The management of penetrating abdominal stab wounds has been the subject of continued reappraisal and controversy. In the present study a novel method which combines the use of diagnostic laparoscopy and DPL, termed laparoscopic diagnostic peritoneal lavage (L-DPL) is describedFive trauma patients with penetrating injuries to the lower chest or abdomen were included. Standard videoscopic equipment is utilized for the laparoscopic trauma evaluation of the injured patient. When no significant injury is detected, the videoscope is withdrawn and 1000 mL of normal saline is infused through the abdominal trochar into the peritoneal cavity, and the effluent fluid studied for RBCs, WBC, amylase debry, bile as it is uced in regular diagnostic peritoneal lavageLaparoscopic peritoneal lavage (L-DPL) was then performed and proved to be negative in all 5 patients. RBC lavage counts above 100,000/mcrl were not considered as a positive lavage result, because the bleeding source was directly observed and controlled laparoscopically. All patients recovered uneventfully and were released within 3 days. This procedure combines the visual advantages of laparoscopy together with the sensitivity and specificty of DPL for the diagnosis of significant penetrating intra-abdominal injury, when the diagnostic strategy of selective consevatism for abdominal stab wounds is adopted.A method of laparoscopic diagnostic peritoneal lavage (L-DPL) in hemodynamically stable patients with penetrating lower thoracic or abdominal stab wounds is described. The method is especially applicable for trauma surgeons with only basic experience in laparoscopic technique. This procedure is used to obtain conclusive evidence of significant intra-abdominal injury, confirm peritoneal penetration, control intra-abdominal bleeding, and repair lacerations to the diaphragm and abdominal wall. The combination of laparoscopy and DPL afforded by the L-DPL method adds to the sensitivity and specificity of DPL, and avoids under or over sesitivty, that have limited the use of DPL in the hemodynamically stable trauma patients with suspicious or proven peritoneal penetration. The management of penetrating abdominal stab wounds has been the subject of continued reappraisal and controversy. Traditionally, the concern for delayed diagnosis of intra-abdominal injuries has led many trauma centers to advocate mandatory abdominal exploration whenever a stab wound was proven or suspected to have penetrated into the abdominal cavity ,2. This Selective operative management of asymptomatic patients ("selective conservatism") was therefore advocated in 1960 when it was recognized that 25% to 33% of the patients with stab wounds have no peritoneal penetration, and of those with proven penetration, significant abdominal injuries are present only in about 45% [In order to overcome the diagnostic delay and reduce the number of non-therapeutic laparotomies, diagnostic peritoneal lavage (DPL) was applied which resulted in reduced incidence of non-therapeutic celiotomies of as little as 7%–15% .Diagnostic laparoscopy in trauma patiens has been first described by Gazzaniga in 1976. Despite this long history of sporadic use, indications for its use in trauma casualties remain controversial. This technique has primarily been used to detect peritoneal penetration in tangential wounds of the abdominal wall and for evaluation of the diaphragm in patients with lower thoracic wounds [In the present study a novel method which combines the use of diagnostic laparoscopy and DPL, termed laparoscopic diagnostic peritoneal lavage (L-DPL) is described. This method was used for evaluation of significant intra-abdominal injuries in hemodynamically stable trauma patients with penetrating lower thoracic or abdominal stab wounds. We believe that this procedure can be safely performed by the trauma surgeon with basic skills in the laparoscopic technique.Five trauma patients with penetrating injuries to the lower chest or abdomen were included.The entrance criteria for diagnostic laparoscopy were as follows:a. The patient was hemodynamically stable, with negative or equivocal abdominal physical findings, b. Stab wound in the lower chest , c. Pentration into the abdominal cavity proven by wound exploration, d. Inconclusive wound exploration, e. suspected peritoneal penetration on tripple-contrast abdominal CT scan or FAST in stab wounds to the flank or back, and f. No significant intra-abdominal injury that necesitates open laparotomy was detected by abdominal CT or FAST.2 is conducted with either a Verres needle or a Hassan cannula. Initial insufflation pressures in the trauma setting should be limited to 8–10 mmHg in the patient with penetrating thoraco-abdominal wounds as this will minimize the development of tension pneumothorax in patients with diaphragmatic defects. If no diaphragmatic injury is detected in lower thoracic stab wounds , the procedure is terminated without lavage. When a diaphragmatic tear is detected, it can be repaired laparoscopically by non-absorbable sutures before peritoneal lavage is performed.Standard videoscopic equipment is utilized for the laparoscopic trauma evaluation of the injured patient. In the hemodynamically stable patient, the