Video assisted thoracoscopic resection of an esophageal leiomyoma offers distinct advantages over an open approach. Many papers have described various techniques of thoracoscopic resection.We describe a 32-year old man who presented with intermittent dysphagia. Imaging studies showed a large esophageal leiomyoma. He underwent thoracoscopic enucleation using a three thoracic-ports technique.Thoracoscopic enucleation can be technically performed using a three thoracic-ports technique. Many authors have described the use of videothoracoscopic surgery to performed enucleation of an esophageal leiomyoma with various techniques -3. Everiet al  was the A 32-year-old man presented with a 6-month history of intermittent dysphagia. Barium swallow showed an extrinsic compression of the lower thoracic esophagus. An upper gastrointestinal endoscopy was performed, which revealed a large submucosal mass in the lower thoracic esophagus without mucosal irregularity. Endoscopic ultrasonography (EUS) and computed tomography (CT) of the thorax showed a large homogeneous tumor in the esophageal muscular layer, measuring about 5 × 6 cm, without paraesophageal lymph nodes and projecting into the right pleural cavity Figure . RoutineThe patient was intubated with a double lumen tube for one-lung ventilation and was positioned in the left lateral decubitus position. Three thoracic trocars were introduced Figure . The camIntraoperative endoscopy with air insufflation was performed to confirm esophageal integrity Figure . The esoBarium swallow at day 3 after surgery revealed no leakage and the patient was started on a liquid diet on day 4. The pathological report showed a leiomyoma with mitotic figure 0–1 per10 high power fields (HPF). Immunoperoxidase stainings were positive for smooth mucle actin, and negative for S-100, CD34 and C-kit. The patient was discharged on postoperative day 6. The patient is currently asymptomatic three months after surgery.Esophageal leiomyoma is an uncommon benign tumor of smooth muscle origin. The most common anatomical location is in the lower third of the esophagus . MalignaA thoracoscopic approach offers potential advantages compared with traditional thoracotomy, including minimal aesthetic disability, less pain, and better postoperative respiratory function. The limited operative trauma should allow a reduced postoperative hospital stay and more rapid resumption of normal activity .The leiomyoma enucleation was easily performed and the esophageal muscular layer was carefully closed because of the reported development of a pseudodiverticulum after the procedure -8. IntraSome authors state that intraoperative endoscopy is not necessary to detect any perforation, because they infused blue dye proximally to the tumor after distal compression to create a bulge in the mucosa . HoweverThe advantages of thoracoscopic removal of esophageal leiomyoma are confirmed in the limited series that have been published in recent years. Of particular significance is the relative simplicity of this kind of approach compared with traditional thoracotomy . Even wiThoracoscopic enucleation is treatment of choice of esophageal leiomyoma. Even with previous reports using four thoracic ports, the technique is replicable with only three ports.The author(s) declare that they have no competing interests.TA is the primary surgeon in charge who prepared the manuscript, VC is the second surgeon in charge who helped in the preparation of manuscript and edited it for its scientific content. TA, JS are the surgical staff who helped in the preparation of the figures for this manuscript. VL edited the manuscript for its scientific content.All authors read and approved the final manuscript.


Peri-appendicular abscess is usually due to primary appendicitis or it may be caused by various pathological conditions: inflammatory, infective, neoplastic, immunological, ischemic, occlusive table .We present a case of right iliac fossa abscess, initially diagnosed as a complication of acute appendicitis that, at surgical exploration, was proven to be produced by a cecal tumour causing appendicitis for an obstructive mechanism.A 63-year-old male patient in reasonably good health suddenly suffered from fever (>38°C) and a painful right iliac fossa tumefaction; no other gastrointestinal problems were noted. On clinical examination, a mass of about 5 cm was palpable in correspondence of right iliac fossa and appeared hard, not reducible and fixed to the parietal muscle.Laboratory data showed mild anaemia  and elevated erythrocyte sedimentation rate (74 mm/hr) as pathological findings. Alvarado score was 6 and therefore a parenteral antimicrobial therapy was administered.Abdominal ultrasound examination evidenced an abscess in the right lower quadrant, with heterogenic echotexture and a thickening of the ileocecal tract. Abdominal computerized tomography (CT), confirmed the presence of a complex, predominantly cystic, mass of large size (6 × 8 cm) with heterogeneous, mainly peripheral enhancement figure . The adjBecause of worsening abdominal pain and clinical signs of right iliac fossa peritonitis, the patient was not submitted to contrast enema and colonoscopy to avoid the risk of extensive peritoneal contamination for abscess rupture or exacerbation of bowel perforation with severe diffuse peritonitis and was directly operated on.Exploratory laparotomy revealed a voluminous cecal mass fixed to the abdominal wall, covering a purulent, loculated abscess cavity that was copiously irrigated and drained. A thick and dilated appendix was finally evidenced strictly adherent to the cecal mass that appeared quite suspicious. Careful inspection of liver, spleen, peritoneal cavity and mesentery was done. The lesion was considered resectable and a right-sided hemicolectomy was performed.Gross examination of the resected specimen showed an ileocecal valve tumour with ulcerated aspect extending to the fundus of cecum. The histopathology revealed a well-differentiated adenocarcinoma invading the muscularis propria. No metastatic lymph nodes were found (B2 stage in the Astler-Coller classification). Resection margins were free. Marked peri-appendiceal inflammation significant for appendiceal perforation was evidenced. Appendicitis and perforation originated from chronic obstruction of the base of appendix caused by the cecal neoplasm.The patient underwent adjuvant chemotherapy with fluorouracil and levo-folinic acid. At a 6 month follow-up no sign of recurrence was evidenced.Pathophysiologically, the relations between cecum cancer and appendicular abscess may be the followings: 1) cecum cancer and appendicular abscess may be two co-existing and independent affections, 2) cecal neoplastic lesion may cause appendicitis by mechanical obstruction at the orifice of the vermiform appendix, 3) adenocarcinoma of cecum may present clinically as appendiceal abscess due to transmural invasion with perforation.Colonic perforation due to carcinoma may occur at the tumour, either proximal or distal to the neoplastic lesion . The perPatients with perforated cancer are at risk of diffusion of cancer cells within the abdomen and pelvis and consequently of peritoneal carcinomatosis . HoweverSonography and computed tomography show a high degree of accuracy in detecting the presence of an abscess . The radPreoperative colonoscopy in presence of cecal abscess and inflammation may be hazardous and be at risk of complications such as diffuse peritonitis for abscess rupture or exacerbation of bowel perforation, development of liver abscess -10. AlsoThe treatment of appendiceal abscesses is still discussed and many different approaches are nowadays adopted. Expectant management, consisting of intravenous antibiotics, percutaneous drainage and interval appendectomy at a later date, is gaining a wide consent as it seems associated with less morbidity and shorter overall hospital stay ,12. HoweTherefore, an aggressive surgical approach of cecal masses, with intra-operative abscess drainage and resection should be performed in selected cases to avoid the risk of delaying the diagnosis and unrecognising malignant lesion as well as to achieve a safe and adequate treatment . Careful


