Ossifying fibromyxoid tumor (OFMT) is a soft tissue tumor of uncertain lineage that most often arises in the extremities of adults. Imaging findings of this uncommon tumor are rare. We, herein, present a case of OFMT occurring in the left thigh of a 36-year-old male. Radiological examinations revealed a well-circumscribed subcutaneous mass with an incomplete shell of peripheral ossification, suggesting a benign condition. Following complete excision, the mass was histopathologically diagnosed as an OFMT. The patient demonstrated no evidence of local recurrence within 11 months of follow-up. We describe the clinicopathological and radiological features, and review the relevant literature. OFMT pret al demonstret al. In thisA 36-year-old male presented with a 10-year history of a slow-growing, painless mass in the left proximal thigh. The patient’s medical history was non-contributory. Written informed consent for publication was obtained from the patient. Physical examination revealed a ∼6×5-cm superficial, firm, non-tender mass in the posterolateral aspect of the left proximal thigh . NeuroloPlain radiographs showed a soft tissue mass with amorphous calcification and extensive foci of ossification . ComputeGrossly, the tumor was well circumscribed and covered by a thin fibrous capsule. A cut section revealed that the tumor was gray-white, solid, multinodular and rubbery to firm in consistency . HistopaThe postoperative course was uneventful, and the patient was doing well with no local recurrence 11 months following surgery.et al study by Graham et al is frequently rearranged in OFMT, including atypical and malignant variants. Moreover, PHF1 was fused to EP400 (at 12q24) in one atypical case with the t translocation. OFMT is the second neoplasm to be identified, in addition to endometrial stromal tumor, in which PHF1 is involved in fusions with ectopic sequences. A FISH assay for PHF1 rearrangements would therefore be useful for the differential diagnosis of OFMT and its histopathological mimics (Only seven cases of OFMT have been cytogenetically described ,12–15. Cham et al revealedl mimics .In summary, we have described the imaging findings of a typical OFMT with clinical and histopathological correlation. Although rare, OFMT ought to be considered in the differential diagnosis of a well-circumscribed, slow-growing, painless, subcutaneous mass with irregular ossifications and/or calcifications.


Introduction. Hydatid disease is caused by the tapeworm Echinococcus granulosus and is still a matter of public health in many regions of the world, where it is an endemic parasitic disease. Although the liver is the most involved organ, hydatidosis can be found anywhere in the human body. Rare forms of location may lead to diagnostic and therapeutic dilemmas. Case Report. Herein we report a rare case of acute abdominal pain and progressively increasing abdominal distension due to abdominal and multiple splenic echinococcosis in a 72-year-old Caucasian male. We also provide a brief review of the literature. Conclusion. Although hydatid disease is found most often in the liver and lungs, rarely any organ of the body can be involved by this zoonosis. Though rare, the possibility of unusual location of echinococcosis must always be considered by the operating surgeon, when dealing with diffuse abdominal pain in endemic areas, because any misinterpretation may result in unfavorable outcomes. Taenia echinococcus, which lives in the gut of dog, wild canines, and carnivorous animals that represent the definitive hosts [Echinococcosis is a zoonosis that continues to be a major health problem in many countries. It is endemic in several countries in South America, the Middle and Far East, and around the Mediterranean Sea , 2. Hydave hosts . Humans ve hosts . To our 3, haemoglobin at 10.5 g/dL, c-reactive protein was at 148 mg/l, creatinine, electrolytes, and liver function tests were normal. The examination of urine was also normal. The abdominal plan X-ray was normal and an urgent abdominal CT was arranged. The CT revealed a huge hepatic hydatid cyst (measuring 14.5 cm) with daughter cysts in the left lobe of the liver and multiple splenic hydatidosis. Additionally, a large echinococcal cyst was revealed at the left hypochondrium attached to the anterior wall of the stomach causing a huge pressure  with no organ resection, if it is feasible. Surgical intervention is required for symptomatic and exceptionally large peritoneal cysts. The diagnosis of uncommon locations of echinococcosis can be very difficult and requires a high suspicion and a lot of awareness on the part of the surgeon. The most important factor in diagnosing hydatid disease in unusual locations is the awareness of its possibility. Hydatid disease should be considered in the differential diagnosis of acute abdomen in endemic areas.


Burkitt lymphoma has many forms of clinical presentations and, in children, it is usually discovered due to the presence of an abdominal mass. This rapidly growing tumor is highly malignant, aggressive, and may cause either indirect symptoms, due to pressure phenomena or direct involvement of the bowel lumen, leading to either intestinal obstruction or intussusception. We describe the case of a 4-year-old girl who exhibited an unusual presentation of ileocolic intussusception on a Burkitt lymphoma lesion of the ileum. Burkitt lymphoma represents 8-10% of all tumors in children under 15 years of age [5].  This type of lymphoma has three clinical forms. The endemic form, common in the African continent, develops due to a chromosomal translocation between chromosomes 8 and 14 and often presents as a rapidly growing tumor of the facial bones. The non-endemic form, observed most often in the United States, typically presents with an abdominal mass or ascites. This non-endemic form results from a chromosome 8 translocation involving the c-myc oncogene. The third form is specific to immunocompromised patients and frequently presents as diffuse lymphadenopathy [6]. Burkitt lymphoma has a high proliferation rate and the histological appearance of a “starry sky" because of the high rate of mitosis and spontaneous cell death [3]. Many pediatric patients with Burkitt lymphoma present with intussusception as a first clinical sign, a presentation that potentially leads to the disease detection at an earlier stage [7]. Intussusception caused by Burkitt lymphoma as a cause of acute abdomen is rare, with often misleading symptoms that make the diagnosis more difficult [8]. Children with Burkitt lymphoma have an excellent prognosis with contemporary treatment regardless of disease stage .Intussusception is one of the most common emergencies in children that require the attention of a pediatric surgeon. Especially in older children, in 10% of the cases, there is a pathological lesion  at the lead point of the intussusceptum [11]. Estimated incidence is 2-3 cases per 1000 live births with boys/girls sex ratio 3:2 [11]. Although 90% of intussusceptions occur in children between 3 months and 3 years of age, with a peak incidence between the fifth and the ninth month of life, intussusception has also been reported in utero, in neonates and in adults [9]. The classic clinical triad described for intussusception is made up of abdominal colic, “red currant jelly" stools and a palpable abdominal mass; in children older than 2-3 years the presentation of intussusception is subtler and the classic triad of symptoms may not be present [9], and combined with clinical observation, is usually sufficient to diagnose the intussusceptions [11]. Barium enema, CT and colonoscopy can also be helpful in diagnosis [12]. Ultrasonography is the most efficient examination for the diagnosis of intestinal intussusceptions with a reported accuracy of up to 100% [14]. Non-Hodgkin’s Lymphoma (NHL) represents the third most frequent cancer in childhood and usually is subdivided into 3 histological subtypes: (1) 65% B-cell NHL including both Burkitt lymphoma and diffuse large B-cell lymphoma; (2) 20% lymphoblastic and (3) 15% anaplastic large cell lymphoma [16]. Burkitt lymphoma represents 40%-50% of all NHL cases in childhood, and is more common in males than females [14]. Predisposing factors for lymphoma of the small intestine includes prior malabsorbtion syndromes, inflammatory bowel disease and immunodeficiency states [14]. All these risk factors were absent in our patient who were previously healthy.Primary gastrointestinal lymphoma represents 1%-4% of all gastrointestinal malignancies. Most cases in children involve the distal ileum or ileocecal region [18]. The rapidity of volumetric doubling of this neoplasm frequently leads to an acute abdomen presentation that may mimic other diseases  [12]. In the case we are presenting, the onset was slow, with the patient losing weight and, prior to admission, showing the following two of the three classic signs for intussusception: diffuse abdominal pain and bloody stools without any palpable abdominal mass. The clinical presentation of undiagnosed Burkitt lymphoma is non-specific, which makes this a difficult condition to diagnose. Abdominal pain is present in 80% of the cases of intussusception with Burkitt lymphoma, along with nausea, vomiting, constipation, diarrhea, fatigue or malaise [15]. Along with the assignment of specific treatment protocols of relatively short duration, event-free survival rates for children with B-cell NHL have dramatically increased within the last 20 years [15]. Surgical procedures are now needed for complete resection in limited disease, diagnostic biopsies, management of life-threatening local tumor effects and second-look operations [15]. Surgery may provide important prognostic information through definitive staging offers a chance for cure with or without adjuvant therapy and may prevent complications such as haemorrhage, obstruction or perforation [13]. In patients with proven localized abdominal disease total resection may be performed if is not mutilating (as hemicolectomy performed in the past). The open surgical approach is still the gold standard in most paediatric surgical facilities [11]. Patients with complete resection had a significantly better prognosis compared with patients with incomplete resection or biopsy [15]. The ideal treatment of gastrointestinal lymphoma must be individualized based on the type of disease and its location so that a multidisciplinary approach with surgery and chemotherapy can optimize the chance of cure [19], therapy courses include the following drugs: cyclophosphamide, methotrexate, cytarabine, iphosphamide, etoposide, vincristine, vindesine, adriamycin, doxorubicin, dexamethasone [15]. Burkitt lymphoma patients who present with intussusception require shorter-duration and less intense chemotherapy than patients diagnosed in other ways. In our patient, we performed a segmental resection of the terminal ileum with total resection of the tumour, 2 courses of chemotherapy with excellent outcome at 18 month after surgery.  Burkitt lymphoma is very sensible to chemotherapy [Burkitt lymphoma is a diagnostic entity that the pediatric surgeon should consider when faced with a child with an unspecific clinical presentation, who complains of diffuse abdominal pain, weight loss, and bloody stool (with a “red currant jelly" appearance). The suspicion of intestinal intussusception with a pathological lesion at the lead point of the intussusceptum necessitates surgery. Laparotomy is the gold standard in both diagnosis and treatment, ensuring the excision of the entire tumor with free margins. A multidisciplinary team with an oncologist assures efficient therapeutic management. Short- and long- term outcomes are generally favorable, as shown by data from a review of the literature and confirmed by our patients’ outcome.