procedure starts with local wound exploration (LWE) to determine peritoneal penetration (Table). This is sometimes difficult and tedious especially in obese patients or patients with thick abdominal musculature. If no penetration is proven the patient is observed and discharged without further investigations. When penetration is proven or exploration of the wound is equivocal, FAST and/or triple-contrast CT can be added to demonstrate the presence of local signs of peritoneal penetration, intra-abdominal bleeding, and intra-abdominal injuries. A nasogastric tube and a urinary bladder catheter are inserted for decompression prior to performing laparoscopy. A 30 degree angled 10 mm scope is preferred, as this provides optimal visualization of difficult areas such as the posterior aspect of the diaphragm and anterior abdominal wall. Initial insufflation of COIn abdominal stab wounds, after exclusion of diaphragmatic injury, insufflation pressure can be increased to 15 mmHg, as used in standard laparoscopy. One or more ports can be added for laparoscopic repair, or if improved exposure is needed. When significant hemorrahage cannot be controlled laparoscopically, or enteric contents are encountered during initial laparoscopic assessment, the examination is terminated and converted to an open exploratory laparotomy.When no significant injury is detected, the videoscope is withdrawn and 1000 mL of normal saline is infused through the abdominal trochar into the peritoneal cavity. The patient is rolled from side to side to promote mixing of the lavage fluid with the peritoneal contents. Fluid returning through the trochar after mixing is inspected and immediately sent for quantitative analysis. The lavage fluid is considered positive if feces, bile, food, bacteria on Gram stain, >500 white blood cells/mcrl, or increased amylase levels are detcted. A red blood cell count >100,000/mcrl by itself is not considered positive.If the lavage fluid return proves to be positive, the procedure is immediately converted to an open exploratory laparotomy. When the lavage fluid proves to be negative, a J-P closed drain is left in the peritoneal cavity, the procedure is terminated without open exploration. Postoperatively the patient is carefully observed for 24 hours, after which the J-P drain is extracted, and the patient can be discharged. In case of change in the patient' s condition or suspicious fluid return appears in the drain during observation, the lavage procedure can be repeated. Complete intraoperative evaluation of the lavage fluid return was accomlished in less than 30 minutes.There were 2 lower thoracic stab wounds, 2 abdominal stab wounds and one stab wound at the costal margin. When peritoneal penetration was suspected or proven, a thorough diagnostic laparoscopic survey was performed, actively bleeding vessels were coagulated, and diaphragmatic or abdominal wall lacerations repaired laparoscopically. Laparoscopic peritoneal lavage (L-DPL) was then performed and proved to be negative in all 5 patients.RBC lavage counts above 100,000/mcrl were not considered as a positive lavage result, because the bleeding source was directly observed and controlled laparoscopically. All patients recovered uneventfully and were released within 3 days. In no patient was repeated peritoneal lavage necessary, and the J-P drains were removed on POD 1.A procedure of combined diagnostic laparoscopy and laparoscopic diagnostic peritoneal lavage (D-DPL) for evaluation of hemodynamically stable trauma patients, with lower thoracic or anterior abdominal stab wounds, that have penetrated the abdominal cavity, is hereby described. This procedure combines the visual advantages of laparoscopy together with the sensitivity and specificty of DPL for the diagnosis of significant penetrating intra- abdominal injury, when the diagnostic strategy of selective consevatism for abdominal stab wounds is adopted. An important advantage of the procedure is that it can be safely performed by trauma surgeons with only basic experience in the laparoscopic technique.The accuracy of DPL has been reported to be greater than 95% and has proven to be highly reproducible among centers , but oveDiagnostic laparoscopy is commonly used only to detect injury to the diaphragm with penentration into the abdominal cavity. Once peritoneal penetration has been detected, the laparoscopic procedure is usually terminated and converted to an open procedure, because most trauma surgeons with only basic experience in laparoscopic technique, regarded laparoscopic abdominal exploration unsafe because it could not definitely exclude small but significant intra-abdominal injury, such as a small intestinal perforation.Salvino et al.  suggesteDeMaria et al.  comparedOur method of laparoscopic diagnostic peritoneal lavage (L-DPL) in hemodynamically stable patients is indicated not only for stab wounds in the lower thoracic region but particularly for evaluation of abdominal stab wounds with proven or suspcious pritoneal penetration. Laparoscopic evaluation allows more specific diagnosis of the nature and degree of injury as well as the need for laparotomy. Once peritoneal penetration through the abdominal wall or diaphragm is proven, the injury in