Teratomas are manifested with a great variety of clinical and radiological features, while sometimes they simply represent incidental findings.A rare case of benign teratoma of the dermoid cyst type, in an adult 40-year-old female patient, is reported. The patient had presented recurrent pulmonary infections for the previous 2 months, persistent cough, and progressively aggravating dyspnea. A chest X-ray showed total atelectasis of the left lung, and the thoracic CT-scan revealed a huge mass, containing multiple elements of heterogeneous density, probably originating from the mediastinum, occupying the whole left hemithorax. The mass compressed the vital structures of the mediastinum, great vessels and airways, and a chest MRI was performed to accurately detect the anatomical relations. The patient underwent left thoracotomy and the tumor was totally resected. The size of the tumor was extremely large although no invasion to the vessels or to the airway had occurred. Adherence to the adjacent left pulmonary artery and left main bronchus was present, but without erosion or fistulization. The postoperative course was uneventful, while the histological examination confirmed a teratoma.A teratoma is a non-homogeneous pathological entity, clinically, radiologically or histologically. It is predominantly diagnosed between the second and fourth decade and the incidence is equal for both sexes. Symptoms are absent in one half of the patients. The case reported is noteworthy as the tumor appeared with total atelectasis of the left lung, and symptoms started 2 months prior to diagnosis. Total removal of the tumor is adequate treatment for this type of teratoma and the prognosis is excellent. Mature teratomas are the most common histological type of germ cell tumors, followed by seminomas . Germ ceA 40-year-old female with negative smoking and medical history was admitted with productive cough, progressively aggravating dyspnea on exertion, and recurrent pulmonary infections for the previous 2 months. The chest X-ray showed total atelectasis of the left lung Figure , and thend postoperative day. Preoperative atelectasis of the left lung was totally resolved  have no signs or symptoms when the mass is initially diagnosed. Symptoms commonly present are chest, back or shoulder pain, dyspnea, cough, fever, pleural effusion, and bulging of the chest wall . In the In spite of the pressure on the mediastinal vessels or the airway, invasion of these anatomical elements does not usually occur. However, erosion of the tumor to the bronchus, as well as to the pleural space, to the skin, and to the aorta has been reported.Diagnostic assessment is performed with classical X-rays, followed-up by CT. Typically, a well-circumscribed anterior mediastinal mass extending to one side of the midline and protruding into one lung field is revealed. CT accurately estimates the density of all included tissues, such as soft tissue , fluid (88%), fat (76%), calcification (53%), and teeth and such imaging findings are considered specific . MRI is Macroscopically the tumors are spherical, lobulated, with a well-defined capsule, and contain a variety of material, lipid-rich fluid, cheese-like substances, teeth, hair, and cartilage. Histologically, benign teratomas comprise at least 2 of the 3 primordial layers: ectoderm (skin and hair), mesoderm , and endoderm .Surgical resection is the treatment of choice and radical extirpation secures a long survival out of recurrence. Median sternotomy is usually preferred for tumor removal, but access via either posterolateral or anteroposterior thoracotomy depends on the size, location, and expansion of the tumor. Difficulty in surgical maneuvers may be a result of the vital structures involved. In a large series of 95 patients with benign mature teratoma, in addition to tumor resection, 3 patients required lobectomy, 5 patients additional partial resection of the lung, and 7 patients pericardectomy. VATS techniques have been introduced in teratoma resection with promising results . The appIn the case reported, left posterolateral access was chosen, as the tumor was almost entirely located into the left hemithorax, reaching and adherent to the left hemidiaphragm. Such a location precludes median sternotomy, otherwise the preferred approach, as surgical manipulations are impossible on the lower lobe of the left lung and on the left hemidiaphragm. MRI was performed after the CT-scan in order to more precisely reveal the relations with the mediastinum vital anatomical structures  because it was critical to clarify whether there was an invasion or simply compression. The examination pointed to the latter and loose tumor adhesions with the great vessels were confirmed on surgery and gently submitted to blunt dissection. The left lateral decubitus position of the patient did not influence ventilation during operation, as ventilation conditions with a double lumen tracheal tube were similar to the preoperative values affected by the left lung atelectacis. Furthermore, left lung operation secured the right lung against potential contamination from infected content of the chronically collapsed left lung.• Such a huge teratoma extending to the whole hemithorax and resulting in total atelectasis of the left lung has been only sparsely reported previously ,15.• Even a huge mass causes simple compression and deviation without invasion of the vascular and airway structures.• Complete resection is adequate treatment for the patient with favorable long-term prognosis.The author(s) declare that they have no competing interests.CZ was the main surgeon of the patient, who reviewed the literature and had the central responsibilityin the management ofthe patient and the writing of this report,DR was the pathologist, who examined and diagnosed histologically the resected specimen,GS was the interventional bronchoscopist and pulmonologist, who performed bronchoscopy, functional respiratory tests and assessed the patient as candidate of major surgical procedure,KV and KS were assistants in this operation and helped in preparation of the manuscript,MA offered his valuable experience in the management of the patient and the writing of this report,IB coordinated writing of the manuscript.All authors read and approved the final manuscript.


In the developed world, small bowel obstruction accounts for 20% of all acute surgical admissions. The aetiology for majority of these cases includes postoperative adhesions and herniae. However, a relatively uncommon cause is a Meckel's diverticulum. Although this diagnosis is primarily reported in the adolescent population, it should also be considered in adults.In the present report, we present a rare case where a fit and healthy 74-year-old gentleman, with no previous history of abdominal surgery, presented with the cardinal symptoms and signs of small bowel obstruction as the result of a Meckel's diverticulum encircling his terminal ileum. Initial investigations included a supine abdominal x-ray showing dilated loops of small bowel and computerised tomographic imaging of the abdomen, which revealed a stricture in the terminal ileum of unknown aetiology. At laparotomy, multiple loops of distended small bowel were seen from the duodeno-jeujenal junction to the terminal ileum, which was encircled by a Meckel's diverticulum. The Meckel's diverticulum was then divided to release the obstruction, mobilised and subsequently removed. Finally, the small bowel contents were decompressed into the stomach and the nasogastric tube aspirated, before returning the loops of bowel into the abdomen in sequence. The patient made a good postoperative recovery and was discharged home 5 days later.This report highlights the importance of considering a Meckel's diverticulum as a cause of small bowel obstruction in individuals from all age groups and especially in a person with no previous abdominal pathology or surgery. In the developed world, small bowl obstruction accounts for 20% of all acute surgical admissions. The aetiology of small bowel obstruction includes several pathological factors, with the most common cause being postoperative adhesions followed by herniae . HoweverA Meckel's diverticulum is a congenital pouch on the wall of the distal ileum, usually about 2 inches from the ileocecal valve. It represents a vestigial remnant of the omphalomesenteric duct and occurs in approximately 2% of the population, found twice as frequently in males as females. Of those individuals who have a Meckel's diverticulum, only 2% are symptomatic and they tend to be typically below the age of two, thereby accounting for why this congenital gastrointestinal anomaly is comparatively better studied in adolescents compared to in adults.The main complications caused by a Meckel's diverticulum, include intersusseption and volvulus in adolescents and acute bleeding in adults . HoweverHere, we present a case of a Meckel's diverticulum causing acute small bowel obstruction in a 74-year-old gentleman as a result of it encircling, and thus constricting, the terminal ileum. To the authors' knowledge, and from an extensive review of the literature, such an unusual presentation of a Meckel's diverticulum has not been previously reported.A fit and healthy 74-year-old gentleman presented to the accident and emergency department at the West Suffolk Hospital with a 3-day history of abdominal pain, vomiting, absolute constipation and abdominal distension. The abdominal pain initially started as a dull generalised discomfort, but later became colicky in nature with a subjective severity of 7/10. There were no other abdominal or genitourinary symptoms. The patient had an unremarkable past surgical history, with no prior abdominal surgery, and a past medical history of only hypercholesterolaemia.On examination, positive findings included marked abdominal distension, generalised abdominal tenderness, tinkling bowel sounds and soft stools high in the rectum. Important negative findings included no herniae and no signs of peritonism.Initial management of the patient involved intravenous fluid resuscitation, nasogastric tube insertion, catheterisation, routine bloods and erect chest and supine abdominal x-rays. Significant elevations in blood concentrations of urea, creatinine and C-reactive protein were noted, with dilated loops of small bowel Fig.  and no fFollowing general anaesthesia, a midline laparotomy was performed on the patient. On entering the peritoneal cavity, gross distension of the small bowel and collapse of the large bowel was identified. The small bowel was subsequently delivered carefully and examined. Loops of distended small bowel were identified extending proximally from the duodeno-jejunal junction to the distal ileum. At approximately 10 cm from the ileo-caecal valve, there was a long tubular structure encircling and obstructing the terminal ileum, which proved to be a Meckel's diverticulum. The base of the Meckel's diverticulum arose approximately 40 cm proximal from the ileo-caecal valve. The encircling Meckel's diverticulum did not appear inflamed or thickened and was divided at the base using a linear stapler  to release the obstruction. Care was taken not to compromise the lumen of the ileum. The tip of the diverticulum was then dissected off the terminal ileum and the anastomosis over sewn with continuous 3/0 sutures. The small bowel was then decompressed and the content milked gently into the stomach before being aspirated via the nasogastric tube. The loops of bowel were then returned into the abdomen in sequence. Closure of the abdomen was performed using loop sutures. Following this, the patient made a good postoperative recovery and was discharged home 5 days later.The management of any acute surgical abdomen, including acute bowel obstruction, follows 4 stages: (I) formation of an initial diagnosis, (II) confirmation of a diagnosis, (III) confirmation of the aetiology underlying the diagnosis and (IV) surgical intervention to treat the emergency.A diagnosis of acute bowel obstruction is made initially on clinical judgement based on the history and physical examination of the patient. The cardinal symptoms and signs are colicky abdominal pain, vomiting, absolute constipation and abdominal distension, all of which were present in this patient.Confirmation of bowel obstruction is then usually made with a plain supine abdominal x-ray. This simple and easily performed test provides the surgeon with several useful pieces of information, including whether there is small and/or large bowel obstruction and the degree of obstruction. In the present case, markedly dilated loops of small bowel with no visible loops of large bowel were seen on the abdominal x-ray Fig. , thus inHaving established and confirmed a diagnosis of small bowel obstruction, the next goal is to identify the aetiology underlying the obstruction. The two most common causes of small bowel obstruction in the developed world are postoperative adhesions and herniae . HoweverBased on these findings, and the absence of clinical improvement whilst on IV fluids and nasogastric tube aspiration, surgery was therefore indicated. However, the surgical approach to acute bowel obstruction of unknown aetiology remains controversial. While some surgeons advocate laparoscopic intervention due to its minimally invasive approach and shorter patient hospitalisation , others At laparotomy, an unusually long Meckel's diverticulum was found, which had managed to entirely wrap itself around the terminal ileum thereby forming an internal hernial orifice in which the bowel had become incarcerated and subsequently obstructed. What makes this case exceptionally unusual is that the Meckel's diverticulum was not thickened or inflamed. This is in contrast to the other cases previously reported in the literature, where an internal hernial orifice was created by the Meckel's as the result of adhesions or bands between an inflammatory end of the diverticulum and either the surrounding mesentery  or the nThis report therefore highlights the importance of considering a Meckel's diverticulum as a cause of small bowel obstruction in individuals from all age groups and especially in a person with no previous abdominal pathology or surgery.The author(s) declare that they have no competing interests.All authors have read and approved the final manuscript.AST : Involved in the conception of the report, literature review, manuscript preparation, manuscript editing and manuscript submission.SSL : Involved in the manuscript editing and manuscript review.DOR (Consultant Surgeon): Involved in the manuscript editing and manuscript review.


