Primary malignant tracheal tumors account for only 0.2 % of all malignancies of the respiratory tract. Tracheal chondrosarcoma is a rare condition and only 17 cases have been described in the literature from 1965 to date. Herein we report the very unusual case of a patient with a tracheal chondrosarcoma, electively treated by curative surgery despite the virtually complete obstruction of the airway.We present the case of a 79-year old Caucasian man with long-lasting wheezing misdiagnosed as asthma and affected by a tracheal chondrosarcoma almost completely obstructing the airway. Videobronchoscopy and imaging investigations revealed a well-circumscribed mass arising from the cartilaginous rings of the cervical trachea with a posterior residual respiratory space of about 1 mm. Because of the mobility and flaccidity of the uninvolved pars membranacea, the tiny respiratory space slightly expanded during inspiration and expiration allowing the patient to be treated without an essential emergency procedure. Standard tracheal intubation was impossible. Rigid bronchoscopy enabled placement of a small tracheal tube distally to the tumor. Successful cervical tracheal resection and reconstruction was then performed, achieving complete tumor excision. Histologically, the mass was characterized as a low-grade tracheal chondrosarcoma. Videobronchoscopy performed 9 months after surgery showed a wide, well healed tracheal anastomosis. Ten months after surgery, the patient is alive and disease free.Complete surgical resection is the treatment of choice for tracheal chondrosarcoma. Rigid bronchoscopy is an essential tool for diagnostic and therapeutic purposes. It allows the palliative maneuvers for obstruction relief but also, in resectable patients, the intraoperative safe and straightforward management of the obstructed airway.The online version of this article (doi:10.1186/s13019-016-0498-8) contains supplementary material, which is available to authorized users. Primary malignant tracheal tumors account for only 0.2 % of all malignancies of the respiratory tract. Tracheal chondrosarcoma (TCS) is an extremely rare condition, with just 17 cases described in the English literature .In all previously reported cases, TCS appeared as a bulky tracheal tumor, with variable airway obstruction: 16 patients presented with a long clinical history  and one We report the unique case of a patient with a TCS treated with a curative resection without need of emergency procedures despite a virtually complete airway obstruction.A 79-year old Caucasian male, non smoker, was referred to our Department for a tracheal tumor. He reported a 3-year history of dyspnea, tirage and cornage, initially misdiagnosed as asthma. A chest X-ray was normal. During a flu episode, dyspnea became extremely severe making the patient bedridden. A computed tomography (CT) scan was performed revealing a 34×29×31 mm tumor in the upper third of the trachea with almost complete airway obstruction. The lesion had an inhomogeneous density characterized by the presence of osteo-cartilaginous clods and solid tissue without significant contrast enhancement, and involved the anterior extra tracheal soft tissues . Because of its slow growth, TCS is characterized by a long natural history, often misdiagnosed, as occurred in our patient: wheezing is the most common reported symptom, followed by vocal cord palsy, cough, hemoptysis, hoarseness and pneumonia –3. HistoFlexible bronchoscopy is the essential method for diagnosis and follow-up. It allows the correct surgical planning through the evaluation of the surface/site/extent of the tumor, vocal cord motility and airway lumen. Rigid bronchoscopy is mandatory before surgery, both for diagnostic and therapeutic purposes. It enables the measurement of tumor length and distance between tumor and anatomic landmarks and the treatment of obstruction, when needed . If preoAnalysis of the literature shows that the optimal treatment for TCS is the complete tracheal resection. Indeed one patient died of multiple distant metastases 14 years after incomplete resection , two outThe slow tumor evolution, along with the integrity of the flaccid, membranous pars of the trachea, can explain why an emergency procedure was not needed in the reported case despite the almost absolute airway obstruction.Complete surgical resection is the treatment that allows the best oncologic results for tracheal chondrosarcomas. Curative surgery is facilitated by the limited submucosal spread of TCS which can be radically resected with a minimum clear resection margin. Rigid bronchoscopy is fundamental for surgical planning and for airway management, especially for those patients not amenable to standard tracheal intubation.Elective management in referring centers improves the outcome, because emergency treatments can be just palliative.CT, computed tomography; TCS, tracheal chondrosarcoma


Primary jejunal gastrinomas are exceedingly rare, and data for long-term follow-up is limited. Until now, only six cases of gastrinomas arising from the jejunum have been reported in the English literature.Presented is a case of a primary gastrinoma located in the proximal jejunum. After surgical resection of the tumor, eugastrinemia was quickly achieved and after a 10-year follow-up period, the patient was still disease-free.This case report demonstrates that surgical resection of a primary jejunal gastrinoma without evidence of metastasis can be curative, with a good long-term prognosis. A gastrinoma is a non-beta islet cell tumor which secretes gastrin. The hypergastrinemic state caused by a gastrinoma results in multiple recurrent and often refractory ulcers in the gastrointestinal tract and is known as Zollinger-Ellison syndrome (ZES). In 1955, ZES was originally reported with the presenting triad of recurrent peptic ulceration, hypersecretion of gastric acid, and the presence of a non-beta islet cell tumor of the pancreas .However, there have been reported cases of gastrinomas outside of these areas, which have been called ectopic gastrinomas. About 5.6 % of patients have a primary gastrinoma located in an ectopic site . PrimaryIn September 2003, a 34-year-old male patient presented at the Department of Surgery at Shin Kong Wu Ho-Su Memorial Hospital  with abdominal fullness and discomfort that had gradually developed over the past year. Upon further review, the patient had an 8-year history of heartburn which was only slightly relieved by antacids. Bowel habits were normal without mention of tarry or bloody stool. Also, he did not notice recent body weight loss.Abdominal ultrasound in September of 2003 showed an abdominal mass in the left upper quadrant of his abdomen. An abdominal computed tomography (CT) confirmed a left upper quadrant abdominal mass (approximately 7 cm × 6 cm × 4 cm) near the proximal jejunum Fig. . EsophagAn exploratory laparotomy was performed. At surgery, a large tumor was identified in the wall of the proximal jejunum, approximately 3 cm distal to the ligament of Treitz. Thorough exploration of the abdomen, including the liver, small intestine, pancreas, stomach, duodenum, mesentery, and retroperioneal regions in the upper abdomen and concomitant intraoperative ultrasonography (US) did not reveal any other tumors. Segmental resection of the upper jejunum, including a 7 cm × 6 cm ×4 cm tumor just distal to the ligament of Treitz, was done, and subsequent jejunal-jejunal end-to-end anastomosis was performed.Pathological findings of the jejunal tumor showed a neuroendocrine tumor consistent with gastrinoma. Microscopically, the tumor was composed of relatively uniform cells having centrally located nuclei and eosinophilic cytoplasm Fig. . The tumOn the fifth postoperative day, his fasting serum gastrin had decreased to 65.8 pg/mL. He was discharged 9 days postoperatively in fair condition without any complications. The patient was regularly followed up, and over 10 years after surgery, the patient remains free of acid regurgitation and abdominal discomfort and is in good health. Current gastrin levels are normal (75.60 pg/mL), esophagogastroduodenoscopy revealed no ulcers, and recent CT and magnetic resonance imaging (MRI) showed no signs of recurrent tumors.Primary jejunal gastrinomas are exceedingly rare, and data for long-term follow-up is limited. To our knowledge, only six cases of gastrinomas originating from the jejunal wall have been reported in the English literature to date Table  3–7].Ta–7.Ta3–7]Gastrinomas of the jejunum are usually small in size, with a reported diameter of 0.5–3 cm , 5. TherAlthough gastrinomas tend to be slow growing, 60 to 90 % of them were associated with metastases –10. LikeMany of the gastrinomas are small in size, which makes preoperative localization highly challenging. Furthermore, since the jejunum is not located within the gastrinoma triangle, this unexpected localization makes the diagnosis difficult. A number of tests are used to localize gastrinomas. CT scan, MRI, and ultrasound are used widely as the initial methods for localizing gastrinoma and are excellent for visualization of larger tumors. Somatostatin receptor scintigraphy (SRS) has a high sensitivity for assessing the extent of the disease. In recent years, SRS has become the imaging study of choice for identifying primary tumors and metastatic lesions in ZES –15. HoweThe discovery of a gastrinoma in the jejunum raises the question of whether the tumor is a primary or metastatic lesion. Exhaustive preoperative imaging as well as careful surgical exploration during laparotomy including intraoperative ultrasound revealed no additional lesions. Thus, the large jejunal tumor was considered to be the primary lesion. Still, it was possible that an occult primary lesion was missed by EUS, surgical exploration, and intraoperative ultrasound. However, plasma gastrin level normalized shortly after the excision of the tumor. Over 10 years after surgery, the patient is still eugastrinemic, and there is no evidence of tumor recurrence in long-term follow-up. Based on these findings, it is apparent that the jejunal tumor is a solitary primary gastrinoma.The current treatment of sporadic, resectable, non-metastatic gastrinomas is early surgical exploration and surgical resection. Reviews have stated that for sporadic gastrinomas, the initial postoperative cure rate is approximately 50–60 %, and the long-term cure rate is 35–40 % –22. TherAlthough primary jejunal gastrinomas are rare occurrences, it should be considered during the differential diagnosis of jejunal tumors if classic symptoms, such as uncontrolled reflux, non-healing mucosal ulcers or diarrhea are present. Here, we report an extremely rare case of a primary jejunal gastrinoma in which the tumor may be the largest reported to date. Over 10 years after segmental resection of the jejunum containing the gastrinoma, the patient is still eugastrinemic and asymptomatic with no evidence of tumor recurrence.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Under a diagnosis of hepatic and splenic metastases from right ovarian cancer, a laparoscopic splenectomy and partial hepatectomy were performed. Histopathological examination showed that a large number of non-caseating epithelioid cell granulomas formed these nodules, which was compatible with sarcoidosis. This case indicates that it is difficult to distinguish sarcoidosis from metastatic disease even using the latest modalities, and that laparoscopic surgery is a minimally invasive and useful tool for forming a differential diagnosis.The current study presents a case of sarcoidosis manifesting as hepatic and splenic nodules, which was difficult to differentiate from ovarian cancer metastases. A 24-year-old female, who was previously diagnosed with right ovarian cancer and underwent surgery at the age of 21, was found to have two nodules in the spleen revealed by contrast-enhanced computed tomography (CT).  Sarcoidosis is a systemic granulomatous disorder of unknown etiology, with pulmonary findings in >90% of patients . The ann2; days 1, 8 and 15), follow-up care was conducted on an outpatient basis every 3 months.In March 2007, a 21-year-old female underwent a right salpingo-oophorectomy, partial omentectomy and appendectomy due to right ovarian cancer at the Department of Obstetrics and Gynecology, Ehime University Hospital . Pathological examination of the tumor revealed multiple mucinous cysts, lined by mucinous tumor cells. Although adenomatous and borderline malignant regions were identified in the tumor, partial areas of carcinoma were also present, which proliferated and invaded the stroma. Thus, the ovarian tumor was diagnosed as mucinous cystadenocarcinoma. After performing adjuvant chemotherapy with three 28-day cycles of carboplatin  and paclitaxel (60 mg/m18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET)/CT was performed and revealed two abnormal high uptake lesions in the spleen, which were 2.5 and 1.0 cm in diameter, with a maximum standardized uptake value (SUVmax) of 21.0 and 18.2, respectively. The PET/CT also showed an abnormal high uptake lesion in the lateral segment of the liver, which was 1.5 cm in diameter, with an SUVmax of 14.5  showed two low density areas in the spleen. Thus,  of 14.5 .In October 2009, the patient was referred to the Department of Hepatobiliary-Pancreatic and Breast Surgery of Ehime University Hospital due to a working diagnosis of splenic and hepatic metastases from right ovarian cancer. A splenectomy and partial hepatectomy were recommended and the patient was admitted. Contrast-enhanced CT was performed again prior to admission and showed a low-attenuation nodule in the lateral segment of the liver, in addition to two low-attenuation nodules in the spleen . HepatomDue to the fact that the main differential diagnosis was splenic and hepatic metastases from right ovarian cancer, a laparoscopic procedure was performed. At the beginning of the laparoscopic procedure, an exploration of the abdominal cavity by a gynecologist was performed, which confirmed no abnormalities in the uterus or the adnexa of uterus, and no ascites or peritoneal dissemination. A cytological sample of peritoneal washing in the pouch of Douglas was obtained, and the presence of no malignant cells in the sample was confirmed the following day. The laparoscopic splenectomy and partial hepatectomy were then performed. Tumors were observed on the surface of the spleen . AlthougThe patient recovered well with no further treatment, such as steroid therapy, and neither recurrence of sarcoidosis in any other organs nor recurrence and metastasis of right ovarian cancer have been observed for 2 years and 5 months since the surgery. At 5 years and 6 months post-surgery the patient remained alive.et al reported that the rate of hepatic involvement was 5.6% among newly biopsy-proven sarcoidosis patients in Japan  was not performed pre-operatively. MRI may show foci of hypointensity on T2-weighted images in the liver, whereas neoplastic and other diseases tend to produce hyperintense signals . If MRI et al indicated that an elevated ACE level may be useful in the diagnosis of nodular hepatosplenic sarcoidosis (Additionally, there was no evidence of hypercalcemia, nor elevated levels of serum ACE and lysozyme in the present case. Epithelioid cells are believed to produce serum ACE within sarcoid granulomas. Warshauer coidosis . The stuFollowing diagnosis, continual follow-up for systemic manifestations and complications associated with sarcoidosis is required. Steroids are an important base in the treatment of active sarcoidosis. In the present case, steroids were not used after surgery, and the only follow-up care was conducted on an outpatient basis. Although it has been reported that the lungs are involved in >90% of patients , pulmonaCompared with previous studies of hepatosplenic sarcoidosis –9,20, thIn conclusion, the aforementioned unusual findings of hepatic and splenic sarcoidosis in the present case made it difficult to differentiate between sarcoidosis and metastases from ovarian cancer. This case suggests that it is difficult to distinguish sarcoidosis from metastatic disease even using the latest modalities, and that laparoscopic surgery is a minimally invasive and useful tool for forming a differential diagnosis.


