical, family, or social history. Physical examination revealed a well-appearing, well-nourished, and interactive girl who appeared as her stated age. Her right upper extremity showed increased girth about the proximal and midshaft region of the humerus, a well-healed biopsy incision, and no skin changes; the mass was not tender to palpation. Her right arm showed normal sensory, motor, and vascular function with full shoulder and elbow motion compared to the left upper limb. The remainder of her examination demonstrated no abnormalities.Postchemotherapy radiographs showed mineralization of her tumor ; repeat Because local control of the tumor would require removal of the entire humerus, a total humeral replacement was recommended to the patient and her family. Due to the patient's young age and anticipated growth of 7.2 cm , a reconSurgical resection of her tumor was performed through an extensile anterior approach to the shoulder joint, anterolateral approach to the humeral shaft, and lateral approach to the elbow joint . The braAt present our patient is three years from surgery and remains without evidence of local or distant disease progression. She has undergone one lengthening operation with a total of 0.7 cm of expansion. Her shoulder demonstrates passive abduction and flexion of 0–120 and 0–135, respectively. Active shoulder abduction and flexion are 0–110 and 0–130, respectively. Shoulder strength in both abduction and flexion are 4/5; strength with internal and external rotation is 2+/5. Active and passive elbow motion are 0–125 with strength of 3/5 in flexion and 4/5 in extension. She was able to resume playing the piano without difficulty or discomfort. Adolescent PODCI self-reported global mean and normative scores were 92 and 46; adolescent PODCI parent-reported global mean and global normative scores were 95 and 50, respectively; PODCI domain scores are reported in The goal of limb-sparing surgery for connective tissue cancers is complete tumor excision leading to disease-free survival with a functional limb . While lEndoprosthesis reconstructions with uniaxial and polyaxial articulations are affected by different failure modes; uniaxial articulations are frequently subject to aseptic loosening and polyaxial joints are commonly unstable . Total hBecause the preponderance of pediatric primary malignant bone tumors occurs about the knee and hip, expandable implants were first described for use in the lower extremity by Scales et al. in 1976 . Since tUse of expandable endoprostheses in the upper extremity is far less common than in the lower extremity, due to both the distribution of pediatric bone cancers and the difficulty of creating a lengthening mechanism that fits neatly into a pediatric humerus-sized package. An expandable humerus implant was first conceived in 1985 by the Biomechanical Engineering Department at the Royal National Orthopaedic Hospital at Stanmore. Lavy and Briggs reported on the use of the Stanmore implant for total humerus replacement and the results were poor with all three implants resulting in failure . AccordiTo our knowledge the case presented here is the seventh expandable total humerus replacement performed in the US and is the first to combine the Stanmore minimally invasive lengthening implant with a porous sleeve for soft tissue ingrowth. Current technology does not allow for noninvasive lengthening; however, this will likely be available in the future. Revision of the current prosthesis is not mandatory and will be undertaken only in the event of failure. Based on the excellent oncological and functional results of our patient, and the antithetical alternative of upper extremity amputation, we believe that expandable total humeral replacement is an excellent option for the skeletally immature patient meeting indications.


Acrokeratosis paraneoplastica is a rare paraneoplastic phenomenon associated with upper aerodigestive tract carcinomas, usually manifesting as psoriasiform keratosis over the acral sites. It is primarily seen in white males above the age of 40 years. Here we report a case of paraneoplastic acrokeratosis in a woman with serous ovarian cancer. To the best of our knowledge, no similar case has been reported previously.We report the case of a 60-year-old woman diagnosed with a serous ovarian cancer and complaining of a thickening and peeling of the skin on her feet. Clinical and histological examination, as well as the course of disease, confirmed the diagnosis of a paraneoplastic plantar keratosis. Under systemic chemotherapy with carboplatin and paclitaxel the lesion resolved gradually in concordance with tumour marker CA 125.We present the reported case of paraneoplastic acrokeratosis associated with advanced high-grade ovarian cancer. Acrokeratosis paraneoplastica of Bazex was first described by Bazex et al. in 1965 . It can Paraneoplastic acrokeratosis affects primarily Caucasian men above 40 years of age. A review of 140 cases described only 12 cases occuring in women .We present the case of a 60-year-old woman with a newly diagnosed high-grade serous ovarian cancer and associated plantar paraneoplastic acrokeratosis. To our knowledge, the association of this carcinoma with Acrokeratosis paraneoplastica of Bazex has not been reported previously.A 60-year-old woman presented with acute upper abdominal pain, fever, nausea and shivering as well as elevated liver and