Neurofibromatosis type 1 is an autosomal dominant neurocutaneous disorder with characteristic features of skin and central nervous system involvement. Gastrointestinal involvement is rare, but the risk of malignancy development is considerable. Zollinger-Ellison syndrome is caused by gastrin-secreting tumors called gastrinomas. Correct diagnosis is often difficult, and curative treatment can only be achieved surgically.A 41-year-old female affected by neurofibromatosis type 1 presented with a history of recurrent epigastric soreness, diarrhea, and relapsing chronic duodenal ulcer. Her serum fasting gastrin level was over 1000 pg/mL. An abdominal CT scan revealed a 3 × 2-cm, well-enhanced mass adjacent to the duodenal loop. She was not associated with multiple endocrine neoplasia type 1. Operative resection was performed and gastrinoma was diagnosed by immunohistochemical staining. The serum gastrin level decreased to 99.1 pg/mL after surgery, and symptoms and endoscopic findings completely resolved without recurrences.Gastrinoma is difficult to detect even in the general population, and hence symptoms such as recurrent idiopathic peptic ulcer and diarrhea in neurofibromatosis type 1 patients should be accounted for as possibly contributing to Zollinger-Ellison syndrome. Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous disorder with an estimated prevalence of 2 to 3 cases per 10,000 population [Zollinger-Ellison syndrome (ZES) is characterized by hypersecretion of gastrin from a gastrinoma that leads to gastric acid hypersecretion and, most notably, clinical symptoms of refractory peptic ulcer disease. Zollinger and Ellison were the first to report recurrent peptic ulcers of the jejunum associated with nonbeta islet cell tumors of the pancreas . The diaIn this report, we describe a patient with NF1 who was diagnosed as ZES and successfully treated by curative surgery.A 41-year-old female NF1 patient was admitted for the evaluation of a 3-year history of recurrent epigastric soreness, heartburn, and diarrhea. Repeated endoscopic examinations revealed recurrent duodenal ulcers. The symptoms were relieved by proton-pump inhibitors, but recurred when the medication was withdrawn. She was previously diagnosed with NF1 based on clinical features and family history. Her first-degree relatives  were also affected by fully developed features of NF1. She had no cognitive dysfunction or learning disabilities, and showed normal intellectual development. A physical examination revealed several café-au-lait spots and multiple small nodules on the anterior chest and areolar area, and also multiple axillary freckles. A skin-nodule biopsy demonstrated characteristic neurofibromas.Helicobacter pylori were both negative. The serum fasting gastrin level was >1,000 pg/mL and 837 pg/mL in two consecutive measurements. A secretin stimulation test was not performed due to the unavailability of secretin. As a diagnosis of gastrinoma was strongly suggested, radiologic evaluations were performed to locate the lesion. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) scans showed a 3 × 2-cm, clearly defined, well-enhanced mass adjacent to the duodenal loop in the subhepatic space  declare that they have no competing interests.CHP, YEJ, HSK, SKC, JSR, and SJK made substantial contributions to the conception and interpretation of clinical data and case-related studies, and clinical decisions. YSK, JCK, and CKC performed the surgery and provided final diagnosis of the disease. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:


Torsion or rupture of an ovarian cyst may present as an acute abdomen. A case is presented where the diagnosis was made at laparoscopy and laparoscopic resection was done. Controlled aspiration of the cyst contents allowed the cyst to be easily removed from the abdomen. Torsion or rupture of an ovarian cyst may present as an acute abdomen. Sometimes the diagnosis will only be made at laparoscopy and this can be unsettling for a non-gynecologic surgeon. We present such a case and discuss our management.A 20 year old young woman presented to the emergency department with a sudden onset of severe right iliac fossa pain of 12 hours duration. The pain was constant with no radiation and was rapidly worsening prior to her admission. It was associated with anorexia and vomiting. On direct questioning there were no relevant gynecoligic or urologic symptoms. The patient reported a similar, but milder episode, one month ago when she was seen at another hospital. No diagnosis was made at the time and the pain resolved after analgesia.On examination, she was haemodynamically stable and apyrexial. She was tender in the right iliac fossa with guarding and rebound tenderness. Hematological blood tests revealed a neutrophilia and a urinary pregnancy test was negative. The patient was listed for an urgent laparoscopy with the provisional diagnosis of appendicitis or ruptured ovarian cyst.After induction of general anesthesia it became apparent that there was a large central abdominal mass. Laparoscopy using one 10 mm and two 5 mm ports showed bleeding from a large torted right ovarian cyst of approximately 15 cms diameter figure  and an eA dermoid is a benign, cystic lesion containing tissue from all three embryonic layers; endoderm, mesoderm and ectoderm. Ovarian dermoids constitute 10–15% of ovarian tumours and tend to occur in young women in the reproductive years, although presentation has been reported in prepubertal and elderly patients [They are slow growing structures; one recent study found a growth rate of 1.8 mm/year in pre-menopausal women . OvarianThe first laparoscopic cystectomy was performed in 1989 and since this time various spillage rates have been reported . This isLaparoscopy is the preferred technique for diagnosing and treating the acute abdomen. In our case no preoperative scan was done and it was not until general anesthesia was induced that a mass was felt. Although in our case there was bleeding from the ruptured, ischemic cyst, the true cyst wall was intact. Unfortunately we did not have a large enough retrieval bag available on the day so a controlled aspiration of the cyst was done prior to placing it in a retrieval bag. No 'dermoidal contents' were spilt and the operation was completed with a thorough washout with normal saline. In our opinion there was no chance of chemical peritonitis from the procedure.Ovarian dermoid cysts may present as an emergency after torsion or rupture and from time to time general surgeons will encounter such a case. Laparoscopic management is beneficial and preservation of ovarian tissue and the fallopian tube is usually possible. We would recommend placing the cyst into a bag and externalizing it prior to aspiration to avoid any risk of contamination.


Wilms' tumor is the commonest primary malignant renal tumor in childhood. Rarely, it may present in the adult age group.et al.We report a 48-year-old male presenting with flank pain and haematuria. Abdominal ultrasound revealed a right renal mass measuring 11 × 10 cms, and a clinical diagnosis of renal cell carcinoma was made. Nephrectomy was performed, and a final diagnosis of adult Wilms' tumor was made based on the criteria proposed by Kilton The possibility of an adult Wilms' tumor should be considered when a patient presents with pain in the flank and a renal mass. Rarity of the tumor favors documentation in literature. Nephroblastoma or Wilms' tumor is the most common malignant renal tumor in children. It accounts for approximately 5–6% of the neoplasms in children and is rare in the adult population . Less thA 48-year-old male presented with haematuria and dull aching pain in the right flank. The general examination of the patient was unrevealing, with no lymphadenopathy or bony tenderness. The chest, cardiovascular and neurological examination was normal. Abdominal examination revealed a mildly tender mass palpable in the right lumbar region. Routine laboratory investigations including a full blood count, chest X-ray, and renal function tests were normal. Urine examination showed plenty of RBCs in high power field.An ultrasound examination of the abdomen revealed an echogenic mass in the upper part of the right kidney measuring 11 × 10 cm. No calcified areas were noted; the renal pelvis and renal vein were not involved. The other abdominal viscera were radiologically normal. There was no lymph node enlargement. A provisional diagnosis of renal cell carcinoma was made and right radical nephrectomy performed.Grossly, the nephrectomy specimen weighed 480 grams and showed a solid spherical tumor measuring 10.5 × 10 cm. The cut surface was variegated with grey white, fish flesh appearance. The adjacent renal parenchyma appeared normal. The renal capsule was intact. There was no involvement of the renal pelvis or vein.Microscopically, the tumor was highly cellular comprising of epithelial, blastemic and stromal elements. The epithelial element comprised of tubules admixed with blastemic cells Fig. . The tub2 and actinomycin D 15 mcg/kg. The patient remains in complete remission after 14 months of follow up.Adjuvant treatment was started as per Intermediate Risk protocol for Stage I Node negative disease with vincristine 1.4 mg/mth century German surgeon Carl Max Wilhelm Wilms, is probably derived from primitive metanephric blastema. The histological appearance is characterized by marked structural diversity. Classic Wilms' tumor is composed of three types of cells – blastemal, stromal, and epithelial; although the occurrence of all three types in the same case is uncommon [Wilms' tumor, named after the 19uncommon .Adult Wilms' tumor is diagnosed based on the criteria given by Kilton, Mathews, and Cohen . These iTo the best of our knowledge, the present case is only the second case in Indian literature to have the classic triphasic histology. Blastemic component was predominant in the histology in 6 out of the 8 adult Wilms' reported in Indian literature ,6.The differential diagnosis of an adult Wilm's tumor with mainly epithelial differentiation includes metanephric adenoma. A predominant blastemic Wilms' tumor has a strong resemblance to small, blue round cell tumors which commonly include lymphoma, peripheral neuro-ectodermal tumor and rhabdomyosarcoma; and rarely metastatic small cell tumors from lung, immature teratoma, and primary renal cell sarcoma. Extensive search for any other components is needed as a poorly differentiated renal carcinoma can have large sarcomatous areas resembling blastema .Most adults present with local pain and haematuria, in contrast to the palpable boggy mass which is more common in children. In adults, Wilms' tumor is larger and ill-defined, with areas of necrosis and hemorrhage. About half of the patients have stage 3 or 4 disease . There aTremendous data on the biology and epidemiology of Wilms' tumor has been generated by the National Wilms' Tumor Study (NWTS) which was established in the USA in 1969. An update from the NWTS group about treatment outcomes in adults with favorable histology Wilms' tumor (FHWT) described 45 patients treated in the modern era. The overall survival rate was 82% [In 2004, Reinhard et al reported their experience with 30 cases of adult Wilms Tumor [Wilms' tumor in adults has a worse prognosis than in the pediatric population, a phenomenon for which there is no adequate explanation . As adulThe possibility of an adult Wilms' tumor should be considered when a patient presents with pain in the flank and a renal mass. Although the prognosis is poorer than that of children when the disease is compared stage for stage, the outcome for adult patients diagnosed with Wilms' tumor is steadily improving.