The adrenocortical rest tumours are the very rare entity in the pediatric population. They are usually found along the gonadal descent paths . They have been also described to occur at rare ectopic sites like intracranial locations, placenta, kidney, pancreas and liver.Here we present a unusual case of an ectopic, virilising, primary adrenocortical tumour localized in the spinal region in a 8 years-old-boy.This is the first case of functional ectopic, adrenocortical tumour localized in the spinal region in a pediatric population. We discuss here the clinical presentation and the diagnostic challenges and provide the review of the literature. A 7.5-year-old boy presented to the Department of Endocrinology with symptoms of virilisation , acne and adult body odour.Our patient was born after a full-term, unremarkable pregnancy and exhibited normal development until the age of 5 years. At the age of 5.5 years, the first symptoms of virilisation started to appear (pubic hair and enlargement of penis) as well as acceleration of growth velocity. On admission to the Endocrinology Department, on physical examination the patient was found to have pubic hair (Tanner stage III/IV), a large penis measuring around 6 cm. His height was 144 cm (+3.25SD) with a weight 28.1 kg (5–10 centile according to the height) , a growth spurt was diagnosed (growth velocity 9 cm/year), his bone age was two years advanced. There was acne limited to the region of his face and adult body odour but no axillary hair and no striae. Testicular size (2 ml each) and structure were normal for age. Moreover, neurologic examination revealed that the patient had difficulty in walking , increased leg reflexes and right site foot-clonus. His bowel and bladder function were normal as well his sensation.Laboratory investigations revealed significantly raised serum androgen levels Table , abnormaThere was no androgen suppression observed during the 4-day Dexamethasone test  but normal cortisol suppression after the small dose of Dexamethasone. Urine catecholamine and their metabolites levels were normal.The abdominal and thoracic computed tomography (CT) scan and the adrenal magnetic resonance imaging (MRI) revealed no abnormalities. The ultrasound of the testes was normal. Adrenal scintigraphy (131 J-norcholesterol) showed no adrenal autonomic function.The brain MRI was normal, however spinal MRI T2- and contrast enhanced T1-weighted images demonstrated an ovoid, well-circumscribed, intradural extramedullary enhancing mass measuring approximately 2.8x2.1x6.0 cm . The patient was followed up and the normalization of androgen levels was observed in tests performed 9 days, 2 and 7 months after surgery Table . Neurolohttp://seer.cancer.gov/). There is a peak incidence before four years of age (0.4 cases per million) which then declines during the subsequent ten years. The incidence then rises to 0.2 per million during the late teens. The majority (60 %) of pediatric adrenocortical tumours occur in children less than five years [Adrenocortical tumours (ACT) are rare in children. It is estimated that there are 19–20 new cases of adrenocortical carcinoma in children and adolescents per year in the United States according to the Surveillance, Epidemiology and End Results Program  are collection of adrenal tissue that are found outside of the adrenal gland. The aberrant adrenal tissues are divided into heterotopia and the accessory adrenal gland. The former is derived from adrenal primordium that has migrated at an embryonic stage from neighboring organs such as kidneys or the liver; whereas the latter is the ectopia of fragmented adrenal tissues into the celiac axis, retroperitoneal cavity, uterus, broad ligament or testis .Ectopic adrenocortical tumour in the region of the spinal cord is a very rare phenomenon ,7. It isSo far there have been only 6 cases of ARTs localized in the spinal region reported in the literature Table .Table 2IThose tumours occurred in the lower spinal region at a median patient age of 22 years (5 months to 63 years) and with a notable female predominance (5:1). In 2004 Cassarino reported the first case of an ACT within the CNS in a male patient . This waIt is difficult to separate benign from malignant tumours. There is no single criterion and histopathological classification may not be reliable. Recently several, molecular markers have been investigated as a diagnostic aid: overexpression of IGF-2, IGF-1R, somatic p53 mutations, ki67 index and loss of heterozygosity of 11q13 . MorpholAll reported, up till now, tumours in the spinal region were non functional. In the literature there were only few cases of clinically-functioning adrenal rest tumours  but all In the present study, the young boy exhibited typical symptoms of virilizing tumour with high serum levels of androgens and characteristic urine steroid profile, normal cortisol circadian rhythm and normal suppressed serum cortisol level following Dexametasone administration.His clinical signs were pubic hair, enlargement of penis, growth acceleration, adult body odour, acne. We encountered difficulty in localization the tumour. Neither ultrasonography, CT or scintigraphy did not reveal the tumour and as well as brain MRI were all normal. Definite diagnosis was made with MRI of the spine. The diagnosis was of a benign adrenal tumour. Vascular invasion and metastasis were not found.Considering the final, adult height prediction in children with advance bone age due to hypersecretion of androgens, it is reported in the literature, that early recognition and treatment can normalize the growth pattern . AlthougIn summary we report the unique occurrence of a primary adrenocortical tumour in the spinal region of a young boy. To the best of our knowledge this is the first report of a ACT with the steroidogenic capacity within that localization. Although the occurrence of adrenocortical tumours in central nervous system is rare, pathologist should be aware that ectopic tumours both benign and malignant that may arise at this localization. Even in cases with no frank histological malignancy features present the patient should be followed-up.Written informed consent was obtained from the patient’s parents/legal guardians for publication of this case report and any accompanying images. A copy of the written consent is available for the review by the Editor-in-Chief of this journal.