pancreatic enzymes. A computed tomographic scan confirmed the suspicion of lithogenic pancreatitis but also revealed an unclear mass on both ovaries as well as signs of peritoneal carcinomatosis, omental cake, infiltration of the liver and spleen and substantial ascites at the same time.CA125 tumour marker was elevated up to 305 U/mL suggesting the presence of an advanced ovarian cancer . No other blood abnormalities beyond high tumour marker levels were found. Consequently a surgical tumour debulking was performed. Intraoperatively, a highly distinct peritoneal carcinomatosis with cementation of all the internal abdominal organs could be seen. The ovaries were buried under this tumourous mass and couldn’t be fully visualised during the operation. Under these circumstances no optimal cytoreduction could be performed. The histology later confirmed the presence of a high-grade serous carcinoma of the ovary. Chemotherapy with carboplatin and paclitaxel was induced postoperatively.Already prior to surgery, approximately when the ovarian cancer was diagnosed, the patient complained about a disturbing skin alteration on both her feet. Inspection revealed laminar hyperkeratosis and scaling of the plantar skin (enhanced in areas under compressive stress), which was unbeknownst to her. Dermatological examination as well as punch biopsy of the skin Fig. ,b suggesAcrokeratosis paraneoplastica is a rare, obligate paraneoplasia initially presenting with palmoplantar hyperkeratosis. The pathogenesis is still not well understood. A few authors have proposed molecular mimicry as a possible mechanism; the crossed reactions between tumour antigens and growth factor receptors on epidermal cells. Another postulated mechanism is the action of epidermal growth factor, transforming growth factor-alpha and insulin-like growth factor secreted by the tumour cells themselves, which can lead to the cutaneous changes. Furthermore, zinc deficiency associated with the neoplasm may play a role in the pathogenesis of this hyperproliferative dermatosis. , 8, 9. HMost cases of paraneoplastic acrokeratosis are associated with squamous cell carcinomas of the upper one-third of the respiratory or gastrointestinal tract . Single case reports also described an association of acrokeratosis paraneoplastica with carcinomas of the thymus, bladder, skin, multiple myeloma, carcinoids, malignant lymphomas and cholangiocarcinomas. Women seem to be less affected by acrokeratosis paranoplastica and only single cases have been reported in association with gynaecological neoplasms  –4, 9, 11Paraneoplastic syndromes are often associated with ovarian cancers, as determined from a clinicopathological study of 908 patients in 1992 by Hudson and colleagues . Few unsIn 2011, Fader et al. described the second case (the first reported in 1983) of Sweet’s syndrome in ovarian carcinoma . Sweet’sDermatological signs and symptoms can be common paraneoplastic manifestations of underlying malignancy. Cutaneous lesions, typically associated with other neoplasms than ovarian carcinoma, are amongst the ones described above: Necrolytic migratory erythema (most commonly associated with islet cell tumours), Paraneoplastic Pemphigus , Leser-Trélat sign  and other, more rare dermatoses .To the best of our knowledge, no case of acrokeratosis paraneoplastica in association with ovarian cancer has been described before. This is a rare but important paraneoplastic skin finding to be recognized, as dermatological symptoms can precede the associated malignancy, unlike that of our patient.As the histopathological findings are unspecific, the diagnosis can only be made on the basis of a combination of clinical and histological criteria and the regression of the cutaneous symptoms during and after therapy of the underlying carcinoma.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.


To describe 19 years of clinical experience managing pediatric patients with testicular yolk sac tumors at the Chongqing Medical University Affiliated Children’s Hospital.This study involved a retrospective review of the records of 61 pediatric patients who presented with testicular yolk sac tumor at our institution between 1995 and 2014.All patients presented with a painless scrotal mass. Serum alpha-fetoprotein (AFP) levels were elevated (n = 15). Ultrasonography identified the yolk sac tumors as solid masses. Color Doppler flow imaging showed rich blood flow inside and around the masses in 84.8% cases. X-ray of the scrotum showed no intrascrotal calcification (n = 38). Inguinal orchiectomy was performed in 60 patients, one case was treated with testis-sparing surgery. In 11 cases, radical dissection of the inguinal lymph nodes was performed. Histological analysis showed pathologies typical of yolk sac tumor including microcapsule and reticular structures, gland tube-gland bubble structures, an embryo sinus structure, and papillary structures. All patients received postoperative chemotherapy. Serum AFP levels returned to normal 1 to 2 months after surgery. No patients treated with surgery in our hospital relapsed.Testicular yolk sac tumor presents as a painless scrotal mass, increased serum AFP levels, and a solid mass on ultrasound. Chest radiography and abdominal ultrasound should be used to accurately stage the tumor. We advocate for inguinal orchiectomy for Stage I disease and postoperative chemotherapy to prevent recurrence