the abdominal wall can or diaphragm can be repaired laparoscopically, and diagnostic peritoneal lavage is performed through the abdominal trochar before termination of the surgical procedure. Reliance on the findings of laparoscopy avoided in our study a nontherapeutic laparotomy, even when a "DPL positive" RBC count of more than 100,000/mcrl was detectedA disadvantages of L-DPL are that it is an invasive procedure, that is usually performed under general anesthesia. There are potential dangers of tension pneumothorax and gas embolization in trauma patient ,11. CompL-DPL is also recommended for trauma surgeons familiar with advanced laparoscopic techniques, when no significant injury was detected by a thorough laparoscopic survey. When laparoscopic diagnostic and therapeutic procedures, such as hemostasis of liver or spleen lacerations, suture repair of the diaphragm, or thorough examination of the small and large bowel have been accomplished, the addition of L-DPL before termination of the procedure, increases the confidence that a small but significant injury, such as a small bowel perforation was not overlooked.The advantages in diagnosis and therapeutics afforded by the L-DPL method are also expected to reduce morbidity, hospital costs, and length of hospitalization associated with non-therapeutic open laparotomies in patients with penetrating lower thoracic or abdominal injuries.A method of laparoscopic diagnostic peritoneal lavage (L-DPL) in hemodynamically stable patients with penetrating lower thoracic or abdominal stab wounds is described. The method is especially applicable for trauma surgeons with only basic experience in laparoscopic technique. This procedure is used to obtain conclusive evidence of significant intra-abdominal injury, confirm peritoneal penetration, control intra-abdominal bleeding, and repair lacerations to the diaphragm and abdominal wall. The combination of laparoscopy and DPL afforded by the L-DPL method adds to the sensitivity and specificity of DPL, and avoids under or over sesitivty, that have limited the use of DPL in the hemodynamically stable trauma patients with suspicious or proven peritoneal penetration.L-DPL- Laparoscopic peritoneal lavageCT- Computed tomographyFAST- Focused abdominal sonography for traumaLWE- Local wound explorationMichael M. Krausz, M.D – Has made substantial contributions to conception, design, collection, and analysis of dataBenyamine Abbou, M.D – Has made substantial contributions to design and conceptionDan D. Hershko, M.D – Has made substantial contributions in revising critically the ManuscriptAhmad Mahajna MD – Has made substantial contributions in revising critically theManuscriptDaniel S. Duek, M.D – Has made substantial contributions in collecting dataBishara Bishara M.D – Has made substantial contributions in collecting dataShlomo H.Israelit, M.D. Has made substantial contributions in design and analysis of data


Gastrointestinal stromal tumours (GIST) are rare tumours, now more frequently identified with the new imaging modalities like computerised tomography (CT) and magnetic resonance imaging (MRI). We report a rare presentation of a GIST with an unusual diagnostic workup in a multidisciplinary setting leading to a definitive diagnosis and treatment.A 55-year-old lady was admitted under the general surgeons, with 3-day history of abdominal pain, three-week history of loss of appetite and weight. The patient was sequentially investigated with ultrasonography, computerised tomography and finally selective angiogram in a multidisciplinary setting. The selective angiogram showed a GIST with intratumour bleed, leading to successful surgical excision and being recurrence free at 22 month follow up.Clinical presentation of these tumours can be varied and gastrointestinal bleeding is the commonest mode described in the literature. The clinician needs to be aware of much more rare presentations of the GIST including an intra tumour bleed. A structured multidisciplinary approach would lead to successful diagnosis and treatment. Gastrointestinal stromal tumours (GISTs) are rare, representing 0.1–3% of all gastrointestinal cancers with an estimated incidence of 15 per million , are preA 55-year-old woman was admitted under the general surgeons with a three-day history of colicky right hypochondrial and epigastric pain together with three-weeks of reduced appetite and weight loss of 5 kilograms. Abdominal examination revealed mild tenderness in the right upper quadrant. Routine investigation revealed haemoglobin of 11.6 gm/dl and marginally elevated alkaline phosphatase of 110 mg/dl. Abdominal ultrasound scans revealed a 7.2 × 6.8 cm thin-walled partially cystic and solid structure adjacent to the inferior margin of the left lobe of liver.Computerised tomography (CT) of the abdomen confirmed a partly cystic and partly solid mass with some contrast enhancement and low volume ascitis figure , 2. She Angiography showed normal hepatic artery architecture. Distal gastroduodenal and gastro-epiploic catheterisation exhibited tumour neo-vascularisation figure , which wThe histology confirmed a 4.5 × 3.5 cm GIST with evidence of recent haemorrhage, strongly positive for CD117 with negative resection margins and