Biliary tract neoplasms are commonly accounted for by cholangiocarcinoma and biliary cystadenoma/carcinoma and other less common tumors including papillary neoplasms, lymphoma and metastases. Although cholangiocarcinoma is a rare tumour , it is the second most common primary hepatobiliary malignant tumour after hepatocellular carcinoma (HCC). This tumour actually encompasses a diverse group of tumours varying greatly in location, growth pattern and histology resulting in a gamut of imaging manifestations. It is important to be familiar with those diverse manifestations to provide accurate detection and characterization. Since only surgery can provide curative therapy, accurate resectablity assessment is critical. Defining an optimal MRI protocol which includes precontrast MR imaging along with high resolution MRCP sequences and Dynamic contrast acquisitions/MR angiography is necessary to ensure accurate results. MRI offers unique advantages via its ability to provide information noninvasively in a single test regards tumour size, extent, vascular involvement, nodes and extrahepatic spread. MRCP can superbly display bile ducts upstream to an obstruction. According to its anatomic origin cholangiocarcinoma is usually classified as intrahepatic, perihilar, or extrahepatic distal or based on growth pattern as mass forming, infiltrating or polypoidal. Staging systems have been designed for anatomical location precise for surgical planning and to establish prognosis after surgery. MRI is not without limitations. In some cases other disease process may mimic cholangiocarcinoma and these will be discussed. At times MRI may not be able to confidently detect or stage the tumor and correlative imaging with Ultrasonography, CT and PET needs to be considered. Biliary cystic tumors, such as biliary cystadenoma (BCA) and cystadenocarcinoma (BCAC) constitute <5% of all liver cysts. BCA occurs predominantly in women (90%) with mean age of 45 years while BCAC can occur equally in men and women with mean age of around 55 years. Biliary cystic tumors are commomly multilocular with thick walls and enahancing sepatations on MRI. The differential diagnosis includes hydatid cyst, liver abscess, embryonal sarcoma, primary or metastatic necrotic neoplasm, and biliary intraductal papillary mucinous neoplasm (IPMN). Lymphoma involving bile ducts is secondary to systemic lymphoma but can result in biliary obstruction that micmics klatskin tumor or inflammatory cholangitic processes. Intrabiliary metastases are most commonly due to colorectal carcinoma metastases and also occasionally secondary to lung and breast carcinoma. Intrabiliary metastases can be mistaken for cholangiocarcinoma in the absence of knowledge of a primary malignancy.


Cutaneous metastasis of cholangiocarcinoma is extremely rare. It can be seen at distant locations or at the site of biliary drainage. To the best of our knowledge less than 30 cases have so far been reported in the English literature. This event should be considered in every skin lesion in a patient with cholangiocarcinoma and be treated promptly by resection and chemotherapy to increase the patient’s survival. Herein, we report our experience with two patients with cholangiocarcinoma and cutaneous metastasis at the site of biliary drainage. Cutaneous metastases of internal malignancies are rare and the reported incidence is about 0.7%–9% . CholangSome of the reported cases have been distant metastasis, especially to the scalp , howeverCase 1A 38-year-old man with a 2-year history of primary sclerosing cholangitis, one year post liver transplantation presented with a subcutaneous mass at the site of biliary drainage. He had a biliary drain for about three months before the transplantation. His laboratory results at the time of presentation with subcutaneous mass included a white blood cell count (WBC) of 10,400/µL, hemoglobin level of 9.6 g/dL, alanine aminotrasferase (ALT) of 43 IU/L, aspartate aminotransferase (AST) of 40 IU, and a total bilirubin level of 1.5 mg/dL.The patient was found to have cholangiocarcinoma after pathologic examination of the explanted liver, however, he did not receive any medications other than immunosuppressives for the liver transplantation. The large subcutaneous mass (4 cm in its greatest diameter) was resected ; patholoCase 2This 41-year-old man had a 2-month history of cholangiocarcinoma. The diagnosis was made based on a liver biopsy and confirmed by immunohistochemical staining. He presented with percutaneous biliary drainage for one month and developed an abscess-like lesion at the site of previous drain in the right upper quadrant with bloody discharge.His laboratory examination at the time of presentation was a WBC of 12,400/µL, hemoglobin level of 8 g/dL, ALT of 226 IU/L, AST of 410 IU/L, and a total bilirubin level of 21.5 (direct: 13.8) mg/dL.The patient received antibiotics without complete response. Gradually, the abscess became firm and a mass-like lesion was formed, measuring about 4 cm in diameter. The mass was excised the pathological examination of which revealed a mucin-producing adenocarcinoma, consistent with the previous morphology of cholangiocarcinoma . At the Since 1973, percutaneous transhepatic biliary decompression has been used as a diagnostic and therapeutic means in patients with obstructive jaundice . This prThe period between placement of the drain and diagnosis of the cutaneous metastasis in cholangiocarcinoma is between 3 and 30 months .Most of the reports on cutaneous metastasis in cholangiocarcinoma reveal that the metastasis is usually presented as a sudden-onset rapid-growing firm painless nodules with different clinical pictures including abscess, infection, panniculitis and scar tissue .Some reports showed that catheter tract subcutaneous metastasis is mostly occur in well-differentiated cholangiocarcinoma. In the 20 previously reported cases, only one had poorly differentiated neoplasm . Both ofAlthough cutaneous metastasis is a sign of advanced disease with 6–18 months survival , early dCase 2 was a middle-aged man with 2-month history of cholangiocarcinoma who developed an abscess-like lesion at the site of biliary drain. He died after less than one month of the diagnosis of the metastasis. He had been treated with a diagnosis of abscess for a while. However,Case 1 was promptly diagnosed; the subcutaneous mass was resected in less than a week, and he is still alive.The problem of how to prevent catheter tract implantation metastasis in cholangiocarcinoma is an important issue yet to be solved. One proposed procedure is to excise the whole catheter tract between the surface of the skin and the punctured intrahepatic bile ducts, which can be difficult .In conclusion, cutaneous metastasis of cholangiocarcinoma at the site of biliary drainage is though rare, should be considered as a poor prognostic sign of the disease and be treated promptly.


Intersigmoid hernia is a rare form of internal hernia. Here, we report a case of intersigmoid hernia and provide a brief review of the 62 cases involving the mesosigmoid reported in Japan from 2000 to 2013. In the current case, a 26-year-old man with no previous history of abdominal surgery presented with abdominal pain and vomiting. Abdominal computed tomography revealed an extensively dilated small bowel and a closed loop of small bowel in the mesosigmoid. The patient was diagnosed with an intestinal obstruction due to an incarcerated internal hernia involving the mesosigmoid. There was no blood flow obstruction at the incarcerated bowel. An elective single-incision laparoscopic surgery was performed after decompression of the bowel using ileus tube. As the ileum herniated into the intersigmoid fossa, the patient was diagnosed with an intersigmoid hernia. The incarcerated small bowel was reduced in order to make it viable, and the hernial defect was closed with interrupted sutures. The patient had an uneventful recovery and was discharged on postoperative day five. Recently, laparoscopic surgery for acute abdomen, including intestinal obstruction, has become widely accepted among surgeons. Single-incision laparoscopic surgery is performed with an increasing frequency in many surgical centers. Although intersigmoid hernia is a rare form of internal hernia , this re3, a C-reactive protein level of 0.3 mg/dL, and all other measured values within their normal limits.A 26-year-old man who presented with abdominal pain and vomiting was transferred to our hospital seven days after the onset of the symptoms. The patient had no history of abdominal surgery. On arrival, his vital signs were normal. Abdominal findings revealed distension and tenderness in the entire abdomen without any muscular defense signs. Laboratory examinations showed a white blood cell count of 7420/mmAn abdominal X-ray examination showed a dilatation and an air-fluid level of the small bowel. Abdominal computed tomography revealed an extensively dilated small bowel and a closed loop of small bowel in the mesosigmoid . There wThe operation was performed using a 2.0 cm transumbilical vertical incision. The fascia and peritoneum were then vertically opened, and three 5 mm trocars were inserted into the abdomen to introduce endoscopic instruments. Carbon dioxide was used to inflate the abdomen at 8 mmHg pressure. The operator used two 5 mm forceps, and the first assistant handled a 30 degree 5 mm laparoscope . During the surgery, it was observed that the ileum had herniated into the intersigmoid fossa. Furthermore, there were mild adhesions of the herniated ileum at the hernial orifice . The incInternal hernia is a rare cause of obstruction. Although the intersigmoid fossa is found in 65% of all autopsies , sigmoidBenson and Killen  classifi


Here, we report the successful treatment of a 40-year-old man with mucoepidermoid carcinoma that originated in the proximal end of the left main bronchus close to the carina. He underwent wide and deep airway wedge resection, including the distal trachea and part of the carina via left postero-lateral thoracotomy. He has demonstrated neither anatomic complications nor disease recurrence 2 years after the operation. A low-grade malignant tumor in the large airway is a good indication for one-stoma-type bronchoplasty, which does not necessitate sacrificing of the parenchyma . HoweverA 40-year-old man, complaining bloody sputum and a non-productive cough, was referred to our hospital. Computed tomography (CT) with multiplanar reconstruction revealed a 25-mm polypoid tumor that had originated in the lateral and distal portion of the trachea and protruded into the airway . Under gTo expose the left main bronchus and the carina, left postero-lateral thoracotomy through the fourth intercostal space was selected. The intraoperative ventilation was performed with the double-lumen endobronchial tube for the right bronchus. Accessing the carina was possible by bringing it down toward the operator below the aortic arch by encircling the trachea with traction tape. While encircling the trachea, the part of azygos vein were exposed, but SVC were not exposed. #10R and #4R lymph nodes were not dissected. After Botallo’s ligament was divided, the aortic arch was retracted upward and the pulmonary artery was retracted downward to create better exposure. The tumor growing outside of the bronchial cartilage was resected via deep wedge and wide resection, including the trachea and part of the carina . TracheaLow-grade malignant tumors of the bronchial airway are often managed via circumferential or wedge bronchial resection, because they require minimal surgical margins . Such neKnotting inside the airway makes anastomosis easier, especially in cases of a deep operative field. Here, the three knots placed inside of the airway at the anastomotic site did not cause any airway trouble during the postoperative course. This technique would be more reliable in cases of a deep operative field in the mediastinum and in cases of a more complicated anastomosis.Bronchoplasty without sacrificing of the parenchyma for a low-grade malignant tumor that originated in the main bronchus is an effective operation. However, it is necessary to consider before surgery, such as approaches and surgical procedures.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