Lipomas are common benign soft tissue neoplasms but they are found very rarely in the deep lobe of parotid gland. Surgical intervention in these tumors is challenging because of the proximity of the facial nerve, and thus knowledge of the anatomy and meticulous surgical technique are essential.A 71-year-old female presented with a large asymptomatic mass, which had occupied the left facial area for over the past fifteen years, and she requested surgical excision for a cosmetically better facial appearance. The computed tomography (CT) scan showed a well-defined giant lipoma arising from the left deep parotid gland. The lipoma was successfully enucleated after full exposure and mobilization of the overlying facial nerve branches. The surgical specimen measured 9 × 6 cm in size, and histopathology revealed fibrolipoma. The patient experienced an uneventful recovery, with a satisfying facial contour and intact facial nerve function.Giant lipomas involving the deep parotid lobe are extremely rare. The high-resolution CT scan provides an accurate and cost-effective preoperative investigative method. Surgical management of deep lobe lipoma should be performed by experienced surgeons due to the need for meticulous dissection of the facial nerve branches. Superficial parotidectomy before deep lobe lipoma removal may be unnecessary in selected cases because preservation of the superficial lobe may contribute to a better aesthetic and functional result. Their occurrence in the head and neck is relatively rare , most co in 1977 , and few in 1977 ,5-9, SurA 71-year-old female presented in our clinic with a large left facial mass requesting surgical excision for a cosmetically better facial appearance. She had been aware of the slow-growing, painless swelling for over the past fifteen years. She had sought medical advice ten years before, but she had been unwilling to undergo surgery due to the fear of the postoperative morbidity. Clinical examination revealed a mobile, soft, and non-tender mass measuring 10 × 7 cm in size over the region of the left parotid and upper lateral neck area  may be helpful in further assessment and diagnosis. Ultrasonography has been used as an initial imaging study in cases suspected to have head and neck lipomas ,15. CompOn CT scans, lipomas have the typical characteristics of homogeneous masses with few septations, a specific range of CT Hounsfield Unit (HU) values , and they show no contrast enhancement . MRI canIn this reported case, however, the high-resolution CT scan provided enough information with respect to the preoperative planning and contributed to the diagnosis. Although MRI may prove to be a better diagnostic tool regarding tumor margin characteristics, this did not change our surgical strategy and it was not necessary to modify the operation based on the MRI differing from CT findings. In addition, the cost of MRI is nearly three times that of CT and so we believe that although MRI is highly useful, the CT scan with specific radiodensity recording is the preferred preoperative investigation.Fine needle aspiration biopsy (FNAB) requires an experienced cytologist, but it still has a significant false negative rate in salivary gland tumors. It has also proved to be unreliable in diagnosing parotid lipomas ,9,17. FuConservative follow-up might be a valid option for patients with clinically static deep parotid lipomas, since lipomas can now be confidently recognized by CT and MRI. Surgical intervention in these tumors is challenging and may be reserved for patients with cosmetic or pressure effects. Possible postoperative morbidities, such as facial nerve dysfunction, facial scar or asymmetric contour, and Frey's syndrome must be explained to the patient before operation. During surgery, most surgeons recommend superficial parotidectomy with facial nerve dissection before removal of the deep lobe parotid lesions -9,12. TrIn our surgical experience of this case, because the lipoma was mainly associated with the lower branches of the facial nerve, we exposed these branches from the main trunk by meticulously dissecting its overlaying superficial parotid lobe instead of performing a formal superficial parotidectomy. Then we reposited the raised superficial parotid lobe to its original site after tumor removal. We find the procedure with preservation of the superficial parotid lobe may have the following advantages: it helps to maintain better facial contour, and the need of resection of the redundant skin can be avoided. In addition, the incidence of postoperative Frey's syndrome is reduced.Giant lipomas involving the deep parotid lobe are extremely rare. Although MRI may provide better tumor margin characteristics, the CT scan with specific radiodensity recording is the preferred preoperative investigation. Surgical management of deep lobe lipoma is challenging and should be performed by experienced surgeons due to the need for meticulous dissection of the facial nerve branches. The postoperative aesthetic and functional results should be the major concerns. Therefore, a formal superficial parotidectomy may be unnecessary in selected cases, especially when the tumor only involves the lower branches of the facial nerve. Reposition and preservation of the raised superficial parotid lobe may contribute to a better postoperative aesthetic and functional result.The author(s) declare that they have no competing interests.CWW, HPC: Took part in the care of the patient, organised clinical photographs and wrote first draft of the manuscript. FYC: study conception and design. YCH: Performed literature search, retrieved articles and helped with manuscript preparation. LPC: Examined surgical specimen, took the photograph of the specimen and helped with manuscript preparation. WRK: Critical revision and supervision.All authors read and approved the final manuscript.None