in the ipsilateral or contralateral testis. Testicular tumors are uncommon in children, comprising approximately 1% to 2% of all pediatric malignancies . HoweverPediatric patients with testicular yolk sac tumors usually present with an asymptomatic scrotal mass early (Stage I) in the disease process. Evaluation of the solid scrotal mass includes: scrotal ultrasound; chest, abdominal, and pelvic computed tomography (CT); and determination of serum tumor marker levels such as alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (β-hCG). An elevated serum AFP level is closely associated with yolk sac tumors in more than 90% of patients .The treatment of testicular yolk sac tumors is dependent on tumor stage and patient age. Resection and chemotherapy with or without retroperitoneal lymph node dissection (RPLND) is often used for children with elevated or rising AFP levels and/or retroperitoneal lymphadenopathy. In cases of recurrence or metastasis, the vast majority of pediatric patients can be successfully treated with chemotherapy , 9.This report describes 19 years of clinical experience managing pediatric patients with testicular yolk sac tumors at the Chongqing Medical University Affiliated Children’s Hospital. These data will improve current knowledge of diagnostic and treatment processes for the disease.This study was approved by the Ethical Committee of Chongqing Medical University.This study involved a retrospective review of 61 patient records at the Chongqing Medical University Affiliated Children’s Hospital from 1995 to 2014. Inclusion criteria were: (1) presence of a painless scrotal mass; (2) suspected testicular tumor; and (3) underwent treatment for testicular yolk sac tumor. Exclusion criteria were: (1) mixed testicular tumors (yolk sac tumor with teratoma); or (2) no accurate pathological evidence of tumor.Information extracted from patient records included: (1) age; (2) clinical manifestation ; (3) physical examination ; (4) personal and family medical history; (5) routine laboratory parameters and serum AFP levels; (6) preoperative imaging data, including ultrasonography of the testis and abdomen, color Doppler flow imaging (CDFI) of the testis, and plain scrotal X-ray; (5) treatment; (6) histopathologic diagnosis and analysis; and (7) follow-up, including abdominal CT imaging to detect evidence of metastasis.All tumors were surgically resected. Inguinal orchiectomy was performed in 60 patients. The lower abdomen was explored through a lateral incision, and the spermatic cord was clamped. The scrotum and the tunica vaginalis on the affected side were incised, and the testis was isolated. A frozen biopsy was immediately sent for histopathology. Complete excision of the mass and inguinal orchiectomy was performed based on the biopsy result and parental preference. If lymphadenectasis was found in the inguinal region, the inguinal lymph nodes were excised.Tumors were staged based on the TNM Classification of Malignant Tumors published by the International Union Against Cancer in 2009 [All patients received postoperative chemotherapy.Postoperative pathological examination was performed at the Institute of Pathology, Chongqing Medical University Affiliated Children’s Hospital. Samples were collected during surgery and fixed in 10% neutral formalin, dehydrated through graded alcohols, and embedded in paraffin. Sections of 4 μm were cut for histology, stained with hematoxylin and eosin (H&E), and examined using light microscopy.Immunohistochemical staining included AFP, cytokeratin (CK), vimentin (Vim), placental alkaline phosphatase (PALP), epithelial membrane antigen (EMA), CD117, and Ki-67. A tissue was considered positive for a marker if >10% cells were stained. A tissue was considered negative for a marker if ≤10% cells were stained.Patients continued chemotherapy and were followed up in the inpatient department of our institution at postoperative 1 and 2 months. Subsequently, patients returned for follow-up visits once every 6 months for 48 months. Follow-up visits included routine blood tests, evaluation of biochemical indicators , electrocardiography, and imaging .P <0.05.Statistical analysis was performed with GraphPad Prism 4.0 . Data are expressed as mean ± SEM. Between group differences were analyzed with Student t-test. Significance was defined as All patients presented with a painless scrotal mass; 26 occurred on the left side, 35 occurred on the right side. The masses were solid with a smooth surface and were obvious on ‘bearing down’. Six cases were misdiagnosed as hydrocele, four cases as inguinal hernia, two cases as testicular inflammation, and one case as adenoma. These cases were misdiagnosed for presenting with a painless scrotal mass, however, all were cleared with transillumination test, ultrasonography, and the frozen biopsy: (1) transillumination test result was positive for the six hydrocele and negative for the yolk sac tumors; (2) the four inguinal hernia were shown as intestinal canal-shaped structures on ultrasonography; (3) the two testicular inflammations were described as diffusely enlarged with rich blood flow, no space-occupying lesion were found inside on ultrasound; (4) ultrasound showed tumor tissue with adenoid structure beside normal testis and clear septum between tumor and testis, while no specific structure change in this case of adenoid carcinoma of testis. Diagnosis was