A mammary-type myofibroblastoma is a rare soft tumor; extramammary myofibroblastomas are especially rare.A 51-year-old woman presented to our department for evaluation of a mass on the right lower abdominal wall. The mass was then excised completely. Gross examination showed a huge, well-circumscribed soft tissue mass. The pathologic diagnosis was an extramammary myofibroblastoma. There was no recurrence after excision at the 6-month follow-up visit.A mammary-type myofibroblastoma is a benign soft tissue neoplasm. No malignant behavior and/or recurrence of mammary-type myofibroblastomas after surgical resection have been described as a function of size and location. The present case aimed to provide a possible differential diagnosis for such abdominal masses. Mammary myofibroblastomas are benign tumors, which were first described in the breast by Wargotz et al. in 1987 , 3 have A 51-year-old female presented to our department  complaining of a mass on the right lower abdominal wall that had been there for 20 years. The patient had normal vital signs. The mass on the right lateral abdominal wall was located through physical examination of the abdomen. The mass was approximately 2 × 4 cm in diameter, well-circumscribed, firm, and painless. Laboratory testing (blood and biochemistry routine examination) was normal. Computed tomography , and Epstein-Barr virus latent membrane protein 1. Reactivity to factor VIII-related antigen , EMA, MAK-6 , and glial fibrillary acidic protein is negative , 12, 13.The differential diagnosis of this tumor includes the following: Kaposi’s sarcoma, leiomyosarcoma, intranodal schwannoma, benign metastasizing leiomyoma (BML), follicular dendritic cell sarcoma (FDCS), inflammatory myofibroblastic tumor (IMT), metastatic malignant melanoma, and metastatic carcinoma .Kaposi’s sarcoma is primarily a borderline vascular tumor implicating the skin and mucosa, but in more advanced stages, the lymph nodes and almost any internal organ could also be involved; human herpesvirus 8 (HHV8) is considered the causative agent for Kaposi’s sarcoma , while mLeiomyosarcoma is a malignant mesenchymal tumor with smooth muscle origin that represents 5–7 % of all soft tissue sarcomas , which iIntranodal schwannoma were often positive for vimentin and S100, which are similar with myofibroblastomas, and negative for SMA and CD34, which are not similar with myofibroblastomas. The findings of intranodal schwannoma were consistent with a diagnosis of primary schwannoma of the lymph node .BML is a rare neoplastic process in which of the uterine leiomyomas metastasize to distant sites, the most common of which are the lungs , and theDendritic cell sarcoma (DCS) been previously described by many terms such as lymphoma, sarcoma, or histiocytic neoplasm, reflecting the controversy about the tumors . FDCS isIMT is a rare mesenchymal tumor and has been considered an inflammatory condition; the presentations of neoplastic proliferation include invasive potential, recurrences, and metastases . It is mMetastatic malignant melanoma and metastatic carcinoma could metastasize to any organ or multiple organs. As typical symptoms are not specific and show organ-specific outcomes depending on metastasized lesions, it is difficult to be suspected at the onset . MyofibrExtramammary-type myofibroblastomas are benign soft masses without malignant potential. Surgical resection is the preferred method for cure, and the recurrence rate is low. As an extremely rare tumor, physicians should pay more attention to differential diagnosis.Written informed consent was obtained from the patient for publication of this case report and the accompanying images.


Acute hemorrhagic leukoencephalitis is a fulminant demyelinating disease and commonly considered as a rare and severe variant of acute disseminated encephalomyelitis. Here, we report the clinical, magnetic resonance imaging, and brain biopsy findings of a 35-year-old female with relapsing-remitting multiple sclerosis, who developed acute hemorrhagic leukoencephalitis. Magnetic resonance imaging revealed symmetrical hemorrhagic lesions in the basal ganglia including the thalami. Disease progression was consistent with acute hemorrhagic leukoencephalitis with rapid deterioration of consciousness and seizures. Besides hemorrhage, infiltration of neutrophils was detected in brain biopsy.Acute hemorrhagic leukoencephalitis, also known as Weston-Hurst syndrome, is an excessive immunological response of unknown etiology. So far, an association with multiple sclerosis has not been reported. The present case raises the question, whether acute hemorrhagic leukoencephalitis is a specific hyperacute form of acute disseminated encephalomyelitis, a severe and unspecific form of an immune response in the central nervous system, or belongs to the spectrum of tumefactive multiple sclerosis. Acute hemorrhagic leukoencephalitis (AHLE) was first described by Weston Hurst in 1941. It is commonly considered as a rare and severe variant of acute disseminated encephalomyelitis (ADEM) and represents a rather fulminant course of this disease . MultiplA then 21-year-old woman was diagnosed with relapsing-remitting MS in December 1993 according to the MS criteria of Poser et al. . In 1996Following intubation, the patient was transferred to the intensive care unit. The computed tomography (CT) showed hypodensities in the basal ganglia, frontal lobe, and mesencephalon. Internal venous thrombosis was ruled out by CT venous angiography; MRI revealed symmetrical hyperintense lesions in the basal ganglia on T1-weighted and punctate hemorrhages on T2-weighted images Fig. . CerebroBorrelia burgdorferi, syphilis, tuberculosis, Cryptococcus, and toxoplasmosis. There was no infection with HIV or hepatitis B and C. Thus, MRI-guided diagnostic brain biopsy of the parenchyma was performed. The predominant findings in the biopsy specimen were perivascular hemorrhagic necrosis and severe edema of the CNS tissue with an inflammatory infiltrate principally composed of neutrophils. Some of the vessels were partially thrombosed  might be another differential diagnosis in this case. It is a new disease entity proposed by Mizuguchi et al. in 1997 and is characterized by symmetrical, multifocal lesions, usually associated with edematous necrosis and hemorrhage, without inflammatory cells . ANE alwThe clinical differentiation between AHLE and ANE is a challenge because both diseases present similar findings on MRI and CSF. In this regard, the diagnostic criteria of ANE, proposed by Neilson et al. in 2010, might help to discern the appropriate diagnosis . The cruA literature search for similar cases was performed. We found a case of a 28-year-old woman who participated in a placebo-controlled trial of oral fingolimod in relapsing MS . She developed hemorrhaging focal encephalitis in the left temporoparietal white matter 7 months after commencing fingolimod . A markeThe term tumefactive MS refers to demyelinating lesions that are larger than those seen in “typical” MS, have an accompanying edema, and have mass effect resulting in symptoms and signs that are atypical for MS. Involvement of basal ganglia have been reported , 12. In AHLE and ADEM share many features since both are commonly preceded by upper respiratory infections or by HSV, EBV, rubella, measles, mumps, or influenza virus infection or vaccination even though overt symptoms of a preceding infection could not be found in the present case . It remaIn conclusion, the present case illustrates the uncommon presentation of AHLE in a MS patient. The diagnostic work-up is challenging, and brain biopsy offers guidance in the diagnosis of AHLE. The present case raises the question whether AHLE belongs to the spectrum of ADEM or displays a severe and discrete form of an immune response in the CNS.Written informed consent was obtained from the legal guardian of the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the editor in chief of this journal.