Dysgerminoma is the most common malignant germ cell tumor of the ovary. This malignancy can be associated with pure gonadal dysgenesis or Swyer syndrome, mixed gonadal dysgenesis and partial gonadal dysgenesis.Dysgerminoma developed in 3 phenotypic female patients with 46 XY pure gonadal dysgenesis. All patients presented first with abdominopelvic mass. Laparatomy was done. 46 XY karyotype was made by lymphocyte culture. Then these patients underwent gonadectomy that histopathology results were streak ovaries without evidence for malignancy. Two patients received postoperative adjuvant therapy.In Patients with Swyer syndrome the risk of dysgerminoma is high and gonadectomy is recommended. Also 5% of dysgerminomas are discovered in phenotypic female and 46 XY karyotype, thus in adolescent with dysgerminoimas and amenorrhea, karyotype should be done. The most common tumor is the often-bilateral gonadoblastoma, but dysgerminoma and even embryonal carcinoma also seen .We present three patients with pelvic mass, of which two patients, in spite of primary amenorrhea had nearly complete secondary sex characteristic. Another patient had secondary amenorrhea. All the patients had dysgerminoma, that underwent unilateral salpingoopherectomy and two of these, received adjuvant therapy. Gonadectomy after diagnosis of XY karyotype was done.st visit, she had nearly complete development of secondary sex characteristics. On examination the abdomen was soft, non tender with a palpable nontender fixed abdominopelvic mass. There was no evidence of ascitis. In vaginal exam, there was a normal length vagina and cervix. Uterus was palpable in rectovaginal exam.A 20 years old girl (160 cm height and 57 kg weight) presented in September 1999 with 6 months history of lower abdominal pain and gradual distention of abdomen. Clinically, she was noted to have an abdominal mass extending 4 cm above umbilicus. She had undergone an ultrasonography, which showed a huge complex abdominopelvic mass arising from the right ovary. Additional evaluation was negative for serum βHCG and AFP, but CA125 was 110 IU/ml. The FSH was 34 mIU/ml, the LH was 28 mIU/ml and serum estradiol was 36 pg/ml. Her past medical history was negative except for primary amenorrhea. She had gradual breast development since eleven years old and at her 1Exploratory laparotomy revealed a normal (infantile) uterus and tubes and a huge abdominopelvic mass 25 cm diameter and 3100 g weight, which was resected . At the end of the second course of chemotherapy (Bleomycin plus etoposide plus cisplatin), liver enzyme rose significantly and jaundiced, and then due to probable drugs induced cholestatic hepatitis, she was referred to radiotherapy. She received 4000 cGy and become severely leucopenic. One-month later exploration laparoscopy was performed and one residual gonad or streak gonad was found at the opposite site and she underwent gonadectomy. Histopathological examination showed a streak ovary without evidence for malignancy. She received estrogen and progesterone postoperatively, until now and she has monthly menstrual flow.A 19 years old female G0P0 (159 cm high and 55 kg Wight) with primary amenorrhea was referred to the Tehran University Gynecology cancer center with 7 months history of abdominal pain, which progressed to abdominal distension. Evaluation included pelvic exam and ultrasound, which documented a solid complex mass of 123 × 80 mm size. The first sonographic diagnosis was hematometrocolpos or endometriosis. Computerized tomography scan confirmed no upper abdominal disease or lymphadenopathy and confirmed pelvic mass and no ascitis. Additional evaluation was normal for serum CA125 and AFP and βHCG. FSH was 80 mIU/ml, LH was 38 mIU/ml, estradiol was 29 pg/ml and LDH was 1450 IU/ml. Her past medical history was negative. Her karyotype was reported as 46 XY, thus she underwent laparotomy, peritoneal washing, resection of tumor (in right gonad), Para aortic and bilateral lymphadenectomy and staging biopsies were done. Infantile uterus preserved and left gonad was resected. The right ovary measured 120 mm and weight 1700 g. Pathology revealed dysgerminoma in right ovary and Tumor was classified as stage IIB. Histological examination of left ovary was infantile gonad including fibrous bands that don't seen malignancy.Adjuvant chemotherapy consisted of 6 cycle of BEP after surgery was administered. Chest X-ray, abdominal and pelvic ultrasound and biochemical markers after 4 years remain normal. She receives estrogen and progesterone and has menstrual flow.st visit she had nearly complete development of secondary sex characteristic. In rectal exam, there was a normal length vagina and cervix, and uterus was palpable. Laparatomy revealed a normal uterus and tubes and a large pelvic mass, which resected. In left side there was no gonadal lesion. Mass resection was performed. Uterus was preserved. Pathology revealed ovarian dysgerminoma of the right ovary. Full metastatic work-up was negative. Karyotype was consistent with 46 XY (pure). Due to tumor was classified as stage IA grade 1, adjuvant therapy was not commented. The patient admitted for unilateral gonadectomy. Histopathological examination of left ovary showed a streak ovary that displays ovarian stroma but no follicles. She had been followed for 18 months and with estrogen plus progesterone had menses out flow.A 17 years old girl (168 cm high and 59 kg weight) with secondary amenorrhea was referred to our center in October 2004 with 4 months history of abdominal pain and distention of abdomen. Evaluation prior to referral included an ultrasound, which documented a complex abdominopelvic mass measured 95 × 80 mm thought to arise from the right ovary. Tumor markers were evaluated, that serum BHCG and AFP was normal, but CA125 was 90 IU/ml, LDH 844 IU/ml FSH 45 mIU/ml, LH 27 mIU/ml and estradiol 44 pg/ml. Past medical history in this patient was negative, except for secondary amenorrhea. She had breast development (tanner4) since 13 years old and at her 1Gonadal dysgenesis is characterized by the histology of the gonads of affected patient and based on that the 46 XY gonadal dysgenesis may be divided in to 3 histological categories:1) Complete or pure gonadal dysgenesis or Swyer syndrome. The patients are phenotypic females with a 46 XY karyotype and hypoplastic gonads without germ cells. They present most often with primary amenorrhea with normal stature. The gonads are usually streaks, but there may be some development of secondary sexual characteristics as well as a few episodes of uterine bleeding .2) Mixed gonadal dysgenesis that primary amenorrhea is associated with various mosaic status, the most common of which is 45X/46xx. When compared to the pure 45X cell line, individuals with 45X/46XX are taller and have fewer abnormalities. Spontaneous menstruation occurs in approximately 20% of these patients.3) Partial gonadal dysgenesis. They have karyotype 46XX with part of one of the X chromosomes missing. The phenotype is variable depending on the amount and location of the missing genetic material. In 46 XY partial gonadal dysgenesis individuals there is some testicular development; therefore they present as newborns with ambiguous genitalia.The etiology of 46 XY gonadal dysgenesis is though to be a short arm Y chromosome deletion involving SRY, a mutation in other genes that leads to inhibition of SRY function or mutation of SRY function .To date, 20% of 46 XY pure gonad dysgenesia are explained by a mutation or a deletion in SRY. In 80%, SRY is apparently normal. A female patient with an XY Karyotype who has a Palpable mullerian system, normal female testosterone levels, and lack of sexual development has Swyer syndrome, Tumor transformation in the gonadal ridge can occur at any age -3.The incidence of neoplasia in patients with gonadal dysgenesis is wider than reported. In 50 reported cases, there were 11 malignancy, 15 adenoma and 10 benign cases; a 22% incidence of malignancy and a 52% incidence of neoplasia. More recent series indicate a lower overall incidence of gonadal tumors about 5–10% but in Swyer syndrome the risk of gonadal neoplasia is high (20–30%), dictating early prophylactic removal of these dysgenetic gonads (2). Also Slowikowska  reportedet al., and Amice showed that SRY may play a formation of gonadal tumors, especially dysgerminoma in Swyer syndrome [Uehara syndrome ,21. HoweThe patients with 46 XY gonadal dysgenesis patients are diagnosed in early adolescence with delayed pubertal development. As expected they show elevated gonadotropins, normal female levels of androgens and low levels of estrogens, female external genitalia, uterus and fallopian tubes. Minimal breast enlargement reflects peripheral aromatization of androgens. Menstrual function suggests tumor development in the streak gonad. These streaks often display ovarian stroma but no follicles.In our cases, gonadotropins were about postmenopausal level (FSH>20 IU/L) and estradiol was at the low end of normal (40–400 pg/ml) in reproductive ages. Also menstrual function and breast enlargement associated with gonadotropin – independent precocious puberty. In this patient, estrogen or androgens produced by ovarian tumor .In accordance with Scully view the germ cells of the dysgerminoma seem the most likely source of the HCG, whereas the increased androgens were probably produced by the cells of the gonadoblastoma. Both hormone levels fell markedly on complete removal of the gonadal masses . But in Estrogen and progestin sequential therapy supports female secondary sex development in patients with gonadal dysgenesis and they are potential candidate for donor oocyte ,2.et al., analyzed seven tumor markers in germ cell tumors of the ovary. He showed positive rate of CA125 was over 50% in all tumor types except mature cystic teratoma. So CA125 was useful for the screening of malignant germ cell tumors. Dysgerminoma had a high positive rate of LDH. Positive rate of AFP was 11.8% in dysgerminoma, 100% in yolk sac tumor and 61.9% in immature teratoma [Kawai teratoma .In this article the plasma levels of CA125 in cases 1 and 3 were higher than expectant range, but in case2 were normal. LDH in case 2 and 3 were high and AFP in all patients was normal.Dysgerminoma is the most common malignant germ cell tumor of the ovary. It can be found either in a pure form or mixed with other germinal elements. Therefore in premenarchal patients with a pelvic mass, the karyotype should be determined. About 65% of dysgerminomas are stage I at diagnosis. About 85–90% of stage I tumors are confined to one ovary; 10–15% is bilateral. Dysgerminoma is the only germ cell malignancy that has this significant rate of bilaterality, other germ cell tumors being rarely bilateral [ The treatment of patient with early disgerminoma is primarily surgical, including resection of the primary lesion and proper surgical staging. Chemotherapy and/or radiation are administered to patients with metastatic disease. In patients whose contra lateral ovary has been preserved, disease can develop in 5% to 10% of the retained gonads over the next 2 years [These three cases presented demonstrate the occurrence of dysgerminoma in patients with Swyer syndrome. There is a relative paucity of published case studies on dysgerminoma in female patients with pure dysgenetic gonad. The development of secondary sex characteristics in these three patients is most unusual. The sexual development were complete and only along females lines, furthermore these patients had withdrawal bleeding response to estrogen and progesterone. A small number of cases have reported that unusual female secondary sexual development in patients with 46 XY gonadal digenesis .Bilateral dysgenesis of the testes (Swyer syndrome) affected individuals has an XY karyotype but normal (infantile) female external and internal genitalia. In these patients the risk of dysgerminoma is high and gonadectomy was recommended. Also in 5% of cases of dysgerminoma, XY karyotype is seen. Thus in adolescents patients with dysgerminoma with primary amenorrhea and secondary amenorrhea, Karyotype should be done. Also menstrual function in patients with 46 XY karyotype may be associated with estrogen secretion of tumoral lesion and investigation of gonads is recommended.The author(s) declare that they have no competing interests.NB: conception and editing of manuscriptMKZ: Literature search, and preparation of manuscriptAll authors read and approved final manuscript.