a favourable mitotic number of <5/50 HPF. The patient made an uneventful recovery and has remained recurrence free for 22 months.GISTs can present in a number of different ways and are often diagnosed incidentally. In a population-based study, approximately 70% patients with GIST were symptomatic, 20% asymptomatic and 10% detected at autopsy. GISTs causing symptoms tended to be large with a mean size of 6 cm as opposed to 2 cm asymptomatic GISTs and 1.5 cm GISTs detected at autopsy [Symptoms caused by GISTs are commonly due to their location with both mass effect and intraluminal bleeding being reported. Large GISTs can cause vague abdominal discomfort, pain, bloating, early satiety or increased abdominal girth. Erosion into the gastrointestinal tract can induce significant hemorrhage causing haematemesis, malena or anaemia from occult bleeding. They have also been noted to cause dysphagia in the oesophagus, biliary obstruction around the ampulla of Vater, intussusception or intestinal obstruction in the small bowel. Other rare presentations described in the literature include hypoglycemia , abdominIn this case increased CT enhancement and close proximity to the liver provided a differential diagnosis of a haemangioma, liver cyst or a vascular metastasis. Further vascular investigations were required to make the diagnosis. An extensive literature review has not shown any report of a GIST presenting with an 'intratumour bleed'. This case demonstrates another unusual presentations for this rare mesenchymal tumour.Clinical presentation of these tumours can be varied and gastrointestinal bleeding is the commonest mode described in the literature. The clinician needs to be aware of much more rare presentations of the GIST including an intra tumour bleed. A structured multidisciplinary approach would lead to successful diagnosis and optimal treatment for the patient in these unusual clinical situations.The author(s) declare that they have no competing interests.SKPJ: Conceived the idea, carried out literature review, wrote the manuscript.SB: Contributed by carrying out literature review and drafting the manuscript.RJL: Overall supervision of manuscript preparation and proof reading.ND: Overall supervision of manuscript preparation and proof reading.All authors read and approved the final manuscript.


Malignant arthritis is a rare manifestation of metastatic disease. We describe the case of a previously well 28 year old man in whom hip pain was the presenting symptom of disease. We describe the case and discuss the aetiology of colorectal cancer in young patients. We then review the literature and discuss the investigation and management of malignant joint arthritis.We present the case of a 28 year old man who presented to the emergency department with an acute monoarthritis of the hip. He had an unremarkable past medical history and was systemically well. A diagnosis of malignant joint effusion was reached after a heightened index of clinical suspicion, magnetic resonance imaging and cytological evaluation of the synovial fluid. Computed tomography and bone scan confirmed widespread metastatic disease from a primary colonic adenocarcinoma. The patient tolerated three cycles of oxaliplatin and capecitabine but died 4 months after presentation.The metastatic spread of cancer to the joint and the synovium is one of the rarest manifestations of malignant disease and has not been previously reported as the presenting symptom of disease. The diagnosis is a difficult one to reach and is associated with a poor prognosis. This case illustrates the importance of thorough investigation in reaching this diagnosis and entertaining the possibility in individuals who do not respond to conventional management of acute monoarthritis, even in young patients and individuals who do not display any other symptoms of disease. The usual presenting symptoms are commonly those of altered bowel habit, abdominal pain, bleeding per anum, general malaise or a microcytic anaemia of unexplained aetiology.Colorectal cancer usually presents in patients in their 6We present the unusual case of a young patient with no apparent risk factors or genetic predisposition for colorectal cancer who presented with an acute monoarthritis of the hip as the first symptom of his disease.A 28 year old male student of Nepalese origin living in the United Kingdom for 3 years presented to the Accident and Emergency department with a 3 week history of increasing right hip pain, with associated fevers but no other symptoms and no history of trauma. 