Smooth muscle tumors of uncertain malignant potential represent a histologically heterogeneous group of uterine smooth muscle tumors that cannot be diagnosed as either benign or malignant. Smooth muscle tumors of uncertain malignant potential are usually clinically benign, but should be considered tumors of low malignant potential because they can occasionally recur or metastasize to distant sites.We report the case of a 62-year-old Mongol woman diagnosed with a retroperitoneal smooth muscle tumor of uncertain malignant potential and lung metastasis, with a history of prior hysterectomy. The case was initially misdiagnosed as retroperitoneal sarcoma, and our patient received chemotherapy. However, no interval change in the size of the retroperitoneal mass and metastatic lung nodules was seen over a period of at least five years. She underwent partial resection of the retroperitoneal mass for the purposes of debulking and establishing a histopathological diagnosis. The diagnosis of the retroperitoneal mass was then confirmed as a smooth muscle tumor of uncertain malignant potential.Smooth muscle tumors of uncertain malignant potential have an unpredictable clinical course, and relapses generally appear to occur after a long disease-free interval of up to several years. Therefore, patients diagnosed with smooth muscle tumors of uncertain malignant potential should receive long-term follow-up. Smooth muscle tumors are histologically categorized into leiomyomas and leiomyosarcomas, based on the combination of histological parameters such as mitotic activity, cytological atypia and coagulative tumor cell necrosis . Smooth A 62-year-old Mongol woman was referred to our hospital for further evaluation of an intra-abdominal mass seen on an abdominal computed tomography (CT) scan. She had visited her primary care physician for recently aggravated chronic constipation and intermittent abdominal discomfort. These symptoms had persisted for more than 20 years. Whenever these symptoms were exacerbated, she took medications such as painkillers and laxatives prescribed by several private hospitals. She had no other gastrointestinal symptoms, and there was no change in body weight. Additionally, there were no respiratory or cardiovascular symptoms.2 on days one to five) and etoposide (80 mg/m2 on days one to five). After completion of the planned chemotherapy, there were no significant interval changes in the size of the retroperitoneal mass. Additionally, she received six cycles of CYVADIC  chemotherapy. She was then lost to follow-up and had received no further treatment since then.Her medical history was significant for the following illnesses. In 1994, she attended hospital for intermittent lower abdominal pain and constipation; on this occasion she was found to have a large retroperitoneal mass on an abdominal CT scan. Additionally, a chest CT scan showed small, well defined nodular lesions in both peripheral lung fields. A sonography-guided fine needle aspiration biopsy of the retroperitoneal mass was performed. The cytological findings revealed an atypical spindle cell tumor, and differential diagnoses of malignant fibrous histiocytoma, leiomyosarcoma, fibrosarcoma and malignant schwannoma were considered. At that time, she was diagnosed with retroperitoneal sarcoma with lung metastases. She received six cycles of ifosfamide  level was mildly increased to 564 U/L. Other laboratory findings were within their normal ranges. Serum levels of tumor markers cancer antigen (CA) 125, CA 19-9, and carcinoembryonic antigen (CEA) were normal. An abdominal CT scan showed multiple large, well defined, highly enhanced masses in the retroperitoneum and pelvic cavity, and there were no significant changes in the sizes of the masses since November 2005  ,10. The STUMPs have an unpredictable clinical course. On the basis of our experience and review of the literature, relapses appear to occur generally after a long disease-free interval of up to several years. Therefore, in the patients with STUMPs, a long-term, close follow-up is required. The focus of future research should be on finding the markers based on better understanding of the molecular pathways leading to malignant transformation, which will allow us to predict the clinical behavior of these tumors of uncertain malignant potential.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.The authors declare that they have no competing interests.HSW drafted the manuscript. HSW and HGC collected clinical data and performed the literature review. KJL evaluated hematoxylin and eosin and immunostained slides. All authors read and approved the final manuscript.


Gallbladder torsion is a rare, but potentially lethal disease, in which early recognition is crucial.We describe the case of an 89-year-old Caucasian woman who presented with clinical symptoms suggestive of acute cholecystitis to our hospital. Radiological imaging confirmed our clinical diagnosis. At first we considered percutaneous gallbladder drainage because of her age and comorbidity, but instead performed laparoscopic cholecystectomy because of rapid clinical deterioration. During laparoscopy a necrotic gallbladder due to torsion of the gallbladder around the cystic duct was found.Because percutaneous drainage could lead to further deterioration in the case of gallbladder torsion, this rare condition should be considered before performing a percutaneous drainage of cholecystitis. Gallbladder torsion is a rare but potentially fatal condition only a few cases of which have been reported in the recent literature ,2. The m9/L, C-reactive protein of 48 mg/L and normal kidney and liver function tests. Abdominal ultrasonography and computed tomography (CT) scan showed a clearly enlarged gallbladder with a thickened wall of 7 mm , followed by vomiting (52.7%), a palpable mass in the upper right abdomen 32.6%) and fever (31.6%) [2.6% and Triple triads of clinical presentation have been proposed, consisting of symptoms , physical signs  and patients' characteristics  ,8,11. LaPreoperative diagnosis of gallbladder torsion is difficult, not in the least because it is a fairly unknown disease. Diagnosis was made before surgery in fewer than 10% of cases ,14. RadiAlthough diagnostic techniques are becoming more sensitive, gallbladder torsion is still mostly diagnosed during surgery. Prompt cholecystectomy is recommended and a laparoscopic approach can be safely used in most cases. The objective of laparoscopy should be decompression and detorsion, followed by cholecystectomy ,8,10. WhRegarding the triple triads of clinical presentation as described above the diagnosis of gallbladder torsion should have been considered in our patient: she had seven out of nine signs, only lacking early vomiting and absence of toxemia. In retrospect the findings of radiological imaging also hinted at gallbladder torsion: showing a horizontal gallbladder with signs of inflammation and fluid between the gallbladder and liver bed, both radiological signs often found in gallbladder torsion. Radiological imaging did not show free air within the gallbladder wall, a common radiological finding when the gallbladder is necrotic. If this had been present, this would have supported the diagnosis of gangrenous cholecystitis, and thus torsion.Gallbladder torsion is a rare disease, which is still difficult to diagnose preoperatively despite advances in diagnostic imaging. It mainly affects elderly women and the clinical presentation is often suggestive of acute cholecystitis. Percutaneous drainage, as is often opted for in this patient group, can result in progressive necrosis followed by biliary peritonitis and even death. Therefore, it is important to be familiar with the clinical signs of gallbladder torsion and to consider the diagnosis before opting for percutaneous drainage for acute cholecystitis.CT: computed tomography.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.The authors declare that they have no competing interests.JB interpreted patient data, collected previous published literature on the subject and contributed in writing the manuscript. DB was also a contributor in writing the manuscript, as well as providing expert revision. TVR performed the surgical procedure, provided the endoscopic images and contributed in writing the manuscript. All authors read and approved the final manuscript.Torsion of the gallbladder. Endoscopic recordings during inspection and laparoscopic removal of the gallbladder, showing a rotated gallbladder, with a gangrenous aspect of the gallbladder, the cystic duct and the cystic artery. The movie shows derotation of the gallbladder (0 to 23 s), followed by decompression (24 to 33 s), and cholecystectomy (34 to 42 s). Clipping of the hilum is not specifically shown, but two proximal and one distal clip are visible at 00:00:35.Click here for file


Early pubertal sign was present with Breast stage 2 and pubic hair stage 1. She did not have skin pigmentation and has no bone deformity or fracture. Investigations suggested peripheral precocious puberty with grossly elevated serum estradiol 2293 pmol/L with suppressed LH < 0.2 IU/L and FSH < 0.2 IU/L, which also failed to rise in the Gonadotrophin stimulation test. Bone age was advanced to 7 to 8 year old. Pelvic ultrasound showed a 3.6 cm cystic lesion in left ovary and the uterus size was prominent with endometrial echoes. Skeletal survey did not show bony lesion. The clinical diagnosis was peripheral precocious puberty from autonomous functioning ovary cyst.This girl presented with problem of vaginal bleeding at 6 year 2 month old. There was no medication history. Physical examination showed that she was tall with height 122.7 cm (97th percentile) and has not yet started menarche. Repeated ultrasound at 8 year 9 month old showed that the ovary cyst again resolved.The girl showed progression with recurrent vaginal bleeding and further breast development. To stop further vaginal bleeding and prevent later compromised height, Letrozole, an aromatase inhibitor, was commenced at dose of 1.25 mg daily. Breast size regressed and serum estradiol dropped to nadir of <73 pmol/L. The ovary cyst resolved 2 months after starting Letrozole but was again detected after stopped treatment at 7 years old. The girl went into true puberty around when she approached 9 year old with progression of breast development and growth spurt. The height at last follow up at 10- year 10 month old was 154 cm (97The case demonstrated that the potent, long-acting aromatase inhibitor Letrozole is effective in arresting sexual precocious puberty resulting from autonomous functioning ovary cyst. The girl goes into normal puberty and attains growth spurt. Further follow up is required to monitor normal menstruation pattern.