Hydatid cyst is an infectious disease characterized by cysts formed primarly within the gastrointestinal tract by echinococci. Hepatic hydatid disease, which is the most common form, remains asymptomatic until complications occur. In this report, we present an 80 years old patient who presented with a hepatic hydatid cyst which fistulized to the abdominal skin into the Emergency Department. Computed tomography of the abdomen showed inactive grade 5 cyst. Drainage without removal of the cyst failed to reveal active disease but the microbiological examination showed Klebsiella pneumonia that was sensitive to ampicillin–sulbactam as the causative agent. The treatment of the cyst with a combination of surgical and medical treatment was the successful treatment of Hepatic Hydatid Disease presenting with a cutaneous fistula. Echinococcus, affecting primarily the hepatobiliary, respiratory, and less frequently, the central nervous system. The cysts resulting from the parasitic infection remain asymptomatic for many years, and the initial presentation is usually due to the complications arising from fistula formation. The hepatobiliary system is the most common site of the fistulae, while cutaneous fistulae are rare  with right upper quadrant abdominal pain and a cutaneous lesion with white, purulent discharge that appeared a week ago in the same region. The patient’s medical history was notable for an operation for the removal of a liver abscess of unknown etiology fifteen years ago; otherwise, no other chronic illnesses were present.Echinococcus infection; however, Klebsiella pneumonia that was susceptible to most commonly used antibiotics was cultured from the purulent material, upon which a 14-day course of ampicillin-sulbactam was initiated. The patient underwent repeated abdominal ultrasonography, which failed to show a recurrence of similar cystic lesions in the abdomen. The patient was discharged 7 days after hospitalization and was followed at the outpatient clinic. The evaluation of the patient at an outpatient setting by ultrasound imaging 6 months after the procedure did not show a recurrence of the cyst.During physical examination, the abdominal wall was tender on palpation, and the examination of the lesion in the upper right quadrant revealed a fistula opening with white fluid discharge. The physical examination of other systems was normal. The abdominal ultrasonography performed in the ED showed a cystic mass with a diameter of 6 cm in the seventh and eighth segment: the cyst had a calcified membrane and a comet-tail artifact that indicated the presence of air. The cystic lesion appeared to protrude to the subcapsular area and subcutaneous tissue: these findings were highly suggestive of a fistula. The biochemical values and complete blood counts were within normal limits. An abdominal computed tomography with intravenous contrast revealed a single, calcified, membranous lesion in segments 8 and 4A with a fistula to the abdominal anterior wall Fig. . In addiEchinococcus parasites. The parasitic life cycle includes both herbivores and carnivores, including dogs and sheep; however, humans may become infected as intermediate through the ingestion of infected organs. Cystic echinococcosis presents as multiple cysts throughout the body, and is epidemic in the Mediterranean and certain parts of Africa and Central Asia. However, increased transcontinental travel has led to an increase in cystic echinococcosis in nonepidemic regions as well.Human echinococcosis is a zoonotic infection caused by one or more of the four known strains of In humans, the asymptomatic initial infection is followed by the symptomatic phase, the type and severity of which depends on the location, size, number and mass effect of the cyst/s. For example, a noncomplicated cyst can cause symptoms secondary to its local affects such as the enlargement of the affected organ and impingement on surrounding tissue. When liver is infected, increased pressure exerted by the cyst can produce symptoms of jaundice or abdominal pain, whereas lung involvement can present as hemoptysis, dyspnea or chronic cough.A common complication of the cystic echinococcosis is the cystic rupture and subsequent allergic reaction to the parasitic material, which ranges from urticaria to anaphylaxis depending on the volume of the leaked material. Other commonly observed complications include the compression of, or communication with, the biliary tree and secondary echinococcosis , a type 5 cyst is defined as calcification of the cystic membranes, as determined by imaging characteristics, similar to our observations in our patient. Typically, a type 5 cyst is presumed inactive; however, in rare cases, an abscess with cutaneous fistula/e can occur in the calcified cavity, and typically presents with symptoms that were also observed in our patient. Importantly, previously reported cases of type 5 cysts include cutaneous fistulae with cystic material drainage in the absence of concomitant bacterial infection. In contrast, the microbiological evaluation of aspirated material in our patient did not reveal any active parasites, but was positive for Lewall and McCorkell have classified the rapture of hepatic echinococcal cysts into three (Lewall and McCorkell Klebsiella pneumonia infections are typically localized to the lower respiratory, urinary, and biliary tracts; surgical wound sites are also commonly infected. Predisposing factors include alcohol abuse, diabetes, malignancy, liver parenchymal disease and chronic obstructive pulmonary disease. The rate of reported pyogenic Klebsiella pneumonia liver abscesses are increasing (Lederman and Crum Klebsiella pneumonia.Based on our experience, we recommend that the drainage of the infected material with antibiotic treatment for the secondary bacterial infection may be an alternative approach in cases when surgical removal of the cyst may not be achievable due to confounding factors.Klebsiella pneumonia may be the cause of abscess formation in a subset of patients.In summary, cutaneous fistulae originating from hepatic echinococcal cysts is a rare complication of active cysts. Grade 5 cysts are considered inactive; however, the formation of fistulae to abdominal wall and superinfection by a second microorganism, as that occurred in our patient, can be successfully treated with drainage. This approach appears to be feasible with no risk of recurrence in cases where the cyst cannot be removed. Finally,


We report a case of an undifferentiated pleomorphic sarcoma in a 73-year-old female, with a solitary lung metastasis involving the pericardium that progressed on first-line chemotherapy. Partial removal of the lesion was achieved after lingular segmentectomy, which required en-bloc pericardial resection due to deep pericardial invasion. However, the residual disease significantly grew despite second-line chemotherapy, and the tumor became unresectable due to near encasement of the left anterior descending coronary artery. Therefore, we opted for a salvage radical dose of intensity-modulated radiotherapy (60Gy in 25 fractions) to the pericardial lesion. No acute side effects were observed, and after three years of follow-up, good local control has been achieved with no significant late effects observed. This case suggests that radical radiotherapy using IMRT could be considered to treat sarcomatous pericardial lesions in patients who do not respond to chemotherapy and who are inoperable or non-resectable. Undifferentiated pleomorphic sarcomas (UPS) account for less than 5% of adult soft-tissue sarcomas (STS) . HoweverPulmonary metastases located near or involving the heart pose a particular challenge to radiation oncologists due to cardiac-dose limitations, which account for potential radiation-induced cardiotoxicity.  Advanced techniques, such as intensity-modulated radiotherapy (IMRT) offer improved tumor-dose conformity and normal tissue sparing, but limited information is available on its effectiveness for treating metastases with pericardial involvement from STS. Here, we report a case in which a radical dose of radiotherapy using IMRT produced excellent tumor control without acute or long-term treatment-related side effects for a chemo-refractory, non-resectable, sarcomatous pulmonary metastasis with pericardial involvement. In 2009, a 73-year-old female noticed a painful left thigh mass that increased in size over 6 months, causing functional impairment. On exam, an indurated and hard mass was palpable in her medial left thigh, measuring 10 cm craniocaudally. Subsequent MRI showed a large heterogeneously enhancing mass in the vastus medialis muscle extending into the vastus intermedius, consistent with a sarcomatous lesion. Core biopsies confirmed high-grade UPS. Staging CT images were negative for metastatic disease. She received preoperative radiotherapy to the tumor volume, 50 Gy in 25 fractions, followed by surgical resection one month later. Pathology confirmed UPS measuring 12x8x7 cm and showing 95% necrosis. Resection margins were negative. The patient declined adjuvant chemotherapy.In July 2010, four months post-surgery, a follow-up CT chest showed two new dominant, well-circumscribed pulmonary nodules consistent with metastases. All other imaging investigations were negative. Surgical resection was initially planned; however, a pre-operative repeat CT thorax showed an increasing number of pulmonary nodules. The patient was no longer a candidate for surgery, and she proceeded to receive six cycles of palliative doxorubicin. All of her lung nodules resolved, but one left upper lobe nodule increased . Needle biopsy of this lesion showed predominantly necrosis. Surgery was again planned to resect this isolated focus of metastasis. However, in July 2011 while waiting for surgery, the patient presented to the emergency department with retrosternal chest pain. A CT chest showed dramatic growth of her left upper lobe lesion, now measuring 6x7 cm, sitting adjacent to the anterior pericardium Figure . Three wThe postoperative CT showed a residual 2.7x1.4 cm soft tissue density in the left anterior mediastinum adjacent to the anterior pericardial line, consistent with residual tumor Figure . PalliatA 4D-CT chest simulation with contrast was completed with the patient in supine position over a lung board. The gross tumor volume (GTV) was the mass in free-breathing CT, and the GTV maximum intensity projection (MIP) was the GTV visualized on the 4D-CT images set, using all phases of respiration as done routinely for most lung stereotactic body radiation treatments. Both GTV and GTV MIP were combined to create the internal treatment volume (ITV). One-centimeter margins were added to the ITV to create the clinical target volume (CTV), with an additional 5-millimeter margin added for the planning target volume (PTV). The PTV was prescribed in a dose of 50 Gy in 25 fractions of 2 Gy, and the ITV concomitantly received a dose of 60 Gy in 25 fractions of 2.4 Gy. IMRT step-and-shoot technique using 5 coplanar beams spaced from 25 to 120 degrees and covering a sector from 25 to 350 degrees were defined. Dose calculations were done on the MONACO treatment planning system using 6MV photons and the Monte Carlo algorithm. The organs at risk were the heart and cardiac subunits (Figure  The patient continued to show no signs or symptoms of moderate-to-late radiation-induced side effects for three years post-radiotherapy, with the patient last seen in-clinic in November 2015. Follow-up CTs during this time also showed a stable pericardial lesion with no progression or disease recurrence, including the most-recent CT in July 2015 Figure . This case highlights the safe and long-term tumor control achieved by using salvage IMRT for a chemotherapy-resistant, non-resectable sarcomatous lung metastasis with pericardial involvement. Generally, chemotherapy and/or surgery are first considered in the treatment of pulmonary metastases from STS--depending on the number of lesions, site, and disease-free interval time . UnfortuThe location of the tumor within the pericardium was challenging due to the possible acute and long-term radiation-induced cardiac side effects including pericarditis, angina and myocardial infarction . In addiDespite this, the role of radiotherapy in managing advanced STS has increased with recent technological advancements, such as IMRT’s ability to improve tumor-dose conformity and normal tissue sparing . We applOur case report suggests that high-dose radiotherapy using IMRT is a feasible treatment for UPS lung metastases involving the pericardium and may provide excellent tumor control without significant radiation-induced side effects.