5 years previously, he had been treated for pulmonary tuberculosis with a 6 month course of chemotherapy. There was no positive family history. The remaining history was unremarkable.On admission he was partially weight bearing on the affected side. On inspection there were no signs of inflammation. Flexion was limited to100 degrees; extension to 20 degrees; internal and external rotation were reduced to 10 degrees with normal adduction and abduction. There were no further positive findings on the remainder of physical examination or X-ray (figure 9/l), Platelets 378 (150–400 × 109/l), C reactive protein (CRP) 205 (<5 mg/l), Erythrocyte sedimentation rate (ESR) 96 (2–12 mm/1st hour). His biochemistry revealed an alkaline phosphatase (ALP) 342 (30–300 iu/L), bilirubin 15 (3–17 μmol/L), alanine aminotransferase (ALT) 45 (3–35 iu/L), aspartate transaminase (AST) 42 (3–35 iu/L), corrected calcium 2.82 (2.12–2.65 mmol/L), lactate dehydrogenase (LDH) 782 (iu/L). Renal function was normal.Blood results on admission were haemoglobin 12.5 g/dl (12.5–18.0 g/dl), white cell count 6.47  of the pelvis and hips figure  confirmeOur working diagnosis was septic arthritis, with tuberculosis of the hip as a differential, in view of his past medical history. He was thus managed accordingly with a washout and commenced on chemotherapy. Microbiology of the synovial fluid revealed no organisms; auramine tests were repeatedly negative, as was syphilis serology, Human Immunodeficiency Virus (HIV), Hepatitis and melioidosis.There was little clinical response to treatment and inflammatory markers 2 weeks after admission were elevated at a CRP of 130 and ESR of 114.He then developed an episode of diarrhoea and vomiting as an inpatient. Initial investigations for amoeba, cysts, ova, parasites were all negative. Colonoscopy was undertaken and revealed a stricture in the sigmoid colon. Multiple biopsies revealed a moderately to poorly differentiated adenocarcinoma on histological examination. An immunohistochemical panel showed the tissue to be cytokeratin 20 (CK-20) positive, cytokeratin 7 (CK-7) negative and villin negative, thus in keeping with a colorectal primary tumour. Carcinoembryonic antigen (CEA) was measured at 100.7. Genetic screening and microsatellite instability (MSI) testing on the tumour were negative.The synovial fluid was re-examined for cytology following the histological diagnosis and this confirmed a malignant joint effusion.A computerised tomogram (CT) revealed multiple para-aortic lymph nodes, with no evidence of metastases other solid organs. A bone scan figure  revealed2 (day one of cycle) and Capecitabine 1.25 g/m2 (twice daily for fourteen days) for a planned eight cycles (each cycle of twenty-one days). He responded well initially with an improvement symptomatically after 2 cycles of chemotherapy, and objectively on computerised tomogram (CT) of his abdomen and pelvis with reduction in the size and number of significant lymph nodes. However was unable to tolerate more than 3 cycles and died 4 months after his initial presentation.In view of the advanced nature of the adenocarcinoma the primary tumour was not resected and he was commenced on Oxaliplatin 130 mg/mAdenocarcinoma of the colon in young patients is rare with only 2% of diagnosed colonic cancers in patients 40 years or younger . Risk faThe first is familial adenomatous polyposis (FAP); the underlying cause is a gene mutation adenomatous polyposis coli (APC) gene on chromosome 5. This usually has a clear phenotype, characterised by numerous adenomatous polyps in the large bowel by the third decade of life and invariably develop into cancers.The second is hereditary non-polyposis colorectal cancer (HNPCC) syndrome. Here the colorectal cancer also arises from adenomas, but the degree of polyposis is less marked than in FAP. This syndrome has a heterogeneous spectrum, and the phenotype is more difficult to define than in FAP . It is nRegular screening of young individuals for colorectal cancer with positive risk factors with regular colonoscopy and genetic testing for FAP and HNPCC (testing for microsatellite instability) is of grPatients who develop metastatic arthritis secondary to solid tumours are rare. It is even more uncommon for this to be a presenting symptom.A review of the English language literature reveals that there have been 22 reported cases of malignant joint effusion associated with solid organ malignancy -7. AlthoThere have only been three cases reported associated with colon cancer -8 and thThe mechanism by which the primary cancer metastasizes to the synovium has been hypothesised to occur by one of two mechanisms. The first is direct haematogenous spread to the synovium . The secWith a high index of clinical suspicion a diagnosis should be sought by examination of the synovial fluid , howeverTreatment is aimed at symptomatic relief. Radiation therapy has been shown to provide some temporary relief , with anPatients with articular metastasis have a worse prognosis, with an average survival of less than 5 months from the time of diagnosis .The metastatic spread of cancer to the joint and the synovium is one of the rarest manifestations of malignant disease and has not been previously reported as the presenting symptom of disease. The diagnosis is a difficult one to reach and is associated with a poor prognosis. This case illustrates the importance of thorough investigation in reaching this diagnosis and entertaining the possibility in individuals who do not respond to conventional management of acute monoarthritis, even in young patients and individuals who do not display any other symptoms of disease.The author(s) declare that they have no competing interests.NR and HA conceived the reportNR performed the literature search and drafted the manuscript and multiple revisions.CC advised and revised the initial manuscript.All authors read and approved the final manuscript.