This report describes a complicated course of a 58-year-old patient with multicentric Barrett’s carcinoma within a long-segment of Barrett metaplasia. After abdominal-thoracic resection of the cancer, with incomplete removal of the long-segment metaplastic lesion, invasive carcinoma was diagnosed in the remnant Barrett’s segment. Endoscopic mucosal resection was done, but Barrett’s mucosa was left in situ again. Recurrent tumor growth was diagnosed only few months later. Finally, transthoracic complete resection on the remnant Barrett’s segment was performed. Thus, our case demonstrates impressively the appearance of multicentric adenocarcinomas in Barrett’s esophagus and underlines the necessity of resection of the complete Barrett mucosa. Barrett’s esophagus (BE) is an acquired disease of the esophagus. Normal distal stratified squamous epithelium is transformed into metaplastic intestinal type columnar epithelium. Since the 1970s, there has been a remarkable change in the epidemiology of esophageal malignancy . IngrediOur case report is that of a patient who developed recurrent esophageal adenocarcinoma during one year after partial esophagectomy for Barrett carcinoma with incomplete resection of the premalignant lesion.In January 2006, a 58-year-old male was admitted to our hospital with intraepithelial low grade neoplasia in the remnant Barrett esophagus. The patient’s height was 174 cm, the weight 86 kg. He was a non-smoker and non-alcohol-consumer. Previously the patient has been suffering from hypertension. Heart and renal function were normal.In February 2005, a moderate differentiated Barrett’s adenocarcinoma (G2) had been found, 3 cm in diameter. No angioinvasion was observed. The carcinoma was initially located in the distal esophagus within a 15 cm long-segment of specialized intestinal metaplasia, reaching 5 cm below the upper esophageal sphincter. No thorough endoscopic biopsy study was done prior to the initial operation. Therefore no information on other occult areas of dysplastic tissue was available at that time.An abdomino-thoracic procedure was performed in September 2005. The primary pathohistological examination revealed an early carcinoma with no lymph node involvement and complete resection, pT1 pN0(0/13) M0 R0. The resection margin was free of tumor or dysplastic areas. For the reconstruction the entire stomach was pulled up into the chest and the anastomosis was performed in the mid esophagus. The entire segment of intestinal metaplasia was not removed at this time.Due to persistent reflux symptoms follow-up endoscopy was performed in October 2005. Biopsies of a slightly elevated area just above the anastomosis within the remnant 3 cm Barrett’s mucosa showed high-grade intraepithelial neoplasia. Only this visible lesion was biopsied, no 4 quadrant biopsies were performed at this time. Endoscopic mucosectomy was done. Pathologically, this 0.9x0.7x0.5 cm superficial tissue showed Barrett’s mucosa, and centrally a well-differenciated adenocarcinoma (M1). Due to persistent reflux symptoms and need for Proton-Pump-Inhibitors the patient was referred to our hospital, a tertiary referral center for esophageal diseases.The patient was admitted in January 2006. On endoscopy, remaining Barrett’s tissue was found with remnant intraepithelial neoplasia in the area of the mucosectomy. Four-quadrant biopsies showed low-grade and high-grade intraepithelial neoplasia in the remaining Barrrett’s mucosa. Tumor marker CA 72-4 was elevated (8.8 U/ml). X-rays, using radiopaque material, showed a free esophageal passage. There were no strictures nearby the gastro-esophageal anastomosis.A re-thoracotomy was performed and the entire segment of intestinal metaplasia removed. In order to locate the proximal extent of the Barrett’s segment metal clips were placed endoscopically one day prior of the operation. The resected tissue, 6x6x0.8 cm in size, showed a well differentiated tubular adenocarcinoma, which was limited to the mucosa (rpT1m pN0 (0/8), R0, G1, diameter 2 mm) with adjacent areas of dysplastic tissue. Extended Barrett’s mucosa was found in the area of the original anastomosis. The clips were verified within the specimen.Since initially the entire stomach was placed in the thoracic cavity a gastric tube could be created through the thoracotomy approach with a high intrathoracic esophago-gastric anastomosis in the posterior mediastinal area was set up. The patient remained stable further on, and was discharged from our hospital on day 16 postoperatively.After initial diagnosis of Barrett’s adenocarcinoma, our patient’s esophagus was just partially removed, including the malignant lesions. Barrett’s mucosa above the resected area remained in situ. This procedure generally requires close follow-up, especially in a situation like described in this case where a premalignant lesion has been left in situ. Four-quadrant-biopsy therefore still represents the gold standard for surveillance of BE (Sensitivity 91%) .Since BE is a tissue of high heterogeneity, and four-quadrant-biopsies usually are taken in a mean distance of 2 cm, the risk of unrecognised malignancies through sampling errors is of notable importance . Even byRetrospectively it becomes obvious that in this patient there was a multicentric disease before the first operation since carcinoma and high-grade intraepithelial neoplasia was found within one month at two different locations within the Barrett’s segment. In face of the short amount of time till recurrence, it remains on suspicion, if the dysplastic areas might have been existent and escaped from diagnosis already in February 2005. Consequently, the benefit of local treatment strategies (e.g. mucosectomy) of malignancies of this entity remains highly debatable. A recurrence rate of high-grade intraepithelial neoplasia and early cancer up to 30% in endoscopically treated patients has been reported previously .Other published studies demonstrated multifocality of high-grade intraepithelial neoplasia and/or adenocarcinoma.,8 So theFor reconstruction after esophagectomy, the method of using the unmodified stomach for thoracic anastomosis with remnant inconspicuous esophagus is obsolete. Due to the lack of the lower esophageal sphincter, there is a high probability of continuous gastroesophageal reflux and—after a latency period—to develop a BE anew. The preferable technique is to create a gastric tube—by a transhiatal en-bloc esophagectomy and proximal gastrectomy . Once a Thus, our case underlines impressively the necessity of resection of all Barrett’s mucosa and creating a gastric tube to be pulled upwards in treatment of adenocarcinomas of the esophagus.


Benign cystic mesothelioma of the tunica vaginalis is a rare occurrence. It usually presents with painless gradual swelling in the scrotum. These types of benign mesotheliomas typically occur in the peritoneum and usually affect young to middle-aged patients. We present in this case an unusual case of benign cystic mesothelioma of the tunica vaginalis in a 77-year-old male patient. A 77-year-old man presented to the urology outpatient clinic with a right scrotal swelling; the patient did not report any symptoms such as pain or discomfort from the site of the swelling. However, the patient did express his concerns about the slow and gradual growth of the mass in his scrotum for months. The patient had no history of asbestos exposure or any surgical procedures.On examination he had a tense swelling in the right hemiscrotum, the clinical features of which were consistent with a hydrocele. The scrotal ultrasound scan reported a large 7-8 cm multiloculated hydrocele .Following a thorough review, the patient underwent surgery to excise the right scrotal mass. Surgery revealed a tense multicystic mass which was completely excised and sent for histopathological assessment. The histopathology review of the scrotal cyst did report a benign multicystic mesothelioma arising from the tunica vaginalis .Four months later the patient presented for post-operative followup, and on examination the right testis was hard and fixed in the hemiscrotum but the right hemiscrotum, returned to the normal size. He was asymptomatic and free of recurrence.Benign multicystic mesothelioma (BMM), also referred to as cystic mesothelioma or multilocular inclusion cyst, is an intermediate-grade neoplasm of the mesothelial cells. Most cases that have been described involve the abdominal cavity, although few cases involving the spermatic cord and tunica vaginalis have been reported , 2. All It is worth noting that BMM tends to recur locally, making it more aggressive than other benign tumors, which arise from mesothelial cells in the genital tract such as adenomatoid tumour; however, it is more benign than malignant peritoneal mesotheliomas . Unlike BMM more commonly occurs in the peritoneum and the patient population is usually young to middle-aged women . Men are There are no proved risk factors for cystic mesothelioma, but cysts are commonly found at sites of prior surgery or inflammation . BMMs arThe present case presents a unique case of this benign tumour in an elderly patient; the histology as well as the radiological features exhibits a similar pattern as the peritoneal BMM. Because of its rarity most urologists might not be familiar with its macroscopic and radiological features. However, especially with its distinct multicystic nature it is worth considering it as part of the differential diagnoses for a scrotal multicystic swelling.


Cutaneous horns are usually found on chronic sun-damaged skin. A cutaneous horn is a rare tumour, often conical, circumscribed, and composed of dead keratin usually derived from base keratinocytes. It occurs mainly in association with underlying benign, premalignant, and malignant cutaneous diseases. The commonest malignancy is squamous cell carcinoma. Thus, to accurately ascertain the nature of the condition at the base of the lesion and to rule out malignancy, an excision biopsy is indicated. Here, we report a case of cutaneous horn over the palmar aspect of the left middle finger in a 45-year-old man whose histopathology showed actinic keratosis. A cutaneous horn is a conical projection of hard hyperkeratotic excrescence of cohesive keratin projecting over skin. Although it grossly resembles an animal horn, it lacks a bony core. Cutaneous horns are found in the upper parts of the body, such as the face, neck, and shoulders, and are frequently related to actinic damage . CutaneoWe hereby report a case of a cutaneous horn at an unusual site, that is, the palmar aspect of the middle finger, with underlying histopathologic changes of actinic keratosis.A 45-year-old man presented with a single lesion over the palmar aspect of the middle phalanx of the left middle finger since 12 months prior. There was no history of trauma or surgery at that site in the past. No itching, pain, or bleeding was present. The patient was a farmer by occupation. No significant family history was present. On examination, a firm, horny, and curved growth of around 2 cm in length with a broad base was present over the palmar aspect of middle phalanx of the left middle finger with no erythema at the base . No lympHistopathology showed extensive hyperkeratosis and focal parakeratosis. The epidermis was thickened and showed irregular downward proliferation in dermis . A varyiCutaneous horns, or cornucutaneum, are benign, elongated, keratinous projections from the skin, ranging in size from a few millimetres to many centimetres and resembling a miniature horn. The base of the horn may be flat, nodular, or crateriform. All animal horns except those of rhinoceroses contain bone casts that are not seen in cutaneous horns in human beings, which are simply composed of compact keratin. Cutaneous horns may be considered a common entity in the Caucasian population; a study reports 643 patients over a ten-year period with 32 new patients annually . ClinicaClassical cutaneous horns have dysplastic epidermal changes similar to solar keratosis with no atypicality or loss of polarity.The pathogenesis of this abnormal formation of keratinised material has not been fully elucidated. It may be of clinical importance because the underlying condition may be a malignant lesion. It is difficult to define the underlying lesion, especially in superficial biopsies; therefore, deep biopsies or the total excision of small lesions is recommended . MalignaActinic keratosis is a premalignant condition. The lesion starts as a flat scaly lesion and later grows into a large wart-like area. It commonly ranges from between 2 and 6 mm in size, may be dark, light, tan, pink, red, or a combination of all these shades. It occurs on sun-exposed areas of the body such as face, ears, neck, scalp, chest, back of hands, forearms, and lips. Up to 20% of untreated actinic keratosis can progress to malignancy. Histologically there is thickened stratum corneum with scattered areas of parakeratosis, associated loss of the granular layer and thickening of the epidermis. The normal ordered maturation of the keratinocytes is disordered to varying degrees, there may be widening of the intracellular spaces, cytologic atypia, such as abnormally large nuclei. The underlying dermis often shows severe actinic elastosis and a mild chronic inflammatory infiltrate. Cutaneous horn that represents actinic keratosis demonstrates similar but more exaggerated changes with massive tiers of hyperkeratosis and parakeratosis.The unique feature of this case is that the cutaneous horn was located on the palmar aspect of the middle finger, which is an uncommon site. Only one such case has been reported at an unusual site over the right index finger tip, but it also did not show any underlying premalignant or malignant changes . It suggExcision biopsy of the lesion and histopathological examination to rule out malignancy is mandatory. As malignancies should be excised with appropriate margins and evaluated for metastasis, a careful physical examination of the lymph nodes draining the area of lesion is required . TreatmeThe authors have no conflicts of interest to declare.