A 45-year-old female patient presented with symptoms of polydipsia and polyuria, menopause, headache, gait disturbance and deteriorated mental state. Brain magnetic resonance imaging (MRI) showed an irregular mass in the anterior third ventricle. The tumor was excised using a transfrontal approach from the anterior section of the third ventricle. The histological diagnosis was of an intracranial pure yolk sac tumor. The patient underwent radiotherapy and suffered no tumor recurrence one year after the surgery. Overall, when heterogeneous enhancement and an irregular mass with surrounding invasion and ventricular dilation are observed in the anterior third ventricle of an adult, a yolk sac tumor should be considered, and MRI may aid the differential diagnosis. A combination of surgical resection and radiotherapy is recommended for the yolk sac tumor. Intracranial germ cell tumors are classified into two fundamental types: Germinomas and nongerminomatous germ cell tumors. Yolk sac tumor (YST), also known as endodermal sinus tumor, is a nongerminomatous germ cell tumor ,2. TwelvA 45-year-old female patient was admitted to the Second Hospital of Shandong University  on October 31, 2012. The patient presented with symptoms of polydipsia and polyuria for 19 years, menopause for two years, headache for nine days, gait disturbance and deteriorated mental state, but with no evidence of visual field defects or papilledema. Brain magnetic resonance imaging (MRI) performed on November 1, 2012 demonstrated the presence of a mass in the anterior third ventricle with dimensions of 3.2×2.4×2.3 cm, appearing with low intensity on T1-weighted images, and hyperintensity on T2-weighted images. Strong gadolinium enhancement was present with the exception of at the hemorrhagic center. The tumor had spread to the left section of the lateral ventricles, and the third and lateral ventricles were dilated . The neuOn November 12, 2012, the tumor was excised using a transfrontal approach from the anterior section of the third ventricle. The tumor was located in the anterior third ventricle, blocking the interventricular foramen, and partially invaded the lateral ventricle through the interventricular foramen. Immunohistochemical staining of α-fetoprotein (AFP) showed strong cytoplasmic staining. The histological diagnosis was of intracranial pure YST. Subsequently, the patient received whole-brain radiotherapy (30–36 Gy) and focal radiotherapy (45 Gy). All the procedures carried out in the present study were approved by the ethics committee of Shandong University  and written and informed consent was obtained from the patient prior to participation. During the 12 months of follow-up, the majority of the symptoms disappeared with the exception of headache. MRI revealed chronic hemorrhage but no residual tumor.Since radiological manifestations can be nonspecific, the preoperative diagnosis of a pure YST is challenging. In the present case, the mass was irregular and located in the anterior third ventricle, appearing as an area of low intensity on T1-weighted images and an area of hyperintensity on T2-weighted images. Strong gadolinium enhancement was present with the exception of at the hemorrhagic center. The preoperative diagnosis in the present case was of craniopharyngioma. Hemorrhage is not a characteristic radiological feature of craniopharyngioma . The difet al (According to the classification of intracranial germ cell tumors described by Matsutani et al , YST belet al . In the In conclusion, to the best of our knowledge, the present case is the first report of a pure YST in the anterior third ventricle of a middle-aged patient. Although the definitive diagnosis of intracranial YST depends on the pathologic examination , MRI may


Double-balloon enteroscopy revealed a submucosal tumor in the jejunum, and histopathology of biopsy specimens from that lesion showed ectopic pancreas without malignancy. Therefore, peritoneal metastasis from colon cancer concomitant with ectopic pancreas or adenocarcinoma arising from ectopic pancreas was considered as a differential diagnosis. She underwent laparoscopic jejunectomy. Pathological examination revealed a moderately differentiated adenocarcinoma arising from jejunal ectopic pancreas, not peritoneal metastasis from colon cancer. Even if histopathology of the biopsy specimen shows ectopic pancreas without malignancy, adenocarcinoma arising from ectopic pancreas should be considered when the tumor markers are elevated or the lesion has an abnormal uptake of 18FDG.Adenocarcinoma arising from jejunal ectopic pancreas is very rare. We report a case of a 69-year-old female with adenocarcinoma arising from jejunal ectopic pancreas after resection of advanced colon cancer. She underwent right hemicolectomy for advanced ascending colon cancer  after chemotherapy. Two and half years after colectomy, her tumor markers were elevated, and computed tomography revealed a mass measuring 20 × 20 mm in the small intestine, having an abnormal uptake of  Ectopic pancreas is defined as pancreatic tissue lacking anatomical and vascular continuity with the normally located pancreas. Ectopic pancreas is usually an asymptomatic, incidental finding at laparotomy or autopsy, and its malignant transformation is extremely rare, especially in persons with jejunal ectopic pancreas –3. There18F-fluorodeoxyglucose-positron emission tomography/computed tomography (18FDG-PET/CT) revealed an abnormal uptake of 18FDG in that region, with maximum standardized uptake values of 5.7 , computed tomography (CT) showed a remarkable reduction in tumor size in the ascending colon. The sizes of the patient’s pulmonary nodules were not changed; hence, they were considered as benign lesions, and the primary lesion was regarded as resectable. She underwent right hemicolectomy with lymphadenectomy as conversion therapy. The specimen could be removed without duodenectomy. Histopathologically, the tumor was 35 × 34 mm, tub2, ly0, v2, ypT3, ypN0, classified as stage IIA according to the Tumor Node Metastasis Classification of the International Union Against Cancer, version 7.0 [The findings of histopathological examination were as follows: ectopic pancreatic tissue was detected below the mucosal layer, and consisted of acini and ducts, without Langerhans cells Fig. a; modera18FDG. Biopsy of these lesions was recommended to the patient; however, she rejected it. Peritoneal metastasis from ectopic pancreatic adenocarcinoma was suspected rather than peritoneal metastasis from colon cancer because the serum CA19-9 level was almost normal before treatment of colon cancer was begun  started to be administrated as adjuvant chemotherapy, according to the protocol for treatment of resected pancreatic cancer . Howevergun Fig. . TherefoEctopic pancreas is the presence of pancreatic tissue located outside the normal pancreas. It is observed in 0.5 % of upper abdominal laparotomies and 0.6 to 13.7 % of autopsy studies . The HeiThe incidence of malignant transformation of ectopic pancreas has been reported to range from 0.7 to 1.8 % , 8. Crit18FDG in that region in 18FDG-PET. First, peritoneal metastasis from colon cancer or primary small intestinal cancer was suspected. However, double-balloon enteroscopy revealed a submucosal tumor in the jejunum, and histopathology of the biopsy specimens showed ectopic pancreas without malignancy. On the basis of the findings of elevated tumor marker and abnormal uptake of 18FDG, peritoneal metastasis from colon cancer with ectopic pancreas or adenocarcinoma arising from ectopic pancreas was considered as a differential diagnosis; therefore, resection of that lesion was required.In our case, the tumor markers, measured for surveillance of recurrence after resection of advanced colon cancer, were elevated. CT revealed a mass with abnormal uptake of We performed a PubMed search of the English literature from 1966 to 2014 using the following keywords: ectopic, aberrant, heterotopic, pancreas, jejunum, small intestine, adenocarcinoma, and cancer. We also retrieved cases from the relevant reference lists. We found six previous reports of adenocarcinoma arising from jejunal ectopic pancreas (Table 18FDG.In summary, we report a rare case of adenocarcinoma arising from jejunal ectopic pancreas after resection of advanced colon cancer. Even if histopathology of the biopsy specimen shows ectopic pancreas without malignancy, adenocarcinoma arising from ectopic pancreas should be considered when the tumor markers are elevated or the lesion has an abnormal uptake of Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the editor in chief of this journal.