Primary diffuse leptomeningeal gliomatosis is a rare neoplasm confined to the meninges without evidence of primary tumor in the brain or spinal cord parenchyma. Cerebrospinal fluid diversion via ventriculoperitoneal shunt may be used as a therapeutic modality. Herein, we describe the first report of cytologic findings of a case of this neoplasm with shunt-related peritoneal metastasis.A 19-year-old male presented with a 6-month history of severe headaches. He had bilateral papilledema on physical exam. Cerebrospinal fluid examination was negative. Four months later a ventriculoperitoneal shunt was placed. Shortly thereafter, he was diagnosed with primary diffuse leptomeningeal gliomatosis based on the biopsy of an intradural extramedullary lesion adjacent to the lumbar spinal cord at a referral cancer center. The histology featured an infiltrating growth pattern of pleomorphic astrocytes with diffuse positivity for glial fibrillary acidic protein. A couple of months later he presented at our institution with ascites and an anterior peritoneal mass. Repeat cerebrospinal fluid cytology and fine needle aspiration of the mass confirmed disseminated gliomatosis. Cytologic characteristics included clusters of anaplastic cells of variable size, high nuclear to cytoplasm ratio and scant to moderate cytoplasm. Occasional single bizarre multinucleated cells were seen with eccentric "partial wreath-like" nuclei, clumped chromatin and prominent nucleoli. Patient expired 13 months after initial presentation.Disseminated primary diffuse leptomeningeal gliomatosis should be considered in the differential diagnosis of chronic aseptic meningitis and in the presence of a peritoneal tumor in patients with ventriculoperitoneal shunts. Immunocytochemistry may be of diagnostic value. Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare uniformly fatal neoplastic condition. It is characterized by widespread infiltration of the meninges by tumor thought to arise from heterotopic glial cell nests, without evidence of primary tumor within the brain or spinal cord parenchyma . Glial hPatients with PDLG most often present with symptoms and signs of intracranial hypertension, such as headache and papilledema. Examination of the cerebrospinal fluid (CSF) characteristically shows raised opening pressure, elevated protein concentration and lymphocyte pleocytosis. Diagnosis is often delayed mainly because of the nonspecific nature of the symptoms and CSF findings, such that empirical antituberculous therapy is frequently tried in patients with PDLG ,5. ThereTo reduce the intracranial pressure, some patients undergo a CSF diversion procedure in the form of ventriculo-peritoneal (VP) or alternate route shunting ,6. PeritHerein we describe the cytologic features of CSF and fine needle aspiration (FNA) of PDLG with VP shunt-related peritoneal spread.2O , protein of 778 mg/dL , glucose of 44 mg/dL , an absolute WBC count of 24/mm3 with 55% lymphocytes and 45% macrophages. A full microbiology workup was negative for organisms. The CSF was negative by polymerase chain reaction (PCR) for enteroviruses and West Nile virus. Flow cytometry showed no evidence of lymphoma or leukemia. Cytologic examination of the CSF was negative for malignant cells, showing predominantly reactive monocytes. The patient was initially managed symptomatically with analgesics and diuretics. By May of 2004, he developed cranial nerve palsies necessitating placement of a VP shunt. The patient sought a medical second opinion at another institution, where a diagnostic biopsy was obtained of an intradural extramedullary lesion at T12/cauda equina via vertebral laminectomy.A 19 year old college freshman presented, in January of 2004, with a 6 month history of severe recurring global headaches. He had no past medical or travel history. Physical examination was notable for bilateral papilledema. His workup included magnetic resonance imaging (MRI) of the brain and intracranial vessels, which initially did not reveal any abnormalities. Initial examination of the CSF revealed an opening pressure of 50 cm HDakoCytomation, Carpinteria, CA, 1:3000 dil; Figure The biopsy showed an infiltrating growth pattern of pleomorphic astrocytes. The nuclei were hyperchromatic with irregular borders. Blood vessels were numerous. Dense, wavy collagen bands were noted throughout the tumor, and seemed particularly apparent in the vicinity of blood vessels Figure . NecrosiDQ-stained smears showed pleomorphic malignant cells in poorly formed, somewhat cohesive clusters with haphazard cellular arrangement. The nuclei were hyperchromatic with great variation in size. Cytoplasmic borders were indistinct Figure . The TP A repeat CSF specimen was processed as two Cytospin slides, one stained with DQ and one with Pap. It showed a cytomorphology similar to that of the peritoneal mass FNA Figures  and 5B.The patient continued to deteriorate clinically. In addition to the non-improving neurologic status, he developed bowel obstruction which was unresponsive to palliative pelvic radiation and peritoneal infusion of phosphorus (P32) radioisotope. He expired in early February 2005, 13 months after initial presentation. An autopsy was declined.Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare neoplastic disorder limited to the meninges, in the absence of primary tumor within the brain or spinal cord parenchyma . The diaThe case presented in this report is unique in several respects. It describes an exceedingly uncommon disorder, PDLG, with peritoneal seeding as a result of symptomatic treatment (VP shunt). While this complication is known to occur in several different types of primary CNS tumors ,11, suchImmunocytochemistry has been advocated as a useful adjunct test in addition to routine CSF cytology in differentiating chronic aseptic meningitis from leptomeningeal carcinomatosis or gliomatosis especially in clinically suggestive cytologically negative cases . It seem


The occurrence of calcitonin-secreting primary carcinoid tumor of the urinary bladder is extremely rare.The case of a 68-year-old male with carcinoid tumor arising in the urinary bladder is presented. Transurethral resection of a polypoid small tumor 0.4 cm in diameter was performed. Immunohistochemical study using neuroendocrine markers allowed a straightforward diagnosis of a low-grade neuroendocrine carcinoma (carcinoid tumor) of the urinary bladder. Immunohistochemistry demonstrated calcitonin immunoreactivity in the most of the tumor cells.This tumor shows specific clinical, macroscopical and histological features and must be considered in the differential diagnosis of bladder neoplasms. The occurrence of primary carcinoid tumor of the urinary bladder is extremely rare. Two cases of these neoplasms have been reported recently by Martignoni and Eble . AccordiA 68-year-old man was admitted in January 2004 for a macroscopic hematuria. The patient had no relevant past medical or family history, and laboratory data were all within normal limits. A cystoscopic examination was performed and a transurethral resection of a tiny, sessile polypoid lesion of the bladder neck was carried out. Clinical evaluation included ultrasonography and computerized tomography (CT) scan. The patients was seen at last follow-up 14 months after diagnosis and he was completely negative.Grossly, the tumor was a well-circumscribed, small ovoid nodule of 0.4 cm in diameter. Histologically a polypoid tumor appeared to be covered by slightly attenuated normal urothelium. The tumor filled-up the lamina propria showing a predominantly glandular arrangement Fig , with scImmunohistochemically the tumor strongly reacted with synaptophysin Fig , chromogThe urinary bladder can be the site of tumors exhibiting various degrees of endocrine differentiation. It has become the most common site of extrapulmonary small cell undifferentiated carcinoma . The speThe present case shows clinically and microscopically the same features showed by two cases of carcinoid of urinary bladder reported recently by Martignoni and Eble . Their rAll 6 cases presented with hematuria, most of the patients aged of seven decade. All tumors were small, polypoid nodules at cystoscopy and most of them were localized in the neck of the bladder or trigone. All previously reported 6 tumors and the present case show a glandular architecture. Previous study, and our results have demonstrated the neuroendocrine differentiation. Strikingly the present case showed, immunohistochemically, a strongly reactivity with calcitonin. To the best of our knowledge this is the first case of a well-differentiated neuroendocrine carcinoma of the urinary bladder reported positive with this neuropeptide.Some tumors could simulate carcinoid of the bladder, in particular the nested variant of transitional cell carcinoma; differences in the cytologic features and immunohistochemistry should establish the diagnosis. The distinction between carcinoid tumor and inverted papilloma with glandular differentiation can be very difficult, however strong staining for neuroendocrine markers support the diagnosis of carcinoid tumor. Like the classic carcinoid tumors of the appendix, small bowel, or lung, all of the carcinoid tumors of the bladder are histologically and clinically not aggressive.In conclusion, we here add another case of carcinoid tumor of the urinary bladder to the existing literature, this tumor shows specific clinical, macroscopical and histological features and must be considered in the differential diagnosis of bladder neoplasms.The author(s) declare that they have no competing interests.MM participated to write the manuscriptVA operated the patient and participated to draft the manuscriptCM carried out imunohistochemical studyGN operated the patient and participated to draft the manuscriptGDR participated in the design of the studyLI participated in the design of the studyThe pre-publication history for this paper can be accessed here:


Anastomotic recurrence is often experienced at colocolic or colorectal anastomoses. Tumor cell implantation has been reported as the mechanism of anastomotic recurrence. However, anastomotic recurrence occurring repeatedly after curative surgery is rare. We herein report a rare case of repeated anastomotic recurrence after curative surgery for sigmoid colon cancer.A 51-year-old man underwent radical surgery for sigmoid colon cancer. However, anastomotic recurrence developed three times during three years and six months after the initial operation in spite of irrigation with 5% povidone-iodine before anastomosis. The serum carcinoembryonic antigen (CEA) level had been within normal limits after sigmoidectomy. Finally, the patient underwent abdominoperineal resection. The clinico-pathological findings revealed that possible tumor cell implantation caused these anastomotic recurrences. The patients survived without recurrence during the follow-up period of seven years and nine months.We experienced a rare case of repeated anastomotic recurrence due to possible tumor implantation after curative surgery for sigmoid colon cancer; however the prognosis was ultimately very good. CEA monitoring was insensitive for detection of anastomotic recurrence in this case. Anastomotic recurrence is likely to develop at colocolic or colorectal anastomoses. The incidence rate is reported at 5–10% . HoweverA 51-year-old man visited our hospital with complaint of fecal occult blood. The laboratory data were with-in normal limits . Barium enema and colonoscopy showed an elevated lesion with depression and three subpedenculated polyps in the sigmoid colon. Pathological findings on biopsy confirmed a diagnosis of adenocarcinoma. We performed sigmoidectomy with lymphadenectomy in June 1999. Histological examination revealed the tumor to be a well-differentiated adenocarcinoma invading the subserosa with two lymph nodes metastases . Tumor cells were not identified at the surgical margins. One of three subpedenculated polyps was diagnosed histologically as an adenocarcinoma in adenoma, and the others were adenomas  on July 2001, and it was nine months after the prior operation that the second anastomotic recurrence was found. Computed tomography (CT) and ultrasonography (US) did not describe distant organ metastasis or local recurrence in the pelvis, but colonoscopy and barium enema performed in July 2001 showed the second recurrence of disease at the suture line. An operation was performed on December 2001, and the tumor was resected T Figure .The patient noticed bleeding per rectum during defecation in July 2002, seven months after the prior operation. Romanoscope and colonoscopy showed a tumor at the suture line, and the pathological examination of the biopsy confirmed a diagnosis of adenocarcinoma. Although the serum CEA level 5.1 ng/ml) was within normal limits, the CA19-9 level was 50.4 U/ml this time. There was no metastasis in either the abdominal cavity or the distant organs by preoperative CT and US preoperatively. We performed abdominoperineal resection with lateral lymphadenectomy for the third anastomotic recurrence on September 2002. The lesion measured 2.0 by 4.0 cm, and was located in the suture line. The pathological examination revealed a moderately differentiated adenocarcinoma invading the subserosa, and there was no invasion of tumor cells to any surgical margin. Metastases of tumor cells were not identified histologically in any resected lymph nodes  declare that they have no competing interests.KF: Primary author. Carried out all the surgical procedures.JK: A contributing author. Carried out the surgical procedures with KF.NS, HS and KS: Literature review and report preparation.TT: Consultant surgeon. Approved the manuscript for publication.All authors read and approved the revised version of this manuscript for publication.