Central nervous system is a very rare site of Kaposi’s sarcoma in acquired immunodeficiency syndrome. Kaposi’s sarcoma, a neoplasm of endothelial origin, occurs mainly in the skin, but can involve many tissues, especially in patients with a poor immunity. Combination antiretroviral therapy, highly active against human immunodeficiency virus type-1, has caused a dramatic reduction of cutaneous and visceral involvements. No report of central nervous system localization of Kaposi’s sarcoma is described since the introduction of combination antiretroviral therapy in the late 90’s.A 42 year-old Caucasian man affected by human immunodeficiency virus type-1 infection treated with combination antiretroviral therapy and showing relatively preserved immunity with low viral load presented gingival squamous cell carcinoma and visceral (lungs and lymph nodes) Kaposi’s sarcoma. Chemotherapy and radiotherapy were performed with improvement of both neoplasms. Afterwards, a magnetic resonance imaging showed focal lesions of the brain. Despite new chemotherapy and radiotherapy the patient died. Histology after autopsy revealed brain lesions due to Kaposi’s sarcoma with the detection of Human Herpesvirus 8 on tissue samples.This is the first report in the combination antiretroviral therapy era of a very rare complication of Kaposi’s sarcoma, such as that of brain localization, in a patient with a relatively good control of human immunodeficiency virus infection. Therefore, Kaposi’s sarcoma should be considered in differential diagnosis with other intracranial mass lesions that can occur in human immunodeficiency virus infected-patients focusing the issue of appropriate treatment for central nervous system involvement. Since the first appearance, acquired immunodeficiency syndrome (AIDS) and Kaposi’s sarcoma (KS), a multicentric angioproliferative spindle cell tumor of endothelial origin, were associated. KS can We here describe the case of a HIV-1-infected patient with KS involvement of the brain, focusing on histopathological, virological and radiological features.3 and HIV-1 RNA in plasma was 68 copies/mL.A 42 year-old Italian man diagnosed with HIV-1 infection since 1993, was admitted at our hospital in April 2011 complaining about an acute pain at the right zygoma. At medical examination he had a vegetated lesion of the gingiva, arising from the superior maxilla, and an enlargement of right supraclavear lymph nodes. Examination of the other organs was normal. CD4+ cell count was 243/mm3 and hepatitis C virus coinfection was documented. In recent years, only occasional bacterial pneumonia had occurred. cART was started in 1997 and changed to various regimens up to 2008, since when the current cART based on co-formulated tenofovir-emtricitabine, raltegravir, and darunavir/ritonavir had been prescribed. This salvage therapy was chosen after HIV genotypic resistance test that documented mutation associated with multidrug resistance. CD4+ cell count had stably risen above 200/mm3 since December 2008 and HIV-1 RNA level was constantly below 500 copies/mL since October 2009.At clinical history, cutaneous and pulmonary KS had been diagnosed in 1993 and at that time successfully treated with interferon alfa and chemotherapy. CD4+ cell count nadir was 52/mm2) were performed, and after only two cycles an improvement of CT findings was documented. Then, ten sessions of radiotherapy on maxillofacial site for carcinoma were performed .During hospitalization a total body computerized tomography (CT) showed multiple nodular lesions in both lungs, minimal right pneumothorax, pathological soft tissue in the right maxillar sinus, and enlargement of supraclavear lymph nodes. CT scan of the brain was normal. Biopsy specimen from gingival lesion revealed squamous cell carcinoma, while tissue taken from supraclavear lymph node showed histological features of KS. HHV8-DNA by polymerase chain reaction (PCR) was negative on blood. Four cycles of chemotherapy using liposomial doxorubicin  of the brain did not show an increased uptake in the brain. 18 F-2-Fluoro-2-deoxy-d-glucose (FDG) positron emission tomography (PET) of the neck, thorax and abdomen showed hyperperfused tissue in the right cervical lymph nodes, skeleton, right adrenal gland, lungs, right maxillar sinus, duodenum. On the 1st of December a cycle of paclitaxel (100 mg/m2) was administered, and then ten sessions of whole brain radiotherapy were performed . At the end of December a massive right spontaneous pneumothorax occurred and pleural drainage was necessary again. The patient died on January 6th, 2012.In October 2011, the patient showed bilateral spontaneous pneumothorax and was treated with chest drainage. After 25 days, he presented generalized seizures. A magnetic resonance imaging (MRI) of the brain showed three focal brain lesions, two that involved the right frontal lobe (20 and 14 mm) and one the right cerebellum hemisphere (16 mm). On FLAIR T2-weighted images the lesions of the right frontal lobe were hyperintense, with hypointense halo and surrounding edema. Diffusion-weighted imaging (DWI) showed restricted diffusion and low apparent diffusion coefficients (ADCs) in the lesions mm(2)/s). A new total body CT displayed a worsening of the previously described pulmonary lesions and the development of new intestinal lesions. A multi-disciplinary consultation excluded the possibility to carry out a cerebral biopsy, because of the lesion high risk of bleeding. CD4+ cell count was 142/mm6 cells, 7789 copies/106 cells and 2316 copies/106 cells respectively.A full autopsy, including neuropathological examination, was performed. Tissue samples were taken from all organs. KS was found in the lungs, the ampulla of Vater, the pancreas, the duodenum, the adrenal gland and the cerebrum. Macroscopic examination revealed hemorrhagic nodules, variegated in appearance, with cystic degeneration in the organs involved. Multiple bilateral areas of consolidation with cavitation and necrotizing nodular lesions were present in the lungs. Cavities were extended to the pleura. Coronal section of the brain showed multiple hemorrhagic nodules with swelling of the cerebrum without displacement of midline structures. Lesions were localized in the frontal lobes at the gray/white matter interface, near the falx and in the cerebellum. Microscopic appearance of the tumor nodules was composed of monomorphic spindle cells arranged in ill defined fascicles Figure A and sep3, HIV-1 RNA of 68 copies/mL, negative HHV8 PCR in the peripheral blood, and diagnosis of concurrent gingival carcinoma made the diagnosis of KS unlikely.This case report describes an HIV-1-infected patient presenting brain localization of KS, despite previously administered chemoterapy and cART treatment. Disease localization was documented by accurate histological, virological and radiological examination with MRI and DWI. At first evaluation of the patient, absence of cutaneous neoplastic lesions, immunological status with CD4+ cell count stably above 200/mmIn the pre-cART era, HIV-1-infected patients with KS typically had a low CD4 cell count and a high HIV viral load. Since the introduction of cART, the incidence of KS has decreased and survOur patient was cART-experienced, with genotypic multi-drug resistance in HIV. Despite the high-level resistance, re-optimized cART allowed to obtain stable low-level viremia in plasma close to 50 copies/mL cut-off. Biopsy of the lymph node revealed histologic KS and allowed starting chemotherapy with improvement of visceral lesions. At the onset of neurological symptoms, brain mass lesions at MRI were detected. Radiologic features of intracranial KS have only rarely been described; intracranial masses of the patient had a vascular component, and appeared as a inhomogeneous mass with hyperintense signal on T1- and on T2-weighted images, with surrounding edema, minimal mass effect and with faintly ring enhancement. The hyperintensity on T1-weighted images probably was due to abnormally increased blood vessels with slow intra-lesional flow. The low ADC value of KS lesions might be due to its histological architecture made of high tumor cellularity and relatively large vascular spaces. Although no studies have elucidated the direct relationship between blood flow velocity and vascular spaces size we hypothesise that blood velocity in KS lesions is low because of the large vascular spaces, and that this may determine the increased signal strengths and lower ADCs on DWI. FDG-PET of the body only showed the suspicion of neoplastic disease in various tissues, but FDG-PET of the brain unfortunately was not performed. Only autopsy specimens allowed obtaining the diagnosis of KS, that was confirmed by the detection of HHV8-DNA in the brain tissues. In HIV-infected patients, differential diagnosis of mass lesions is required. Mass algorithms in the pre-cART era were well defined, but in the cART era, when the epidemiology of opportunistic infections changed, are lacking. Brain biopsy, if feasible, is no longer mandatory for the diagnosis of mass lesions in HIV-patients, howeveret al. reported two cases at the beginning of the AIDS era[et al. described in 1997 a patient who had never taken cART[et al.[et al. in CNS diseases studies performed in the 80’s[et al. found one case of cerebral KS[et al. in a retrospective study on 450 consecutive autoptical AIDS cases between 1984 and 1999, only in the cohort of 1984–1992[Only few histologically proven cases of KS metastatic to the brain in HIV-1 patients have been described: up to now Gorin AIDS era, and ButT[et al. and one the 80’s. In a moebral KS, so did 984–1992. Most of3 are risk factors of poor evolution of KS[The cause of brain involvement in this patient despite an initial improvement of visceral lesions with the appropriate treatment, is diffon of KS, but theon of KS,24. In tThis case describes a rare complication of KS, never reported in the cART era, and raises a question about the diagnosis and the treatment suggesting that KS should be considered for the differential diagnosis with other intracranial mass lesions that can occur in HIV patients and focusing on the problem of appropriate treatment for CNS involvement.The case described was approved by an internal revision group at the Direction of Clinical Department.Written informed consent was obtained from the wife of the patient for publication of this case report and any accompanying images. A copy of the consent form is available for review by the Editor of this journal.The authors declare that they have no competing interests.FB: study concept, acquisition, analysis and interpretation of data, drafting and critical revision of the manuscript. AB, VS, CA, FDN, MRC: acquisition, analysis and interpretation of data, drafting of the manuscript. MLG, AA: study concept and design, drafting and critical revision of the manuscript. LA, SG: acquisition of data, critical revision of the manuscript. All the authors approved the final version of the manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2334/13/600/prepub