Extraskeletal cartilaginous tumors are uncommon. Osteochondromas usually arise from the metaphyseal region of the growing skeleton.A 53 year old man presented with a three years history of anterior knee pain and inability to flex his knee more than 90°. Clinical examination and imaging studies revealed a nodular calcific mass in the anterior portion of the knee, displacing the medial portion of the patellar tendon. Following excision, histopathology confirmed the diagnosis of extra-osseous osteochondroma-like soft tissue mass, with no recurrence 24 months after surgery.An integrated clinical-pathologic diagnosis helps to clarify the nature of extraskeletal cartilaginous tumors that can arise at unusual anatomic site. Complete local surgical excision is the management of choice. Osteochondromas usually develop in relation to the periosteum, and occur around the growth plate of long bones, especially the knee . The tumThe behaviour of extraskeletal osteochondromas is poorly characterised . FurtherWe report a patient with an extra-osseous osteochondroma-like soft tissue mass in the anterior portion of the knee joint. The tumor displaced the patellar tendon and affected knee motion.A 53 year old man presented with a three year history of left anterior knee pain. He was unable to flex his knee more than 90°. He did not report any trauma, and serology did not reveal any metabolic or rheumatic conditions.Clinical examination revealed a firm, nodular mass medial to the patellar tendon. The range of motion of the knee joint was limited to 90° of active flexion, with full extension. Neurovascular examination was normal. Plain radiographs showed a well delineated mass in the anterior portion of the knee. The femoral condyles and tibial plateau were normal Fig. . The masUnder regional anaesthesia with the patient supine in a bloodless field furnished by a tight tourniquet, a 5 cm longitudinal incision was made medially to the patellar tendon. The mass lied medially and behind the patellar tendon in Hoffa body. It was dissected and excised. The medial tibial plateau and the patellar tendon were not involved in the process. The capsule and the skin were sutured with subcuticular undyed 3/0 vicryl  and steristrips . A non adherent dressing, velband and crepe bandage were applied. Weight bearing was allowed in the immediate post-operative period.Post-operative recovery was uneventful. The patient returned to his activities of daily living, and regained full flexion two weeks after the procedure. When last reviewed 24 months after the excision, he was asymptomatic, with no clinical and radiographic signs of recurrence of the lesion Fig. .The firm mass showed a nodular appearance with a diameter of 7 cm with sharply demarcated edges Fig. . MicroscOsteochondromas usually arise from the metaphyseal region of the growing skeleton, with the medulla and cortex of the lesion being continuous with that of the parent bone. Continued growth after skeletal maturation can cause concern given the pre-malignant nature of osteochondromas. Most involve the knee region, although they may develop in any bone that forms by enchondral ossification. Usually growing away from joints, they are not articular lesions ,2,5.Extraskeletal osteochondromas are rare. Usually arising in the juxta-articular soft tissues without attachment to bone, these lesions may be large, and show the clinical and radiological features of a malignant process. Close to 40 extraskeletal osteochondromas not protruding into the joint cavity have been reported as para-articular, soft-tissue, capsular, intracapsular or intra-articular osteochondromas, ossification of the infrapatellar fat pad, and ossifying chondroma ,5-13.Only few intra-articular osteochondromas have involved the anterior and, more rarely, the posterior knee space joint. ,3,14-19.At MRI, there were no irregularities or thickening of the cartilaginous cap greater than 1 cm. Hence, there was no suggestion of malignancy . The borIntraoperatively, the tumor was completely intra-articular, well demarcated, with no evidence of continuity with bone. The gross appearance and histological examination demonstrated the features of an extra-osseous osteochondroma-like soft tissue mass with secondary bone formation in a fairly regular pattern through a process similar to normal enchondral growth . The benSarcomatous degeneration of osteochondral lesions such as synovial chondromatosis may be easier to