Positron emission tomography (PET) has come to play an increasingly important role in the evaluation of pulmonary lesions, which are suspicious of malignancy. As is true for other techniques, PET gives false positive and negative results. We report a case of pulmonary amyloidosis with multiple pulmonary nodules showing different uptake of 18 F-fluorodeoxyglucose (FDG) on PET. There are limitations of specificity of FDG-PET in characterising pulmonary nodules and it is important to confirm a suspected malignancy with histology before potentially curative treatment is undertaken. PET imaging is a technology that is used to identify focal areas of increased cellular metabolism. FDG is an excellent tracer for detecting malignant disease because of high glucose metabolism observed in cancer cells. With this technique whole body image can be created to accurately identify malignant primary neoplasm and metastatic disease.For pulmonary lesions FDG-PET has been used to differentiate malignant from benign lesions and to stage and follow patients with lung cancer. PET has demonstrated sensitivity and specificity rates of 80–90% compared to 50–60% for CT . Low-graAmyloidosis is a disorder in which insoluble protein fibres become deposited in tissues and organs. The exact mechanism that causes amyloidosis is unknown. However, primary amyloidosis appears to be related to abnormal production of amino globulins by plasma cells whereas secondary amyloidosis is associated with the presence of chronic inflammation of infectious disease. A variety of inflammatory and infectious disorders can result in increased FDG activity. Gallium 67, an inflammatory seeking agent, has been reported to have shown intense uptake in amyloid goitre as a result of inflammatory action. It is conceivable that inflammatory action caused by amyloid lesion result in increased FDG uptake[top panel). CT findings were suspicious of multiple pulmonary metastasis. He had FNA of one of the peripheral nodules on the left side under CT guidance and the microscopy was suggestive of nodular amyloidosis. To rule out malignancy in the rest of the nodules he underwent an FDG-PET scan . There was no other metabolically active lesion seen in the lungs that would correspond with the nodular opacities identified on CT. Though some of the nodules were below the size resolution of PET and remained uncharacterised, however, others were large and in the absence of significant uptake these were thought to be benign on PET scan. Given the dangers of reporting benign disease on FNA, it was thought prudent to confirm the diagnosis with excision biopsy. Surgical exploration revealed a well-demarcated palpable mass in the apical segment of the left lower lobe. There was another smaller nodule present in the left upper lobe. Both of these were wedged out and sent for frozen section. The histopathology findings were consistent with pulmonary amyloidosis in both the nodules. Afterwards, the patient was investigated for evidence of myeloma or plasmacytoma. All subsequent investigations including bone scan, bone marrow aspiration trephine were normal and ruled out secondary amyloidosis.A 55-year-old farmer was referred to our hospital for further evaluation of multiple pulmonary nodules detected on his chest x-ray for his chronic cough. He was a smoker with a history of asbestos exposure. His physical examination and laboratory findings were unremarkable. A computed tomography (CT) scan of the chest revealed multiple bilateral focal pulmonary lesions varying in size from 1 to 2.5 cm with no evidence of lymphadenopathy. The largest nodule measuring 2.5 × 2.5-cm was noticed in the apical segment of the left lower lobe  declare that they have no competing interests.Sumit Yadav – sequence alignment and drafted the manuscript.Sanjay Sharma – helped to draft the manuscript.Ian Gilfillan – conceived of the case report.


Extremely rare cases of paraneoplastic syndromes or ectopic production of proteins associated with liposarcoma are reported in literature. Production of Granulocyte-Colony Stimulating Factor, alpha-fetoprotein, paraneoplastic pemphigus and leucocytosis, Acrokeratosis paraneoplastica (Bazex's syndrome) are reported.The present report describes a case of retroperitoneal liposarcoma associated with small plaque parapsoriasis. Our search in the English literature of such a kind of association did not reveal any case reported.A 74 year male patient was admitted to our hospital because of the presence of an abdominal mass in right iliac fossa. He also complained of a two-year history of psoriasiform eruptions. The CT scan showed a retroperitoneal pelvic mass. Therefore surgical resection of the tumor was performed. After surgery, the skin eruptions disappeared completely in seven days and so a diagnosis of parapsoriasis syndrome was done.Parallel disappearing of skin eruptions after surgery, typical clinical picture and not specific histology of the cutaneous lesions suggest the diagnosis of small plaque parapsoriasis. Therefore we propose to add Small Plaque Parapsoriasis to the list of paraneoplastic syndromes associated to liposarcoma. Cutaneous paraneoplastic syndromes are a large group of clinical manifestations that can occur in association with an internal malignancy. Characteristically, they follow a course parallel to the tumor and resolve with the successful treatment of primary tumor.They may precede symptoms associated with the neoplasm and its recurrence will trigger a relapse . The preA 74 year old male patient was admitted to our hospital because of subjective sensation of weight in right abdominal lower quadrant lasting 20 days. The patient also complained of a two-year history of skin eruptions.3. Hemoglobin was 12,5 g/dl and the platelet count was 123.000/mm3. Hematocrit was 35.5%. Serum concentrations of carcinoembryonic antigen (CEA), CA 19-9 and CA 72-4 were within normal limits.A physical examination revealed an abdominal mass in right iliac fossa. It wasn't floating and sore and it had a roundish form. The patient presented anular, erythematous, itchy skin lesions with irregular borders and shaded margins over the trunk and lower limbs. Each lesion was from 1 cm to 5 cm in diameter , vascular dermatoses (Trousseau's syndrome), papulosquamous disorders , and disorders of hair growth (acquired hypertrichosis lanuginosa) .et al., described a unique case of relapsing undifferentiated liposarcoma with biochemical, immunoistochemichal and molecular evidence of alpha-fetoprotein (AFP) ectopic production [Therefore extremely rare cases of paraneoplastic syndromes or ectopic production of proteins associated with liposarcoma are reported in literature. Nakamura et all reported for the first time a case of a mesenteric liposarcoma producing G-CSF presenting with iperpirexia and other signs of severe inflammation . Bosco eoduction . Sporadioduction ,6.Recently liposarcoma has been added to the growing list of malignant neoplasm associated with Acrokeratosis paraneoplastica (Bazex's syndrome) . Our casThey are divided into three subtypes :parapsoriasis en gouttes, today referred as pytirias lichenoides;1. parapsoriasis lichenoide, today referred as parakeratosis variegata;2. parapsoriasis en plaque, today classified in a small plaque parapsoriasis (SPP) and large plaque parapsoriasis (LPP).3. Small plaque parapsoriasis affects adult men and is characterized by well bounded oval erithematous lesions, with a slightly desquamative surface, pink-yellowish colour and diameter inferior to 5 centimeters. The skin eruptions, sometimes associated with itchness, are localized over the sides of trunk and limbs, as in our patient. Unlike small plaque parapsoriasis, the psoriasiform lesions in Bazex's syndrome are distributed symmetrically over the acral locations, involving helices of ears, nose, fingers, toes, nails, palms, soles and they tend to spread, if the tumour remains untreated, on the elbows, knees and trunk. Since the typical acral locations were not present in our patient, the diagnosis of paraneoplastic acrokeratosis (Bazex's syndrome) has been excluded.Histopatologic aspects are not specific and show dermic perivascular lymphocitic infiltration, parakeratosis and sometimes spongiosis .Parallel disappearing of skin lesions after surgical resection of retroperitoneal liposarcoma supports our diagnosis of paraneoplastic syndrome. Moreover typical clinical picture and non specific histology support the diagnosis of small plaque parapsoriasis. Therefore we propose to add SPP to the list of paraneoplastic syndrome associated with liposarcoma.The author(s) declare that they have no competing interests.FT – main author, was involved in the diagnosis of the case and performed surgery;SB – concept development and draft the manuscript;MS – gathered the data and draft the manuscript;PP – performed literature review;LT – was involved in diagnosing the case and wrote the manuscript;AB – supervised the process and finally approved the manuscript for been published.All authors have read and approved the final manuscript.