Introduction. Splenic hematomas usually occur after blunt abdominal trauma. Most of the subcapsular hematomas will be resolved and reabsorbed spontaneously. However in rare cases, some of them organize and form calcified splenic masses. Angiosarcoma is an uncommon primary tumor of the spleen. Splenic angiosarcoma behaves extremely aggressive and has poor prognosis. Case Presentation. We report a forty-nine-year-old white male with organized splenic hematoma due to traffic accident mimicking splenic angiosarcoma. Conclusion. Both angiosarcoma and splenic organized hematoma have nonspecific symptoms and clinical findings. Because of the risk of hemorrhage and rupture, fine-needle biopsy should not be preferred. In case of splenic masses, excision and spleen-conserving surgery or total splenectomy should be performed. The spleen is one of the most injured organs after blunt abdominal trauma , 2. Of tPrimary angiosarcoma of spleen is a rare malignancy –6. It isWe reported a case of splenic mass in a patient with previous blunt abdominal trauma that was reported as splenic angiosarcoma at magnetic resonance imaging. We planned laparotomy for a definitive diagnosis and also for treatment. The histopathological examination revealed it as splenic hematoma. A forty-nine-year-old male with bilateral hypocondrial and epigastric nonspecific abdominal pain, sometimes with nausea and vomiting, was admitted to our hospital. He had a medical history of hospitalization and medical treatment due to car accident 18 months ago. Clinical examination was unremarkable. His complete blood count and biochemical tests were between normal ranges. Abdominal ultrasound (USG) images revealed 8 mm stone with normal wall thickness in gallbladder and a heterogenic complex mass of 8 cm in diameter at the inferior part of the spleen. Then magnetic resonance imaging (MRI) was performed to the patient. It revealed the same findings as USG, an 8 mm single stone in gallbladder and 80 mm heterogenic hyperintense on T2 weight pulse sequence, and heterogenic hypointense on T1 weight pulse sequence mass in spleen. The mass showed heterogenic enhancement after contrast agent injection, and its margins were regular . The USGThe splenic masses are quite rare and may be benign, malign, granulomatous, and even inflammatory or traumatic pseudotumors . The mosDuring laparotomy, the macroscopic view of the mass may vary from nodular structure to enlarged mass with areas of hemorrhage and necrosis . The onlSometimes splenic angiosarcoma may be presented as splenic rupture (32%). This is usually fatal, and half of these cases can be diagnosed on postmortem examination , 6. The spleen is one of the most vulnerable organs after blunt abdominal trauma. Almost two-thirds of blunt abdominal traumas causing splenic injuries occur due to road traffic accident. Subcapsular hematomas are seen in 37% of splenic injuries , 2. FortSubcapsular hematomas presenting as splenic masses are usually asymptomatic. Sometimes nonspecific abdominal pain and palpable mass may be the symptoms. During clinical examination, physicians rarely can palpate the splenic hematomas –3. RadioOur patient's presenting symptom was the abdominal pain as seen in splenic angiosarcoma or splenic subcapsular hematoma. Physical examination findings were not diagnostic except suspicious left upper quadrant mass. Anemia can be seen in 80% of splenic angiosarcoma patients, but it is not a diagnostic criterion alone . In our Splenic masses have disadvantages in diagnosis because of infrequency and inadequate investigation with laboratory and radiologic imaging , 5. On tOne of the serious complications of splenectomy is overwhelming postsplenectomy infection (OPSI). It is a rare but very dangerous situation. Spleen is the largest organ of lymphatic system and is one of the sites of antibody production. After splenectomy, patients become immune suppressed. The mortality rate due to fulminant sepsis is 4.4% in children and 0.9% in adults. Vaccine immunoprophylaxis against pneumonia, influenza, and meningitis is essential for asplenic patients. Particularly, pneumococcal vaccine is recommended at least 2 weeks before the splenectomy procedure. The vaccine should be repeated for every 5–10 years. Also patient education against OPSI will be very useful [We recommend surgical removal and histopathological examination of the splenic masses, if there is suspicious about its nature. Blunt abdominal trauma history may suggest subcapsular hematoma in such cases. Spleen-conserving surgery in proper cases should be the appropriate choice. However, if there is doubt about the nature of the mass, splenectomy should be preferred.


Myeloid sarcoma is known to precede the development of acute myeloid leukemia (AML) and can be the only clinical manifestation. Gastrointestinal involvement by AML is rare with the commonest site being small intestine. Patients present with vague abdominal pain and/or obstruction. Prognosis is usually poor as most of them rapidly progress to AML. We report a case of 25-year-old man with complaints of abdominal pain and vomiting of one-year duration. OGD scopy revealed infiltration of lesser curvature of stomach. Subsequently patient came back within a week with signs and symptoms of acute intestinal obstruction for which an ileal resection was done. Although the histology of stomach biopsy and ileal segments showing similar features were thought to be non-Hodgkin's lymphoma, immunohistochemistry confirmed the diagnosis of myeloid sarcoma. Bone marrow investigations confirmed involvement by AML. Patient succumbed to the disease due to extensive involvement of AML. This case highlights the primary gastrointestinal manifestation of AML which can often prove to be a diagnostic difficulty clinically and histologically. Prompt diagnosis is essential to hasten the management. Myeloid sarcoma is a manifestation of acute myeloid leukemia (AML) with involvement of extramedullary sites, first described by Burns in 1811 . The comA 25-year-old man presented with complaints of vague epigastric pain and vomiting of one-year duration. Radiological investigations revealed diffuse wall thickening along the lesser curvature with multiple enlarged lymph nodes in peripancreatic, paraaortic, and mesenteric group. Upper GI endoscopy revealed thickening of entire stomach especially lesser curvature. Rest of the routine blood investigation was within normal limits. Biopsy obtained from the stomach showed diffuse infiltration of mucosa and submucosa by neoplastic monotonous medium-sized cells with pale nuclei having occasional prominent nucleoli and brisk mitotic activity. Thus suspecting lymphoid cell morphology and a diagnosis of non-Hodgkin's lymphoma involving the stomach were given to be confirmed by immunohistochemistry.Subsequently patient came back within a week with signs and symptoms of acute intestinal obstruction for which an emergency laparotomy was done and a segment of thickened ileum which had led to ileo-ileal intussusception was resected to relieve the obstruction. Grossly, a 4 cm long segment of ileum was received which showed mucosal thickening of 1.5 cm, and cut surface of the same showed diffuse white appearance.Histology from the thickened ileum showed similar neoplastic monotonous cells of suspected lymphoid morphology as observed in the stomach . ConsideSubsequently, a bone marrow aspirate and biopsy done showed a hypercellular marrow containing almost 85%–90% cells of blast morphology, thus confirming marrow involvement by acute leukemia . Flow cyMyeloid sarcoma (MS) can occur concomitantly or can precede AML in 2%–7% . It can Common sites of MS involvement are skin, lymph nodes, spine, and so forth. Gastrointestinal MS although rare has been described both in adults and pediatric age group , 7. KohnIn the absence of abnormal hematological parameters, diagnosis of MS on histopathological specimens is challenging and 47%–56% of patients may be diagnosed with conditions other than MS, and the most common misdiagnosis being non-Hodgkin's lymphoma (large cell type) as observed in the present case, too , 8. DuriTo conclude, myeloid sarcoma occurring at any site is the forerunner of AML and gastrointestinal MS involvement with most commonly to small intestine is a rare presentation due to its wide spectrum of clinical manifestations. As diagnosis of myeloid sarcoma in small intestine involvement is initially unrecognized, especially with the setting of normal-hematological parameters, suspicion in any mass with diffusely infiltrating tumour cells with right IHC profile is essential to hasten the appropriate management.