identify by careful histological examination and correlation with the radiographic and operative findings, but clear histological features indicating the origin of an osteochondroma are not available . Given tMRI is recommended for further characterisation of the nature and extent of an intra-articular osteochondroma. Operative removal is the procedure of choice when function is reduced and the nature of the tumor uncertain. In our patient, the absence of recurrence at follow-up after 24 months confirms the benign nature of the lesion.In conclusion, an integrated clinical-pathologic diagnosis helps to clarify the nature of extraskeletal cartilaginous tumors that can arise at unusual anatomic site. Complete local surgical excision is the management of choice.The pre-publication history for this paper can be accessed here:


Inflammatory myofibroblastic tumours are reported to occur in a variety of sites, including the head and neck, abdominal organs, central nervous system and urinary tract. They only rarely occur in the lung.We report a case of a 25-year-old male admitted with haemoptysis. His chest radiograph showed a peripheral right lung opacity and computed tomography revealed a right lower lobe soft tissue density mass. Bronchoscopy and fine needle aspiration were unhelpful. a diagnosis of pulmonary carcinoma was made, and the patient underwent a right lower lobectomy. On pathology, the tumor was found to be an inflammatory pseudotumor. These lesion are extremely rare, constituting less than 1% of pulmonary malignancies, but are known to occur in young patients. We believe clinicians need to retain an index of suspicion for the presence of this disease in young patients, which can masquerade as more common malignancies. Inflammatory myofibroblastic tumor (IMT) is a rare disease that usually occurs in the lung. It is also known as plasma cell granuloma, inflammatory pseudotumor, xanthogranuloma and fibrous histiocytoma . The notA 25 year-old-male presented with a history of cough with bloody mucoid sputum for the previous two months. He also had anorexia and a history of intermittent upper respiratory tract infections for two years. Clinical examination revealed diminished breath sounds at the right lung base. Laboratory investigation reported normochromic – normocytic anaemia, with low iron serum level and elevated erythrocyte sedimentation rate (76 mm/first hour).His chest radiograph revealed a perbipheral right lung opacity, and computed tomography (CT) demonstrated a right lower lobe soft tissue density mass Figures , 2. EnlaInflammatory myofibroblastic tumor of the lung is rare and its incidence is reported to be 0.04–1% of all pulmonary tumours . PulmonaCommon symptoms include cough, dyspnoea, fever, pleuritic pain and haemoptysis. A significant proportion of cases 30–70%) remain asymptomatic . A histo0% remainPrevious studies have reported anaemia, elevated erythrocyte sedimentation rate, thrombocytosis and polyclonal hypergammaglobulinemia ,9. The fThe radiologic features of inflammatory pseudotumors of the lung have been analyzed by Agrons et al . ComputeNeedle biopsy has been suggested as a feasible approach to the diagnosis . Fine neGross pathology demonstrates that pulmonary inflammatory pseudotumors typically form a well-defined, firm, lobulated parenchymal nodule or mass with a whorled and often heterogeneous appearance on cut section. Histologically, IMT is composed of a variable inflammatory and mesenchymal cellular mixture including plasma cells, histiocytes, lymphocytes and spindle cells. Therefore, depending on the predominant cellular components, many synonyms for this disease have been described. Pettinato et al referred to this entity as IMT because the bulk of the lesion invariably consisted of not specific inflammatory cells but proliferative myofibroblasts and fibroblasts . Most ofThe treatment of choice of inflammatory pseudotumor of the lung is surgery . Wedge rThe prognosis of patients who undergo radical resection is excellent ,7. NeverIn conclusion, IMT is a rare disease with similar characteristics to those of a true tumor. clinicians should retain an index of suspicion for the presence of this disease in young patients with symptoms or chest radiographs which suggest the presence of a malignancy. Surgical resection when possible, is recommended as the treatment of choice with an excellent outcome. Long-term follow up is imperative to detect recurrence.