Epidermoid cysts are rare, slow‑growing, benign, developmental cysts, which are derived from abnormally situated ectodermal tissue. Epidermoid cysts of the floor of the mouth represent <0.01% of all oral cysts. So far, only a few cases have been reported. Hereby, we present a case of a giant sublingual epidermoid cyst, which was completely asymptomatic upon presentation. However, due to its large size, it pushed the epiglottis posteriorly and created difficulty during intubation. The patient developed respiratory distress after its surgical excision and extubation, requiring tracheostomy post operatively. The patient recovered well and a successful weaning of tracheostomy was performed, giving the patient a healthy life.Epidermoid cyst is a rare differential diagnosis of sublingual swelling that should be kept in mind for large asymptomatic swellings in this region. The only symptom it can cause might be respiratory distress due to its large size. This can happen not only pre-operatively but also post-operatively and the surgeon should be ready for immediate tracheostomy. Epidermoid cysts are rare, slow‑growing, benign, developmental cysts that are derived from abnormally situated ectodermal tissue. It is defined as, “a simple cyst lined with stratified squamous epithelium and lumen is filled with cystic fluid or keratin and no other specialized structure” . EpidermA 60 yr. old male presented to ENT OPD of our hospital with a large swelling in the submental region, which had been present for 30 yrs. Swelling was initially small but gradually increased over the years to reach its present size. The only complaint he had was a large visible swelling in the submental region. No other complaints like pain, dysphagia, dyspnea or difficulty in speech were associated with swelling. There was no history of sudden increase in size of swelling. Past medical, surgical, and dental history was also not relevant. During clinical examination, the swelling was found to be globular, around 13cm x 12cm in size, involving the whole submental region and extending to the right submandibular region ,2.Upon palpation, the swelling was soft to firm, with doughy consistency, non-tender, non-mobile, non-fluctuant, and non-trans illuminant. The swelling did not move with deglutition or with protrusion of tongue. Overlying skin was normal in color and texture and free from underlying swelling. Skin was easily pinchable. There was no sinus, discharge or ulcer seen. The swelling did not produce any significant bulge intra-orally in the floor of the mouth. However due to its large size, it caused significant posterior displacement of the epiglottis and hence indirect laryngoscopic examination could not be performed. With this clinical finding, differential diagnosis of epidermoid cyst, dermoid cyst or plunging ranula was made.USG of the neck revealed a 10cm x 8cm well-defined, pseudo-solid cystic, space occupying lesion, containing dense homogenous internal echoes with few calcific specks giving acoustic shadow (mostly keratin). The lesion was pushing the right submandibular gland laterally.CT scan of the affected area showed a large, well-defined, thick walled, peripherally enhancing cystic lesion measuring 10.3cm x 8.1cm in the midline submandibular region ,4. th post-op day. Tracheostomy weaning was done on the 10th post-op day. The patient was discharged with a smiling face after successful weaning of tracheostomy and complete recovery , III: blockage of Wharton’s duct, IV: lipoma, V: thyroglossal duct cyst, VI: cystic hygroma, VII: branchial cleft cyst, VIII: benign and malignant tumor of the floor of the mouth and adjacent salivary gland . AbsolutEpidermoid cysts are benign, ectoderm lined, inclusion cysts without any dermal appendages. Various theories have been proposed regarding the origin of the dermoid/ epidermoid cyst. As mentioned earlier, they may develop from the entrapment of epithelial remnants at the fusion site of the first and second branchial arches during the third to fourth fetal week. Some authors consider it as a variant of the thyroglossal duct cyst. Finally, previous surgical or accidental events may cause traumatic implantation of epithelial cells into deeper tissues which may give rise to an implantation dermoid ,11. Epidermoid cysts may be categorized as congenital or acquired based on their origin, although there is no disparity between the two either clinically or histologically. They may be found in any age group, but show preponderance between 15 and 35 years of age with male predilection ,13. In oClinically, epidermoid cysts are painless, slow growing lesion with doughy consistency, often soft and well encapsulated without any associated lymphadenopathy. They are usually not attached to any underlying structures and are completely free. They often remain asymptomatic for many years and grow painlessly. Usually attention is drawn when complications like masticatory or breathing difficulty arise as a result of a huge growth, as seen in this particular case. Large intraoral cysts usually present with dysphagia, dysphonia or dyspnea. Contents of the cyst can be keratinous, casseous, sebaceous or purulent . CliniciHistologically, dermoid/epidermoid cysts are lined by stratified squamous epithelium with few rete processes.Quite often, there is no granular cell layer and keratin from the surface of the epithelium can be seen to be sloughing into the cystic lumen, which is usually filled with degenerated and necrotic keratinaceous detritus. Areas of epithelial degeneration or ulceration may be seen, usually associated with a mild to moderately intense chronic inflammatory cell reaction. Inflammation may extend deeply into the sub-epithelial fibro-vascular stroma. In our case, the cystic lumen contained only keratin. No skin appendages in the cystic wall or any special structures like bone, muscle or odontogenic tissues were associated with it. Therefore, it was diagnosed as epidermoid cyst ,14,15.Endotracheal intubation in this kind of patient is also a challenging job. There is a high chance of rupture of the cystic lesion at the time of laryngoscopic examination, which may lead to aspiration of the cystic fluid. In addition, in our case, it was impossible to perform intubation in a regular laryngoscopic fashion as the epiglottis was pushed posteriorly; therefore, we had to perform fiber optic intubation which is a preferred procedure for such cases. The surgeon should keep the tracheostomy set ready to manage the airway in case of emergency to avoid catastrophe. The treatment of choice for epidermoid cysts is complete surgical excision. Recurrence is rare. Many surgical accesses have been described depending on the location and size of the lesion. Surgical approaches-such as transcutaneous, extended median glossotomy, median glossotomy and mid line incision, may be performed ,16. AlthTracheostomy was performed and the airway secured. The patient recovered well and successful weaning of tracheostomy was performed later on. Throughout literature, epidermoid cysts of such a large size or such an incident have never been mentioned. This highlights a probable risk of such a large swelling causing respiratory distress even after successful removal of cyst. A surgeon must remain cautious until successful extubation of the patient and be sure that the patent’s airway is clear.Epidermoid cyst is rare occurrence in the head and neck region and more so in the oral cavity.This diagnosis should be kept in mind when dealing with a large, painless, asymptomatic swelling in the sublingual region. Surgical excision is the treatment of choice for epidermoid cysts. All measures of securing the airway and tracheostomy must be kept ready while operating such a swelling, even after extubation.


Dear Sir,Lipoblastoma is an unusual benign soft tissue tumor composed of immature white fat. This tumor usually occurs in young children and involves extremities, head and neck, trunk and rarely mediastinum, mesentery and retroperitoneum.[1] Most patients present with asymptomatic gradually enlarging soft tissue mass. Tumours at certain locations may lead to pressure-compression symptoms to the adjacent organs.[1] Magnetic resonance imaging (MRI) is considered the method of choice of radiologic diagnosis of lipoblastoma with characteristic high signal intensity on T1-weighted images.[2] The cytological features of this rare tumor are described only in few reports in the available literature.[3-5]A six-year-old girl presented with a gradually enlarging painless lump in the right upper back since birth. Local examination revealed a 20 cm x 20 cm soft non-tender lump in the subcutaneous tissues of the back. MRI scans showed a large lobulated mass lesion with predominantly fat intensity on T1-weighted and T2-weighted images. Multiple septae were seen within the lesion. The right inferolateral aspect of the mass showed a small soft tissue component with minimal enhancement on gadolinium contrast . Fine needle aspiration (FNA) smears showed adipose tissue fragments composed of mature adipocytes as well as lipoblasts with multivacuolated cytoplasm, central indented normochromatic nucleus without nucleoli . A cytologic impression of lipoblastoma was rendered. The child underwent in-toto resection of the tumor. Cut-section of the mass showed a lobulated appearance with yellow and focal grey-white areas. Multiple sections showed a lobulated adipocytic tumor composed predominantly of mature adipocytes separated into lobules by fibrous septae. The septae showed stellate cells and occasional multivacuolated lipoblasts with small central nucleus. No nuclear atypia, mitosis or necrosis was seen . Histopathology was consistent with lipoblastoma. The child is doing well one year after surgery.There are only few reports of cytologic descriptions of lipoblastoma in the available literature.[3-7] Earlier authors have reported smears with uni- or multi-vacuolated cells with plexiform capillary pattern in a mucoid background. The proportion of lipoblasts and lipocytes vary considerably from case to case. In the index case, smears showed tissue fragments composed of mainly lipocytes with few lipoblasts and thin capillaries and a cytologic diagnosis of lipoblastoma could be made. The two main differential diagnoses in cytology of lipoblastoma are myxoid liposarcoma and lipoma. Other entities to be considered include fibrous hamartoma of infancy and other myxoid neoplasms. Myxoid liposarcoma may be indistinguishable from lipoblastoma. The patients' age and location of the tumor can help in this distinction to some extent, since most lipoblastomas occur before three years of age while liposarcomas, usually deep-seated, affect older children and adolescents.[3] Cytologically, myxoid liposarcoma is characterized by myxoid material, small oval cells and branching capillaries. Adipose fragments or lipoblastic differentiation is scarce or absent in most case.[4] The presence of a range of maturation of lipoblasts and lipocytes is characteristic of lipoblastoma on aspiration smears. Surgery is the standard treatment of lipoblastoma. A small risk of local recurrence exists in cases with incomplete resection.[3]In conclusion, FNA can serve as a useful diagnostic tool in pre-operative diagnosis of lipoblastoma. An accurate cytologic diagnosis can assist the surgeon in making a pre-operative decision.Source of Support: NilConflict of Interest: None declared


Far-out syndrome was reported by Wiltse et al. in 1984, which is a condition characterized by L5 spinal nerve radiculopathy due to nerve compression between the L5 transverse process and sacral alar. Although many cases of far-out syndrome have been reported, to our knowledge, the present case firstly showed far-out syndrome due to assimilated L4 hemivertebra and L5 vertebra through which abnormal nerve root passed.A 71-year-old man presented with left lower back pain and intermittent claudication accompanied by severe left buttock pain. Radiological examination showed assimilation between the L4 hemivertebra and L5 vertebra, which had two pedicles on the right side, with no canal stenosis. However, computed tomography and magnetic resonance imaging of coronal sections showed extraforaminal stenosis between the L5 transverse process and sacral alar, whereby the L5 spinal nerve was pinched (“far-out lesion”), and an abnormal nerve root passage in the assimilated vertebral corpus. We performed transforaminal lumbar interbody fusion, then resected the L5 transverse process to decompress the extraforaminal stenosis, and finally installed pedicle screws, but not at the one of pedicles of the assimilated vertebra in order to prevent nerve injury. Postoperatively, the patient had no symptoms up to 1.5 years after the surgery.The current case suggests the importance of detailed preoperative examination of patients with anatomical abnormalities such as assimilated vertebrae, which may result in incorrect diagnosis and failed surgery. Far-out syndrome, first reported by Wiltse et al. in 1984 [A 71-year-old man presented with an over 10-year history of left lower back pain and intermittent claudication accompanied by severe pain radiating to the left buttock. Physical examination confirmed hyperesthesia in the left hip but no apparent motor weakness. On both sides, the straight leg raising test yielded negative results. A plain X-ray scan showed L4–5 assimilated vertebra and junctional scoliosis. Flexion and extension views showed lumbosacral instability with 10° sagittal rotation Fig. . HoweverSince Wiltse’s first report of far-out syndrome, many cases with similar pathological condition have been reported –9. HowevWith regard to congenital vertebral deformities in the lumbosacral junction, previous reports have described some cases of pathology-associated lower back and leg pain. For example, Yoshioka et al. reported the case of a 39-year-old man with congenital absence of the L5–S1 facet joint and a conjoined nerve root who was treated by decompression and fixation surgery. This pathology was not detectable on plain X-ray scans but was observed in CT and MRI . SimilarGenerally, established surgical procedures such as MIS can be used to treat pathological lesions in normal anatomical structures, because the degenerative changes are predictable to some extent. However, in the case of congenital anatomical deformities, detailed preoperative imaging tests and well-planned elaborate surgeries are needed on a case-by-case basis.The current case was a unique one of far-out syndrome with anatomical abnormality due to assimilated vertebrae. At the first visit, the patient was suspected to have simple canal stenosis because of the intermittent claudication and the observation of assimilated vertebrae and regional scoliosis on plain X-ray scans. However, CT and MRI of coronal sections and myelography following CT showed no canal stenosis and instead indicated stenosis outside of the L5–S1 intervertebral foramen, that is, a “far-out lesion.” Finally, enhancement and infiltration of the L5 spinal nerve confirmed the final diagnosis. To treat the severe stenosis at the far-out lesion with the assimilated vertebrae, we decided to perform rigid intervertebral fusion with adequate bone resection, which completely relieved the patient’s pain. In addition, detailed preoperative CT and MRI investigations facilitated successfully screw insertion into one of the right L5 pedicles, which was located in an anatomically complex lesion.The current case suggests the importance of detailed preoperative examination of patients with anatomical abnormalities such as assimilated vertebrae, which sometimes result in incorrect diagnosis and failed surgery.We described the case of a patient with far-out syndrome associated with a uniquely deformed L5 vertebra involving an abnormal nerve root passage. CT and MRI examination of coronal sections were useful to diagnose this anatomical condition. After detailed preoperative imaging, we successfully performed TLIF surgery.