Primary angiosarcoma of the ovary (AS) is a rare entity with only 31 reported cases. The majority are pure angiosarcomas, the remainder are associated either with teratomas or conventional epithelial tumors. More than 50% of ovarian AS are disseminated at the time of diagnosis, the minority is detected in stage I. The prognosis of ovarian angiosarcoma in general is poor. Most reports refer to younger individuals, aged from 7 to 46 years, and only 2 case reports could be found for patients older than 64 years. Here we present a very unusual case of angiosarcoma in a 81-year-old patient. Primary angiosarcomas (AS) of the ovary are rare malignancies. Approximately 25% of them are associated with other neoplasms, e.g. mature cystic teratoma, -3, mucinThe 81-year-old patient was admitted to the hospital with abdominal pain and distension. Ultra-sonography and computed tomography revealed a huge cystic mass of the right ovary. There were no signs of distant tumor deposits. The patient underwent hysterectomy and adnexectomy on the right, the left adnexa had been taken out decades ago. Further exploration of the abdomen and all other clinical investigations were without pathological findings, six weeks after laparatomy the patient underwent chemotherapy with 4 cycles of doxyrubicin, and after an follow up of 5 months she is still alive, and there are no signs of recurrence.Macroscopically the tumor had a weight of 2122 grams and measured 30:18:12 cm. The peritoneal surface was inconspicuous. The cut surface showed a big central cyst with a diameter of 14 cm, containing hemorrhagic debris and some luminal projections. The cyst wall and its immediate surrounding consisted of yellowish fibrous tissue with some myxoid glistening changes and hemorrhagic areas, but no significant necrosis . HHV8, epithelial markers , and smooth muscle actin were negative. Fluorescent in situ hybridisation for SYT-SSX  was performed with LSI SYT Dual Colour Break Apart probe  and was negative. Based on these findings, the patient was diagnosed with primary angiosarcoma of the ovary, high grade.Ovarian angiosarcoma (AS) is with rare exceptions a disease of premenopausal woman. Only two patients have been reported in postmenopausal age ,18 and tOf 31 reported cases of ovarian angiosarcomas, 23 were pure lesions without coexisting benign or malignant epithelial components. In 5 reports, angiosarcoma was found to be associated with mature cystic teratoma, and in this context it was discussed, whether angiosarcoma is a sarcomatous teratoma, particularly those tumors occurring in younger women-6, and pThese examples show that the suspicion of ovarian AS opens a broad range of differential diagnostic considerations. The correct histopathological diagnosis, however, is of importance because prognosis of ovarian AS is uniformly poor. This could be related to the fact that most patients are diagnosed with advanced disease in stage III or IV. Although patients with stage I disease were shown to survive and even become pregnant ,20, therWritten 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.The authors declare that they have no competing interests.HB conceived the case report and drafted the manuscript, CG and SP carried out histology and immunohistochemistry, DW supplied clinical data, GW carried out molecular analyses, SH supervised the case report and participated in its design and coordination. All authors have read and approved the final manuscript.


Malignant fibrous histiocytoma (MFH) is a common sarcoma affecting soft tissues of the body, especially of the extremities or trunk. Prognosis of the abdominal MFH is usually poor.A 52-year-old female presented to our surgical outpatient clinic with a lower abdominal tumor that had been gradually increasing in size. Clinical examination revealed a firm, irregularly surfaced, fixed, painless, child-head-sized tumor located in her lower abdomen. Computed tomography (CT) and magnetic resonance imaging (MRI) of the abdomen revealed a polycystic tumor at the lower abdomen which was 15 × 13 × 11 cm in diameter and encased the colorectum to the left back side. A barium enema and a colonoscopy showed direct invasion to the rectum. In 2001, the tumor had been excised along with a low anterior resection of the rectum because of direct invasion. The origin of this tumor was the mesorectum. The weight of the excised tumor was 1,500 g, including 800 ml of a brown fluid. A histopathological diagnosis revealed a common type of MFH, in which mitotic figures are frequently seen.This patient has survived without recurrence, for approximately 8 years since the completed tumor resection. It is important to obtain a complete resection during the MFH treatment. Malignant fibrous histiocytoma (MFH) is a common sarcoma affecting soft tissues of the body, especially of the extremities or trunk -3. The tPrimary mesenteric MFH is a rare disease and few cases have been reported in the English literature -10. We h3, hemoglobin of 8.3 g/dl, hematocrit of 25.4%, platelets count of 429,000/mm3, normal electrolytes, as well as normal blood urea nitrogen levels and the liver function.A 52-year-old female presented to our surgical outpatient clinic with a lower abdominal tumor that had been gradually increasing in size. She first noticed a fist sized, painless tumor about four months ago. Clinical examination revealed a firm, irregularly surfaced, fixed, painless, child-head-sized tumor in her lower abdomen. Laboratory data showed that she had a white blood cell count of 5500/mmComputed tomography (CT) of the abdomen demonstrated a large tumor in the lower abdomen which was 15 × 13 × 11 cm in diameter and encased the colorectum to the left back side Figure . MagnetiIn 2001, this tumor was excised along with a low anterior resection of the rectum for the complete resection Figure . The mesThe postoperative course was uneventful, and she left the hospital on the 15th postoperative day. A postoperative follow-up CT revealed no recurrence of the MFH in any organs since the resection was completed.Retroperioneal and mesenteric MFH have been reported to occur in the rate from 5.7% to 16% of the total MFHs cases ,5,11. SeMFH is an aggressive tumor with a high potential of metastasis to other parts of the body. The rates of local recurrence and distant metastasis are 44% and 42%, respectively . The mosMesenteric MFH initially grows without any significant clinical symptoms, and finally produces clinical symptoms and signs later when it compresses or invades the adjacent organs . In thisThe efficacy of chemotherapy against advanced or metastatic MFH is still unclear. On the other hand, several phase studies which investigate the efficacy of chemotherapy against advanced or metastatic soft tissue sarcoma including MFH, have been reported -16. ThesThis patient has survived without recurrence for approximately 8 years after the completed tumor resection. A complete resection is important to obtain a favorable prognosis for MFH.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.NY, MN, TT and YK contributed equally to this work; NY designed the research; NY, MN and TT performed and analyzed the data; NY and YK wrote the paper.


Intracranial osteomas are uncommon lesions that usually arise from the inner table of the cranium. There are few reports in the literature of intracranial osteomas with meninges attachment and without direct relation with the skull bone; these osteomas were mostly attached with dura. We report a rare osteoma with falx attachment.A 64-year-old woman presented with a 3-month history of intermittent tinnitus and dizziness. The scout film of petrous bone computed tomography scan revealed a high-density lesion in the frontal area. Magnetic resonance imaging showed a 2.5-cm mass attached to the surface of the falx in the right frontal parasagittal area. The patient underwent right frontal craniotomy, and a bony hard mass was found located in the right frontal parasagittal region extra-axially, with its medial surface attached to the falx. It could not be broken down by the cavitron ultrasonic surgical aspirator or even the cutting loop and was detached from the falx and removed in one piece. Histopathological examination showed a nodule with bony trabeculae and bone marrow tissue, compatible with osteoma. The postoperative course was uneventful, and the patient was discharged from the hospital with no neurological deficits one week after operation.This is the first case report in the English literature of an intracranial osteoma arising from the falx. Because of their slow growth and their locations in silent brain areas, intracranial osteomas are usually diagnosed incidentally. Surgical resection is the primary treatment choice. Osteomas are benign neoplasms consisting of mature normal osseous tissue. They commonly arise from the long bones of the extremities. In the head and neck region, they are usually found in the sinuses, facial bones, skull, and mandible -9. IntraA 64-year-old woman presented with a 3-month history of intermittent bilateral tinnitus, occasionally accompanied by dizziness. She had no history of major head injury or systemic infection. She visited an otology clinic where a temporal bone computed tomography (CT) scan of the petrous bone, semicircular canal, and hearing apparatus showed neither temporal bone lesions nor associated lesions in the ear; however, the scout film of the CT scan incidentally revealed a radiopaque nodule in the frontal area Figure . MagnetiPathologic examination revealed lamellated bony trabeculae lined by osteoblasts, and the intertrabecular marrow spaces were occupied by loose fibrovascular tissue Figure .The postoperative course was uneventful. The patient was discharged from the hospital with no neurological deficits after suture removal. During six months of post-operative follow-up, the frequency and degree of dizziness and tinnitus decreased progressively.Osteomas are recognized as benign bone neoplasms of uncertain etiology. In the head, osteomas often arise from the periosteum of the frontal or ethmoid sinuses and the mandible -9. Less On the basis of their natural history, intracranial osteomas are more benign than osteomas of the frontal sinuses . The tumThe pathogenesis of osteomas without bone involvement is still unknown. A dural osteoma can be confused with “meningeal ossification or meningeal calcification” originating from the dura or the falx because of the similarities in CT and MRI examinations and microscopic appearance . This neThe factors leading to the genesis of intracranial meningeal osteomas are as yet unknown. New bone formation from the dura or falx has been postulated as one of the causes because the meninges may function as the periosteum of the inner table of the skull ,12,15.In our patient, the operative findings confirmed an intracranial bony hard mass arising from the falx. This intradural osteoma might have been the result of a focal falx osteogenic activity. To the best of our knowledge, this is the first report of an intracranial osteoma that originated from the falx in an otherwise normal patient.The patient consented to the study and publication of this case report and images. A written consent is available for review.CT: Computed tomography; MRI: Magnetic resonance imaging.The authors declare that they have no competing interests.SMC participated in the preparation of the manuscript, literature search and drafted the manuscript. CCC participated in its design and coordination. CHT accomplished and analysed the CT scans and MRI. SMJ carried out the tissue preparation, (immuno)histology and edited the manuscript for its scientific content. TNL was responsible for the operations, follow-up of the patients and data preparation and conceived of the study. All authors read and approved the final manuscript.