Congenital alveolar rhabdomyosarcoma is an extremely uncommon and invariably fatal tumor with the current therapy. Less than 25% of patients present with evidence of cutaneous metastasis.We report a case of congenital alveolar rhabdomyosarcoma in an 18-month-old male who presented with a progressively increasing mass lesion in the left post-auricular region since birth. Radiological examination did not show any intracranial involvement of the mass lesion. Upon resecting the mass that was 10-cm in largest dimension, the gross, microscopic, and ultrastructural findings were consistent with congenital alveolar rhabdomyosarcoma.The suspicion of alveolar subtype on histological grounds and proper evaluation of this tumor by immunostain and ultrastuctural examination is necessary. In the Medline literature search, there is no report of large congenital alveolar rhabdomyosarcoma in the post-auricle region. Although rhabdomyosarcoma (RMS) is an uncommon childhood malignancy, it is the most frequently encountered soft tissue sarcoma found in infants and children . Six perCongenital alveolar RMS, defined as disease present at birth, is a rare subtype of RMS. We present a case of congenital alveolar RMS, which was completely resected at the age of 18 months.An eighteen-month-old male child presented with a mass lesion in the left post-auricle region. The mass was present at birth and was gradually increasing in size. The measurement of the mass was 10 × 6 × 4 cm. The skin over it was unremarkable, and the other systemic examination was within normal limits. A non-contrast computed tomography of head and neck revealed the mass in the left post-auricular region  was made after complete excision of the mass. Grossly, the mass was fairly well circumscribed, multinodular with a glistening, gelatinous gray-white surface. Cystic areas were seen in between Figure . NeitherMicroscopically, it revealed a varying degree of cellularity with densely packed cellular areas separated by a framework of hyalinized fibrous septa. The tumor cells were discohesive in the centre. The individual cells were large, round with darkly staining hyperchromatic nuclei, inconspicuous nucleoli, and scant indistinct cytoplasm Figure . High miUltrastructuraly, the tumor cells showed few cytoplasmic organelles and occasional bundles of thin myofilaments measuring 4–6 nm in diameter. Bone marrow examination didn't reveal any metastatic deposit. A cytogenetic analysis was not performed because of technical reasons. In the one year follow-up period, there has been no recurrence or metastatic deposit.Rhabdomyosarcoma (RMS), a malignant tumor originating from skeletal muscle, usually occurs in the first two decades of life. The vast majority of RMS occurs in head and neck region, extremities, genitourinary system and trunk. RMS bears a close resemblance to various stages in the embryogenesis of normal skeletal muscle, but its pattern is much more variable and ranges from poorly differentiated tumours that are very difficult to diagnose without immunohistochemical or electron microscopic examination to a well-differentiated RMS.A retrospective study has shown the incidence of tumours in the neonatal period to be 7.2 per 100,000 live births per year 1:13,700) .Only a few cases (less than 20) have been reported in the literature about alveolar subtype of congenital rhabdomyosarcoma. Alveolar RMS has been previously described in the region of the eyes, ears, infratemporal, cervical and parameningeal regions in neonates and children . But to Our case belongs to Group II of clinical staging (Intergroup Rhabdomyosarcoma Studies Classification) . The alvDifferential diagnosis includes neuroblastoma, neuroepithelioma, extra skeletal Ewing's sarcoma, melanotic neuroectodermal tumour of infancy and malignant lymphoma.The clinical management of neonates with malignancy presents considerable problems due to the physiologic immaturity of many organ systems in infants. Neonates in the Intergroup Rhabdomyosarcoma Studies Classification (IRS) study received 70% of the prescribed dose of the three principal agents used to treat RMS . Severe myelosuppression and infections were the principal side effects. It is interesting to note that our patient had no recurrence of the lesion or any metastatic deposit elsewhere at the end of one year follow-up. Surgery followed by the standard chemotherapy regimen was given according to the treatment plan. Grundy et al reported the longest survivor who underwent myeloablative therapy with peripheral stem cell support, and hence suggest that more intensive treatment may be of value in this rare condition .Our case exemplifies the rare sub-type of congenital RMS occurring in the post-auricular area. This is a highly malignant tumor with no record of long-term survivors. In this setting, clinical outcome and radiological findings must be accounted for by both pathologists and clinicians who must be aware of such an unusual presentation of congenital rhabdomyosarcoma, whose diagnosis requires representative tissue sampling and whose treatment may require an adjustment of drug dosage.


Spontaneous rupture of the spleen is an uncommon dramatic abdominal emergency that requires immediate diagnosis and prompt surgical treatment to ensure the patients survival. Infections have been cited in most cases involving splenic rupture but are rare in hematological malignancies despite frequent involvement of the spleen.We present a case of a splenic rupture caused by infiltration of B-cell lymphoma. A 43 year old gentleman presented with a 1 day h/o left upper quadrant pain; nausea and vomiting for 2 days with associated dizziness and anorexia. The CT showed abnormal spleen 20 × 11 cm with free fluid in the abdomen and enlarged retroperitoneal LNs. The patient underwent a splenectomy after initial resuscitation and the operative finding was that of a massively enlarged spleen with areas of tumor extruding through the splenic capsule.Although the spleen is often involved in hematological malignancies, splenic rupture is an infrequent occurrence. In a recent literature review 136 cases were of splenic rupture secondary to hematological malignancy were identified. Acute leukemia and non Hodgkin lymphoma were the frequent causes followed by chronic myelogeneous leukemia. Male sex, adulthood, severe splenomegaly and cytoreductive chemotherapy were factors more often associated with splenic rupture. Emergency splenectomy remains the cornerstone treatment for splenic rupture.We present a case report of a "spontaneous splenic rupture" and discuss the presentation, etiology and treatment options along with discussion of relevant literature Spontaneous rupture of the spleen is an uncommon, dramatic abdominal emergency that requires immediate diagnosis and prompt surgical treatment to ensure the patient's survival. Spontaneous rupture rarely occurs in a histologically proven normal spleen and in such cases is called a 'true spontaneous rupture'. Spontaneous rupture usually occurs in a diseased spleen and is called 'pathologic spontaneous rupture' .Spleen is an immunological organ commonly involved in both hematological and non hematological diseases. Infectious diseases has been cited in most cases involving splenic rupture -5 but ar43year old male presented to the Emergency Department with a day old history of acute left upper abdominal pain; nausea and vomiting for 2 days with some associated dizziness and anorexia. The patient underwent an emergency contrast enhanced CT scan of the abdomen/pelvis which showed an markedly abnormal spleen, inhomogeneous and enlarged, approximately 20 × 11 cm with fluid in the inferior aspect of the mesenteric fat, with free fluid in the abdomen and enlarged retroperitoneal lymph nodes.The patient was volume resuscitated following admission to the hospital, and his Hct was serially followed. Since there was no history of trauma and the CT did not look like a traumatic splenic laceration the oncology service was contacted who felt that the findings were most compatible with lymphoma and because there was some bleeding going on did not recommend trying to treat it without a tissue diagnosis.The patient underwent a splenectomy. He was found to have a massively enlarged spleen with areas where it appeared to be tumor extruding through the splenic capsule. There was a lot of ecchymotic tissue inferior to where the spleen had been and there was slight oozing which was controlled as much as possible as the area was quite friable. The tail of the pancreas was identified and left alone as there was no bleeding. The estimated blood loss was approx 2 L.Following extubation the patient had some issues with agitation which was attributable to ETOH withdrawal. The patient had a history of 6pack qd ETOH abuse. He was placed on CIWA protocol. His mental status remained at the level of agitation and confusion for the next 4 days not helped by serax or ativan or haldol. Psychiatry consult was given and they felt the agitation was secondary to post op delirium vs. paradoxical ativan reaction. At that point it was thought that the agitation and delirium was secondary to possible 'pain vs. sepsis.' He was started on cefazolin 1 gm q8 hrs post op which was changed to meropenem 1 gm q 12 hr on POD#4.In the post operative period the patient's Hct remained stable on serial examination. however his Bilirubin continually rose and peaked at 10.5 on post op day # 3. Other LFT s were mildly elevated. Gastroenterology team was consulted who felt that the increase bilirubin was secondary to sepsis. Differential diagnosis of hemolysis was considered but was thought unlikely as the Hct remained stable. Biliary obstruction was unlikely given his LFT s were only mildly elevated. A hepatitis panel was run and the patient was found to be positive for Hepatitis C. Anesthesia related rise in bilirubin was also considered but again AST and ALT were mildly elevated.The patient was discharged home on post op day #9 on PO Moxifloxacin 400 mg q d for 5 days to complete a 14 day course of antibiotics. His liver function test lab values were almost near normal on discharge.According to the criteria described by Orloff and Peskin in 1958  and 14]14].The spontaneous rupture of a diseased spleen, even if rare, is more frequent and, according to the criteria of Orloff and Peskin, should be categorized as a pathologic rupture. Diseases that more commonly may induce the spontaneous rupture of the spleen are the oncologic and hematologic diseases ,17, someHowever, in most reports in the current literature, splenic rupture is commonly categorized as spontaneous when it occurs without trauma, whether or not the rupture is pathologic. Oncologic and hematologic diseases often cause spontaneous rupture of the spleen; in a review of the literature, Giagounidis et al.  found 13There are three main pathogenetic factors that may explain the rupture . First, Our case occurred in a patient affected with non-Hodgkin's lymphoma. Here, the infiltration of the splenic parenchyma by blast cells was the main cause of the rupture because the coagulation tests of the patient were normal and no clear splenic infarcts had been demonstrated. The splenic rupture occurred before the start of antitumoral chemotherapy, which may have released lytic and vasoactive enzymes due to chemotherapy-induced cell necrosis. Giagounidis et al. reported that approximately 18% of the 136 spontaneous ruptures due to oncologic diseases occurred a few hours before beginning antitumoral chemotherapy. Moreover, we suggest that patients with oncologic or hematologic diseases and with splenic tumor-related lesions should be monitored carefully, in particular at the beginning of chemotherapy, for signs of pathologic spontaneous rupture of the spleen.Emergency splenectomy represents the only feasible treatment for splenic rupture. Among 136 cases of pathological splenic rupture reported in the literature, 88 patients underwent surgical intervention, while 43 patients were managed without operation. No information could be obtained for the 5 remaining cases. Of the 88 patients who underwent surgical intervention, 55 (63%) survived and 33 (37%) died. Of the 43 patients who did not undergo surgery, 40 died . SplenicUsing ultrasonography or computer tomography,  and 27]27] perit