Desmoid tumors are rare, soft-tissue neoplasms that do not metastasize, but exhibit aggressive growth and local invasion. They originate most frequently from abdominal fascial structures, although they can also appear at extra-abdominal sites. The most common extra-abdominal locations include the shoulder, chest wall, back, thigh, and head and neck. In children, desmoid tumors are more infiltrative, having a tendency towards osseous involvement more frequently than in adult patients. We report acase of a supraspinatus muscle desmoid tumor in a female patient with clavicle destruction. Desmoid tumors (DTs) are benign, locally aggressive neoplasms that arise from connective tissue in muscle, fascia or aponeuroses . The incA 37-year-old woman presented with a pain in her left shoulder of six month's duration. She had no history of trauma or neoplasm. On physical examination, her shoulder range of motion was full, with intact motor and sensory functions in the upper limb; however, the lateral edge of the clavicle was tender with palpation. Radiographs of the left shoulder showed an osteolytic lesion located at distal 1/3 of the left clavicle . On MR iDesmoid tumors (DTs)are rare, soft-tissue neoplasms that do not metastasize, but exhibit aggressive growth and local invasion . They arFor DTs, which arise from soft tissue, MR imaging is the most appropriate imaging modality to assist with diagnosis and to evaluate the margins and extent of the disease . The appThis case illustrated the unusual features that can be associated with a soft-tissue desmoid tumor; namely, cortical disruption and medullary extension of the clavicle in an adult patient with a relatively small tumor.


Acute appendicitis is often simulated by other entities like mesenteric adenitis, worm infestation, Meckel’s diverticulitis, urinary tract infection and rarely omental torsion. We report two cases, a 6 year old boy and an 11 year old girl, who presented with symptoms and signs of acute appendicitis but upon exploration turned out to be omental torsion. Omental torsion is a rare cause of acute abdominal pain. It often mimics acute appendicitis preoperatively.[1-5] We present two such cases in which a pre-operative diagnosis of acute appendicitis turned out to be primary omental torsion at surgery.Case 1A 6-year-old boy presented with pain in right iliac fossa for one day. Pain was continuous in nature, severe in intensity with no aggravating or relieving factor and no shifting or radiation. On examination, a30 kg child, with pulse of 124 beats per minute and temperature of 99°F was lying on bed in agony. His abdomen had marked tenderness and guarding in the right iliac region. Leucocyte count was 15600 per mm³ and urinalysis was normal. Ultrasound abdomen showed minimal amount of fluid in the right iliac fossa. Provisional diagnosis of acute appendicitis was made; at exploration moderate amount of blood stained fluid was found in the peritoneal cavity. Appendix appeared normal. Further exploration revealed a congested omentum twisted around itself . Appendectomy and omentectomy of the affected omentum were done. Post-operative recovery was uneventful.Case 2An 11-year-old girl presented with right lower quadrant pain for 3 days. It was associated with low grade fever for one day. On examination, pulse was 80 beats per minute and temperature 99°F. There was mild tenderness present in the right lower quadrant. Leucocyte count was 8600 per mm³ with normal urinalysis report. Ultrasound abdomen was reported normal. The next morning, her pain abdomen worsened with one episode of bilious vomiting. Her abdomen became markedly tender with guarding in the right iliac fossa. Upon exploration, small amount of serosanguineous fluid was found in the peritoneal cavity. Appendix was found to be normal. Further exploration revealed a congested omentum twisted around itself. Appendectomy and omentectomy of the affected omentum were done. Post-operative recovery was uneventful.Omental torsion is rare in children and out of more than 300 reported casesonly 15% occurred in children.[6]Omental torsion can be primary or secondary. In the absence of any associated intra-abdominal pathology, if a segment of omentum rotates around its own axis, it is a primary lesion.[7] Primary Omental torsion accounts for one-third of the cases. The etiology remains unclear but several factors have been implicated in the pathogenesis, such as anatomical malformations of the omentum, local variations in omental fat distribution as in obesity, vascular abnormality of omentum, trauma, and hyperperistalsis due to heavy meals and increased intra-abdominal pressure due to exercise or coughing. Obesity is considered to be an important predisposing factor and is present in almost 70% of patients.[5]Secondary torsion is more common and occurs as a result of underlying abdominal pathology  resulting in distal fixation point.[4]Patients with omental torsion frequently presents with pain and tenderness in the right lower quadrant of abdomen, thus mimic acute appendicitis. It is very difficult to diagnose it preoperatively. Obesity, paucity of GI symptoms and relatively long duration of symptoms should raise the suspicion of primary omental torsion. Color doppler ultrasonography and computed tomography are useful diagnostic modalities. The presence of free serosanguineous fluid within the peritoneum cavity with normal appendix should raise the possibility of primary omental torsion. On CT scan a mass of fat density may be present showing whirling pattern of concentric streaks.[8]Management of omental torsion is relatively straight forward with resection of the involved omental segment. But some authors have suggested a conservative approach if the diagnosis of omental torsion is confirmed and other pathologies have been ruled out.[9]Surgical treatment results in a much faster recovery and pain alleviation. It also prevents possible sepsis and longer duration of hospital stay.[7]In conclusion, though very rare, omental torsion should be ruled out if normal appendix is found during surgery for acute appendicitis.Source of Support: NilConflict of Interest: None declared


Hepatoid adenocarcinoma (HAC) is a rare neoplasm with aberrant hepatocellular differentiation. HAC occurs in extrahepatic organs such as the gastrointestinal tract, testes, ovaries, and lungs and frequently produces alpha-fetoprotein. A 69-year-old patient was diagnosed clinically with T2aN0M0, stage IB, non-small cell lung carcinoma. Because the tumor showed tight adhesion to the chest wall, we performed left upper lobectomy, combined resection of the 3rd and 4th ribs, and lymph node dissection. Pathological examination confirmed the diagnosis of HAC of the lung , and four courses of cisplatin and gemcitabine were administered as adjuvant chemotherapy. Genetic analysis of the epidermal growth factor receptor showed wild type. Preoperative serum alpha-fetoprotein level, a useful marker of disease progression, was elevated to 4497 ng/ml, decreasing within the normal range by about 3 months postoperatively. The patient remains alive without recurrence as of 51 months after surgery. Hepatoid adenocarcinoma (HAC) is a rare neoplasm showing aberrant hepatocellular differentiation. HAC occurs in extrahepatic organs such as the gastrointestinal tract, testes, ovaries, and lungs and frequently produces AFP. HAC in any extrahepatic organs is reportedly associated with poor prognosis. We describe herein a case of pulmonary HAC treated by complete resection and adjuvant chemotherapy.In March 2011, chest X-ray detected abnormal shadows in the left lung of a 69-year-old man with a 40-pack-year history of smoking. In 2006, he underwent surgical resection of early tongue cancer, which was identified as squamous cell carcinoma. Chest computed tomography (CT) revealed a mass lesion in the left segments 1+2, with a diameter of 4.3 cm Fig. . The tumEGFR) was conducted using polymerase chain reaction assay, showing wild-type EGFR. Given the diagnosis of HAC, we examined preoperative serum AFP levels in stored blood samples. AFP was found to be elevated to 4497 ng/ml at 1 month preoperatively, decreasing within the normal range postoperatively. The patient received four courses of cisplatin and gemcitabine as adjuvant chemotherapy and remains alive without recurrence as of 51 months after surgery.The resected specimens showed hepatocellular features such as a pseudo-biliary tract pattern Fig. . ImmunohSome cases of AFP-producing lung carcinoma were reported in the 1980s. Ishikura et al. first reviewed seven cases of AFP-producing lung carcinoma in 1990 and diagnosed five of the seven cases with HAC , clarifyHAC is an extremely aggressive tumor with poor prognosis, and effective chemotherapies have yet to be established. On the other hand, survival for 9 years and for 37 months has been reported in patients with clinical stage IV HAC in the lung . Those pThe possibility of pulmonary metastasis from hepatocellular carcinoma was excluded for the lung tumor in this case, because no liver tumors were apparent on preoperative contrast-enhanced CT, FDG-PET, or postoperative ultrasonography. Metastasis from hepatocellular carcinoma of the stomach or other organs was also ruled out. HAC of the lung was therefore diagnosed.In this case, serum AFP levels decreased from 4497 ng/ml at 44 days preoperatively to 125.5 ng/ml at 25 days after surgery and within normal range by 82 days after surgery. From that time, serum AFP level has remained within normal limits and no recurrence has been identified. Serum AFP level could offer a useful marker of disease progression for HAC.In our case, EBUS-TBNA was useful for accurate staging. As the absence of lymph node metastases was confirmed by EBUS-TBNA, the patient was able to undergo surgery while the tumor was still in the early stage.In conclusion, we encountered a case of HAC in the lung, representing a rare neoplasm with aberrant hepatocellular differentiation. The patient underwent complete resection and adjuvant chemotherapy with CDDP+GEM and as of the time of writing has survived without recurrence for 51 months postoperatively. Although more cases are required, our findings suggest that complete resection and multimodal therapy can improve the prognosis for patients with HAC in the lungs.Written informed consent was obtained from the patient for publication of this case report and the accompanying images. A copy of the written consent is available for review by the editor in chief of this journal.