Thyroglossal cysts are one of the most common midline neck masses. They usually present as midline painless cystic neck mass in the first three decades of life. These anomalies are very rare in elderly patients and may pose difficult diagnostic and therapeutic challenges. Here, we report a case of giant thyroglossal cyst in a 72-year-female patient who presented with stridor, hoarseness of voice, and vocal cord paresis with gross distortion of normal airway anatomy secondary to pressure effect of the mass. The gross distortion and displacement of airway along with respiratory distress in this patient posed a difficult situation in securing the airway. The airway was secured by a unique way of orotracheal intubation with the help of a ventilating airway exchange catheter. The cyst was excised in toto under general anaesthesia. The stridor completely resolved after surgery and tracheostomy was avoided. Thyroglossal cysts are one of the most common nonneoplastic neck masses in the paediatric population . ThyroglA 72-year-old female patient presented to the emergency otolaryngology services with a huge anterior neck swelling, hoarseness of voice, and stridor. The swelling started as a painless lump in the upper part of neck which gradually increased in size over 5 years. The patient developed progressive difficulty in breathing and hoarseness of voice of one-month duration and noisy breathing (stridor) of 4-days-duration which brought her to the emergency department. There was no history of recent rapid increase in size of the swelling. The patient did not give history suggestive of any neurological illness, prior surgery, or trauma.On examination, the patient had severe stridor and hoarseness of voice which worsened in supine position. The pulse rate was 110 per min and regular in rhythm. The blood pressure was 110/80 mm of Hg. The respiratory rate was 42 per minute. Neck examination revealed a 20 cm × 18 cm mass in the anterior neck more on the left side extending from the level of hyoid superiorly to the level of the suprasternal notch inferiorly . The masUltrasound revealed a 14 cm cystic mass which was hypoechoic with homogenous echogenicity compressing the airway. The right carotid was found to be pushed laterally. Contrast enhanced computed tomography (CECT) showed a homogenous mass of size 14 × 10 cm on the left side extending from the hyoid to the manubrium sterni compressing the tracheal lumen to a longitudinal slit. The cyst was intimately related to the hyoid bone. There was distortion of the laryngeal framework with localized remodelling of hyoid bone Figures . BaselinAs the airway anatomy was distorted, difficult intubation was anticipated. The airway was secured in the awake patient, with endoscopic guidance by railroading the endotracheal tube over number 14.0fr/65 cm ventilating airway exchange catheter . Intraoperatively approximately 14 cm × 10 cm cystic mass was seen compressing the left lobe of thyroid gland. The left recurrent laryngeal nerve was found to be displaced from the tracheo-esophageal groove and stretched beneath the mass.The left lamina of thyroid cartilage, the cricoid cartilage, and the tracheal rings were distorted and compressed by the mass. The cyst was partially decompressed by needle aspiration which helped to identify the tissue planes and the entire cyst was removed completely after identifying the recurrent laryngeal nerve on both sides. The patient was extubated after surgery without any need for tracheostomy. Histopathological examination revealed a cyst with squamous epithelial lining with underlying thyroid tissue. There was no evidence of malignant foci within the cyst. The findings were consistent with a benign thyroglossal cyst. The cyst fluid was sterile with plenty of exfoliated epithelial cells and negative for malignant cytology. The postoperative period was uneventful. Post-op thyroid function tests revealed an euthyroid status. The stridor resolved completely immediately after surgery but hoarseness persisted. The patient was discharged on the 10th post-op day. No recurrence was found in the patient on followup three months after surgery.In this case report we want to highlight the atypical presentation with acute airway compromise in an elderly patient with thyroglossal cyst and the novel approach of securing the distorted airway by avoiding tracheostomy and traumatic intubation. This report is intended to communicate our experience in managing a case of thyroglossal cyst in a 72-year-old lady which has reached giant proportions (14 cm × 10 cm) and presented with severe stridor, hoarseness of voice, gross distortion of airway anatomy, and vocal cord paralysis secondary to mass effect of the cyst. To our knowledge there has been no report of thyroglossal cysts presenting with acute airway compromise and vocal cord palsy in the elderly patients in the English literature [The recommended treatment of thyroglossal cyst in the elderly population varies and includes medical therapy, no intervention, and surgical treatment . A classThyroglossal cyst should be considered in the differential diagnosis of a large neck mass presenting with stridor and cord palsy in the elderly. In case of gross distortion of airway, as in this case, traumatic intubation and tracheostomy can be avoided by using a ventilatory airway exchange catheter. Controlled decompression of the cyst by intraoperative aspiration helps in identifying and preserving vital structures like the recurrent laryngeal nerve.


Hemangioblastomas are rare, benign tumors occurring in any part of the nervous system. Most are found as sporadic tumors in the cerebellum or spinal cord. However, these neoplasms are also associated with von Hippel-Lindau disease. We report a rare case of a sporadic sellar hemangioblastoma that became symptomatic due to pituitary apoplexy.An 80-year-old, otherwise healthy Caucasian woman presented to our facility with severe headache attacks, hypocortisolism and blurred vision. A magnetic resonance imaging scan showed an acute hemorrhage of a known, stable and asymptomatic sellar mass lesion with chiasmatic compression accounting for our patient's acute visual impairment. The tumor was resected by a transnasal, transsphenoidal approach and histological examination revealed a capillary hemangioblastoma . Our patient recovered well and substitutional therapy was started for panhypopituitarism. A follow-up magnetic resonance imaging scan performed 16 months postoperatively showed good chiasmatic decompression with no tumor recurrence.A review of the literature confirmed supratentorial locations of hemangioblastomas to be very unusual, especially within the sellar region. However, intrasellar hemangioblastoma must be considered in the differential diagnosis of pituitary apoplexy. Hemangioblastomas (HBLs) are benign, slowly growing and highly vascular tumors of the central nervous system (CNS), accounting for just 1% to 2.5% of all intracranial neoplasms, and 7% to 12% of primary tumors located in the posterior fossa . SporadiWe report here what is, to the best of our knowledge, the seventh sporadic case in the literature of sellar HBL, which presented with pituitary apoplexy. We also review the literature on cases of HBL within the sellar and suprasellar region.An 80-year-old Caucasian woman was admitted to our hospital with a 12-year history of an endocrine inactive steady sellar mass lesion  scan were suggestive of an acute hemorrhage of the sellar process, consistent with pituitary apoplexy Figure . Except Due to these clinical and radiological findings, the decision was made to surgically remove the tumor. A gross total extirpation using a transnasal, transsphenoidal approach to the pituitary mass was successfully performed. Intraoperatively, the tumor appeared yellowish-brown, was relatively firm and was located within a sellar hematoma cavity, which was evacuated.Postoperatively, our patient's visual field deficits improved markedly on clinical examination and Goldman perimetry confirmed a partial recovery of her bitemporal visual field deficits. Endocrinological studies showed panhypopituitarism with partial and transient diabetes insipidus. Our patient received substitution therapy with hydrocortisone, levothyroxine and transient therapy with desmopressin. Overall, our patient remained in good health with a satisfactory level of performance. A repeat MRI scan taken 16 months after surgery showed good chiasmatic decompression with no residual tumor mass Figure .The resected tumor was examined with light microscopy, which revealed a small, well circumscribed, non-adenomatous tumor surrounded by slightly compressed remnants of adenohypophyseal parenchyma Figure . The tumGiven the above findings, we identified the tumor as an intrapituitary example of capillary hemangioblastoma . Since our patient displayed no clinical stigmata of VHL disease, genetic testing was not performed.Based on previous studies, the occurrence of supratentorial HBLs is thought to be in the range of 2% to 8% of all HBLs ,4,6, accNo more than 27 reported cases to date (including our patient's case) describe HBLs originating in the sellar and suprasellar region (see  and refeWhile information on clinical features is derived from reports of sellar and suprasellar HBLs causing symptoms generally related to mass effect, a long presymptomatic stage can be assumed. Of a total of 250 patients with VHL disease enrolled in a prospective study, eight incidentally discovered HBLs located in the pituitary stalk remained stable during a mean follow-up of 41.4 ± 14 months . Also, iOverall, the unexpected nature and the unspecific presentation render an accurate preoperative diagnosis of sporadic HBLs challenging. In our patient, the apoplexy of a well known sellar mass suggested a pituitary macroadenoma; clinical apoplexy was observed in 0.6% to 9.0% of these cases . The typThe main histological differential diagnosis of HBL, irrespective of location, is metastatic clear cell carcinoma. In our patient, lack of immunoreactivity for cytokeratins along with a negligibly low proliferation index allowed for this alternative to be confidently ruled out. In the peculiar context of intrapituitary occurrence, we also addressed the possibility of xanthomatous hypophysitis and Langerhans cell histiocytosis ,14. The Supratentorial HBLs are rare, especially within the sellar region and without an association with VHL disease. However, our patient's case shows that intrasellar HBL must be considered in the differential diagnosis of pituitary apoplexy.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.The authors declare that they have no competing interests.RTS was responsible for the conception and drafting of the manuscript, and analyzed and reviewed the literature relevant to this case report. IV performed the histological examination and was a major contributor to writing the manuscript. RS was largely involved in patient management and also contributed to writing the article. RWS performed the operative resection of the tumor and critically revised the article. All authors read and approved the final manuscript.


Internal intestinal hernia has been defined as bulging of the intestines through a normal or an abnormal peritoneal or mesenteric opening. Paraduodenal hernias comprise 30%–53% of all internal intestinal herniations and account for 0.2%–0.9% of all bowel obstructions. In this paper, we aimed to present a male patient of 42 years of age who developed intestinal obstruction due to left paraduodenal hernias. Left paraduodenal hernia should be taken into consideration in the differential diagnosis in patients of relatively younger ages with no prior abdominal surgery who frequently have bowel obstruction episodes. Internal intestinal hernia has been defined as bulging of the intestines through a normal or an abnormal peritoneal or mesenteric opening. This clinical entity can occur for congenital or acquired reasons and accounts for around 1%–5.8% of all intestinal obstructions . Althoug3. We were not able to obtain ultrasonographic or contrast-enhanced abdominal CT evaluation because of the fact that the patient was admitted to our emergency department at 11.00 pm. Given the physical examination findings, plain X-ray imaging and the absence of any prior surgery in the case history, the patient was taken into surgery considering that widespread peritonitis was likely to exist due to a possible congenital brid or a perforated appendicitis with delayed diagnosis. We observed, in the surgical exploration via midline incision, that all of the small intestinal segments except a distal ileal portion of 50–60 cm length was stuck in the sac of a left paraduedonal hernia. The inferior mesenteric vein constituted the anterior portion of the hernial sac from the orifice (A 42-year-old male patient was admitted to our emergency department with the complaints of nausea, vomiting, and abdominal pain that had lasted for 3 days and had shown progressive worsening. The patient especially expressed that the pain had intensified around the umbilical region and that he had been unable to pass stool or gas. The patient's history revealed no systemic disease or prior operation. As for the vital signs of the patient, the blood pressure, the pulse rate, and the body temperature were measured to be 130/85 mmHg, 83 beats/min and 37.3°C, respectively. Marked abdominal distension and rebound tenderness were detected in the physical examination performed, whereas no abdominal guarding was observed. In standing abdominal direct X-ray, widespread air-fluid levels were observed. While no pathological finding was detected other than mild uremia in the blood chemistry, leukocyte counts was reported to be 16.450/mm orifice . After rIntestinal hernias are entitled according to the orifices they pass through or to the position of the herniating organ. Based on the former definition, the most commonly seen hernia types are as follows: paraduedonal, transmesenteric, pericecal, intersigmoid, supravesical, foramen Winslow, retroanastomotic, and omental hernias. Paraduedonal hernia was first defined by Treitz in 1857. Paraduedonal hernia is the most common form among intestinal hernias, accounting for 30%–53% of all cases. Paraduedonal hernias result from abnormal rotation of the midgut during embryonic development and can be divided into two subtypes, left and right paraduedonal hernias, according to their distinct pathogenesis and the resultant anatomical derangement. 75% of them are located on the left side in the Landzert's paraduedonal fossa [In conclusion, left paraduedonal hernia is one of the rare causes of small bowel obstruction. It should be taken into consideration in the differential diagnosis in patients of relatively younger ages with no prior abdominal surgery who frequently have bowel obstruction episodes.