confirmed by intraoperative frozen biopsy.Mean age at diagnosis was 1.5 years ± 1.0 years   . Mean serum AFP level 14 days after surgery was 2,581 . There was no significant difference between preoperative and postoperative serum AFP levels .Ultrasonography identified the yolk sac tumors as solid masses; focal masses were evident when the tumor replaced the entire testicle. The masses were described as hyperechoic in 8% of cases (5/61); hyperechoic and isoechoic in 46% of cases (28/61), isoechoic in 28% of cases (17/61); isoechoic and hypoechoic in 6.5% of cases (4/61), hypoechoic in 5% of cases (3/61), and ‘mixed abnormal echo’ in 6.5% of cases (4/61). In 14 cases, there was an anechoic area, which was identified as a hydrocele. Mean dimensions of the masses on ultrasound were 53 cmCDFI showed rich blood flow inside and around the masses in 85.5% cases (52/61). Mean blood-flow velocity inside and around the masses was 0.071 m/s ± 0.018 m/s . Mean blood flow inside and around the normal testes was 0.024 m/s ± 0.007 m/s  which shows blood-flow velocity inside and around the masses is faster than it inside and around the normal testes  or necrosis (n = 1). In one case, CT revealed a lesion occupying the left retroperitoneal space that displaced the left kidney.3 ± 17.95 cm3 , , and these patients repeat the course for 3 weeks and last for 3 to 5 weeks on average. One patient received a combination of carboplatin, etoposide, and bleomycin (JEB), and one patient received PEB and cyclophosphamide.9/L). Levels normalized following blood transfusion and myocardial preservation, allowing the patients to complete chemotherapy.One to two months after the initiation of chemotherapy, 16% (5/31) of the patients at follow-up experienced anemia  and leukopenia  of patients. The average length of follow-up was 48 months. Of these, three patients experienced relapse. Overall survival rate was 100%, and there was no evidence of metastasis. AFP, chest radiography, and abdominal ultrasonography were used as follow-up indicators. In 16 patients, serum AFP level was normal after 2 years of follow-up. In all patients, chest radiography and electrocardiogram 1 and 2 months post surgery were normal. In three patients, ultrasonography revealed an inguinal mass. Two patients underwent inguinal mass resection; histology confirmed the absence of tumor cells. One patient underwent inguinal mass resection and hernia sac high ligation. The mass was no longer present at the next follow-up.Table  Table  Yolk sac tumors are the most common primary tumors of the testes in prepubertal patients . The paiTesticular yolk sac tumors frequently secrete high concentrations of AFP; therefore, AFP is considered important in the diagnosis and follow-up of the tumors . EvidencUltrasound is the most common method for diagnosis of testicular tumors; it can also be used to differentiate benign and malignant testicular lesions , 15. ImpWe used intraoperative analyses of frozen-sections for routine evaluation of testicular yolk sac tumors to provide evidence for orchiectomy. Paraffin-embedded sections were used for histologic analysis after surgery. Histopathologic analyses of solid yolk sac tumors frequently show microcystic/reticular patterns. In a previous study reporting the histology of 52 solid yolk sac tumors, 75% were microcystic/reticular, 35% were glandular, and 25% were myxoid . These dIn the past, management of prepubertal testicular tumors was based on experience with adult testicular cancer. At present, clinicians agree that prepubertal testicular neoplasms differ greatly from postpubertal lesions. Traditionally, high inguinal orchiectomy was recommended for testicular yolk sac tumor because of the cancer’s aggressive nature . More reRPLND may prevent metastasis in patients with testicular yolk sac tumors , 19, 20.Chemotherapy is recommended for the treatment of yolk sac tumor. Platinum-based cancer drugs have recognized efficacy . HoweverThis study improves current knowledge of diagnostic and treatment processes for testicular yolk sac tumor. However, it is associated with a number of limitations. First, some patients’ records were incomplete. Second, parent/patient preferences influenced management decisions, which may have affected outcomes. Future studies with longer follow-up are required to fully inform the safe and effective management of testicular yolk sac tumor.In summary, testicular yolk sac tumor presents as a painless scrotal mass and an increased serum AFP level (>100 ng/mL). Based on our clinical experience, we recommend ultrasound as a reliable method for preoperative localization and evaluation of testicular yolk sac tumor. Chest radiography, CT, and abdominal ultrasound should be used to accurately stage the tumor. We propose high inguinal orchiectomy for Stage I disease, RPLND for Stages II or III, and more weeks are needed for the older patients (>18 months) with higher AFP levels to fall to normal, which could be classified as Stage II. But intraoperative histopathological evaluation of frozen sections should guide the extent of surgery. Postoperative chemotherapy (PEB) should be used to prevent recurrence in the ipsilateral or contralateral testis. AFP and ultrasonography is considered important in the follow-up of the tumors, and the initial AFP monitoring is proposed in 2 to 3 weeks after operation. Long-term follow-up (>48 month) is recommended.