Pulmonary carcinoids associated with ectopic adrenocorticotropic hormone secretion have a good prognosis if histological examination shows typical pulmonary carcinoid and low proliferation, whereas a poor outcome is linked to atypical pulmonary carcinoid and high proliferation. Here we describe the diagnostic challenges to find the tumor in Cushing’s syndrome secondary to ectopic adrenocorticotropic hormone secretion in two cases with an atypical and a typical pulmonary carcinoid, respectively.111Indium-pentetreoide somatostatin receptor scintigraphy could not visualize any ectopic tumor. The patient was referred for an 11C-5-hydroxytryptophan positron emission tomography, and a small 8mm nodule in her left lung was found. The tumor was removed via a lateral thoracic incision and wedge excision. The histological examination showed an atypical carcinoid with Ki-67 index of 9 to 10%, and an additional lobectomy was performed.A 63-year-old Caucasian woman presented with aggressive clinical features related to Cushing’s syndrome, having very high levels of urinary cortisol and circulating adrenocorticotropic hormone and cortisol. Magnetic resonance imaging showed no pituitary tumor, and bilateral inferior petrosal sinus sampling revealed no central peripheral ratio of adrenocorticotropic hormone. Computed tomography and The second patient, a 22-year-old Caucasian man, also presented with aggressive Cushing’s syndrome, with very high urinary cortisol levels and increased circulating cortisol as well as adrenocorticotropic hormone levels. A magnetic resonance imaging scan of the pituitary showed no tumor, whereas a 12×9×14mm tumor was detected in the right lung on the primary computed tomography scan and no further investigation was performed. The tumor was removed via a lateral thoracic incision and wedge excision. A typical carcinoid with Ki-67 index of 1 to 2% was found and no further surgery was performed.After surgical removal, the biochemical disturbances resolved and significant clinical improvement were achieved in both patients after 24 months of follow up.11C-5-hydroxytryptophan positron emission tomography could be considered early as a secondary diagnostic tool when primary computed tomography and/or magnetic resonance imaging scans fail to show any tumor.Diagnostic evaluation time is limited due to the aggressive course in ectopic adrenocorticotropic hormone-dependent Cushing’s syndrome. We suggest that  In caide-SRS) or technide-SRS). Pacak e and MRI, whereas and SRS.11C-5-HTP-PET and thoracic CT scan, respectively. The localization techniques and prognostic factors of pulmonary carcinoids are further discussed.Here, we describe two cases with ACTH-producing pulmonary carcinoid tumor diagnosed preoperatively with 111In-pentetreotide-SRS was performed in the whole body planar after an injection of 166 MBq 111Indium-labeled octreotide. The scan demonstrated no uptake typical for an ectopic ACTH source.A 63-year-old Caucasian woman was referred from a county hospital with an initial diagnosis of CS. She presented with generalized fatigue, muscle weakness, insomnia, flushing, hypertension, stress symptoms and psychiatric symptoms . A physical examination revealed the typical clinical features of a severe CS: hypertension, changed body constitution, moon face, supraclavicular fat, buffalo hump, hirsutism on her chin, proximal myopathy, but also a weight loss (5kg over a few months). Her previous medical history included breast cancer (right side) 16 years earlier, cured with surgery and radiation. She had also had a superficial malignant melanoma on her right arm in year 2000 with no recurrence. Results of a biochemical test confirmed the diagnosis with 24-hour urine free cortisol . She had no diurnal rhythm and serum cortisol was around 600 to 1000nmol/L and plasma ACTH levels were between 30 and 45ng/L during the whole 24-hour sampling period. She did not suppress her cortisol and ACTH levels at all on either 1mg or 8mg dexamethasone suppression tests. An MRI scan of her sella turcica found no pituitary tumor. Bilateral inferior petrosal sinus sampling with corticotrophin-releasing hormone (CRH) testing revealed no central to peripheral ratio of ACTH, nor did ACTH increase during the CRH test. Fasting CRH was normal 3.5pmol/L (reference <5) and catecholamines and hydroxyindoleacetic acid in urine showed normal values. Chromogranin A was slightly elevated to 8.2nmol/L (reference <6). The patient did not have any proton-pump inhibitor. At this stage the clinical and imaging findings raised suspicion of an ectopic ACTH-producing tumor. A neck, thoracic and abdominal CT scan was done, showing only a slight enlargement of her left adrenal gland with attenuation of 25 Hounsfield units. Further, 11C-5 HTP-PET are centralized to one place in Sweden and finally we contacted the PET center in Uppsala, Sweden, and an 11C-5-HTP-PET was performed showing an 8mm mass in the left lung lower lobe with focal uptake  in increasing doses up to 2.5g, which could not be increased more due to side effects with increased flushing and anxiety attacks. Regardless of the increase in dose of metapyrone (metyrapone) in addition to ketoconazole no clinical improvement was seen, and due to further increase in the deterioration in liver enzymes ketoconazole was discontinued. However, her cortisol and ACTH levels were stable and did not further increase during the investigational period. Due to the deterioration of her health condition a bilateral adrenalectomy was planned. Investigations with After the surgery ACTH was below the detection limit (<5ng/L) with low morning cortisol levels below 100nmol/L, and replacement therapy with hydrocortisone was initiated.11C-5-HTP-PET was performed 6 and 24 months after the second operation without any sign of new endocrine tumors. A dual-energy X-ray absorptiometry (DXA) scan showed osteoporosis with T-scores of −3.0 both in L2 to L4 lumbar spine and in total hip and she was prescribed annual zoledronic acid infusions. At 24 months follow up, she still has low morning cortisol levels below 100nmol/L and plasma ACTH <5ng/L before hydrocortisone intake in the morning .Due to the finding of an atypical carcinoid and the narrow radical surgery an additional lobectomy was performed. The histology of the whole lobe showed no signs of recurrent tumor tissue and dissected regional lymph nodes were benign. A new A 22-year-old Caucasian man presented with clinical features of a severe CS including centripetal fatness, moon face, severe edema, excessive purple striae on his trunk, and frequent nocturia. During the last years the patient had gained 30kg in weight despite diet and exercise plans. Despite a loss of 10kg in weight during the past months his centripetal fatness increased and he was so swollen that his eyes hardly could be seen. The serum cortisol concentration was 670nmol/L at 8:00 a.m., and 703nmol/L at 7.30 p.m. Plasma ACTH was 140ng/L at 8.00 a.m. (reference <46) and 140ng/L at 7.30 p.m. Sampling of 24-hour urinary free cortisol showed increased values, >3000nmol/24 hour (reference <183). He did not suppress his cortisol or ACTH levels at all on 1mg dexamethasone suppression testing. His chromogranin A levels and blood sugar levels were normal, whereas a very low serum testosterone of 2.6nmol/L (reference 7.6 to 31) was recorded. An MRI scan of his sella turcica did not visualize any pituitary tumor, whereas a pulmonary chest radiograph and subsequent CT scan revealed a 12×9×14mm diameter mass in the right lower pulmonary lobe (FigureEven if lung carcinoids have a better prognosis than other ACTH-secreting neuroendocrine tumors like small cell carcinomas, thymic carcinoids, medullary thyroid carcinomas and gastrinomas, they are a malignant disease with potential for metastases and increased mortality,10. The 111In-pentetreotide-SRS used in our patient, with lower radiation dose and lower costs[11C-5-HTP-PET instead. Neuroendocrine tumors have a capacity for uptake and decarboxylation of amine precursors like 5-HPT, which can be used as a tracer for PET imaging[11C-5-HTP-PET has been shown to visualize more and smaller lesions down to 4 to 5mm[11C-5-HTP-PET was essential for the diagnosis. The patient was only days from bilateral adrenalectomy, which is an effective treatment[11C-5-HTP-PET in an earlier stage had been preferable. Moreover, we did not perform any SRS or 11C-5-HTP-PET before primary surgery in the second patient as suggested by some authors[The usefulness of Tc-99m-SRS has been described in selected cases with success and may er costs. Howeverer costs. FDG-PETer costs. Thereforeatment, but it reatment. We suggIn our department the routine procedure for pulmonary carcinoids is surgery with wedge excision aiming to save lung parenchyma. Young age, central tumor, and no nodal enlargement are highly suggestive of typical carcinoid and recent guidelines advocate no further diagnostic or staging tests beyond chest CT and bronchoscopy before resection using parenchyma-sparing techniques. However11C-5-HTP-PET preoperatively in addition to laboratory specimens the post-surgery follow up is facilitated[Taken together, if the tumor can be visualized with SRS or 11C-5-HTP-PET in selected cases is preferable when a CT and MRI examination fail to detect any ectopic ACTH-producing tumor.Diagnostic evaluation time is limited due to the aggressive course in ectopic ACTH-dependent CS. We suggest that a referral to a specialized center for investigation with Written informed consent was obtained from the patients for publication of this case report and any accompanying images. A copy of the written consents are available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.JW and BE both participated in the design and coordination of the case reports, drafting the manuscript and interpreting the radiological figures and revised the article for important intellectual content and helped draft the manuscript. Both authors read and approved the final manuscript.