Abdominal-pelvic masses containing spindle cell and fibroblastic proliferation are very rare. Since scant studies have reported on the pathologic characteristics inherent in this disease, appropriate clinical management is undetermined.We report on an 87 year-old woman who presented with large abdominal pelvic masses, ascites, ureteral obstruction, and an elevated CA-125 serum level. The patient underwent surgical resection of the lesions and has since done very well. Final pathology revealed a low-grade ovarian tumor with spindle cell and fibroblastic proliferation.To the best of our knowledge, this appears to be the only reported clinical case of a patient with this rare histology. Low-grade pelvic masses with spindle cells and fibroblastic proliferation are very rare, poorly recognized, and not well described in the literature . This hiMyxofibrosarcomas and fibromyxoid sarcomas containing spindle cell and fibroblastic proliferation are not well recognized or classified, and primarily are reported in pathology journals ,3,4. ConAn 87 year-old  Caucasian woman was referred to our clinic with a large abdominal pelvic mass, ascites, ureteral obstruction, and a serum CA-125 of 745 U/ML in November 2005. Despite her advanced age, the patient insisted on aggressive management.The patient underwent a laparotomy, abdominal hysterectomy and bilateral salpingo-oophorectomy. She had approximately 150 cc of clear, straw colored ascites. There was a 20 cm intact, largely solid lower abdominal pelvic mass adherent to the mid and distal sigmoid colon mesenteric serosa, which extended down into the cul-de-sac and left pelvic sidewall peritoneal regions. The patient had a preoperative right ureteral obstruction that also appeared to result from extrinsic pressure related to the 20 cm abdominal pelvic mass. Further examination revealed approximately 6 to 7 cm bilateral solid, whitish ovarian tumors involving the serosa of the mid and upper sigmoid colon mesentery. There were no intra- or post-operative complications and estimated blood loss was 500 cc. The patient is currently doing well clinically ten months postoperatively and adjuvant therapy was not indicated.Frozen section of the 20 cm abdominal pelvic mass was consistent with a benign fibroma. Pathology further reported that the ovary was white and enlarged, with a smooth and glistening surface. Cross section revealed a unilocular cyst containing yellow translucent fluid. The remainder of the tumor was solid except for small areas of yellow cystic degeneration. There was no identified necrosis. The immunohistochemical findings are significant in that the tumor is only positive for actin, which is not specific but is typically seen in fibrous or smooth muscle tumors. The findings of trichrome and reticulin positivity also favor a fibroblastic lesion.The case was referred to Stanford Pathology for an independent review. They remarked that the low-grade spindle cell proliferation with fibroblastic features were very rare. The negative immunologic findings exclude more specific markers for smooth muscle, fat tumors, solitary fibrous tumor, hemangiopericytoma, or GIST. They also indicated that these multiple tumors shared the same low-grade appearance and demonstrated a lack of nuclear atypia, increased mitosis, or necrosis. The differential diagnosis included low-grade fibrosarcoma and the solid/low-grade dedifferentiated portion was indicative of an atypical lipomatous tumor Figures  &2.We report the rare status of a patient with low-grade abdominal pelvic tumors exhibiting spindle cell and fibroblastic proliferation. This pathologic subtype is very rare and not well described in the literature but may be similar to a low-grade myxofibrosarcoma or fibromyxoid sarcoma ,3.Antonescu et al. compared the histologic characteristics of low-grade myxofibrosarcomas with fibromyxoid sarcomas . The myxFibromyxoid tumors demonstrating a proliferation of spindle fibroblastic cells are uncommonly identified in the pelvic region and can be incorrectly categorized as either sarcomatoid carcinoma or sarcoma . Koh et The woman in our case study initially presented with multiple abdominal pelvic masses and ascites and underwent surgical resection of her lesions. Although final pathology revealed a low-grade ovarian tumor with spindle cell and fibroblastic proliferation, the multiple masses were suggestive of a neoplasm that could recur or metastasize. We recognize that the tumor histology could be classified as a sarcomatous variant. However, since these lesions are often poorly categorized  and two The proper clinical management for low-grade abdominal pelvic tumors with spindle cell and fibroblastic proliferation should involve surgery and extended follow-up, even if the histology appears benign . We contThe author(s) declare that they have no competing interests.JM originated the study and contributed to the design and drafting of the manuscript. BG compiled the data and significantly participated in the analysis and draft of the manuscript. DE conducted pathologic analysis and review of patient diagnosis. MR and JB significantly contributed to compiling of patient data, analysis, and written manuscript. All authors read and approved the final manuscript.


Epithelioid sarcoma (ES) is an uncommon soft tissue sarcoma. Lately, subtypes of ES, including proximal-type ES have been recognized, with relatively few reports on such cases.A 47-year-old male presented with a perineal soft tissue mass that was excised elsewhere and the biopsy was submitted for a review diagnosis. On histology, a multi nodular tumor was seen comprising sheets of oval to polygonal cells with moderate amount of cytoplasm. Interspersed were larger, rhabdoid cells with abundant eosinophilic cytoplasm and prominent nucleoli. Focal necrosis was noted. A wide panel of immunohistochemical (IHC) markers was performed to rule out a range of differential diagnoses, including a poorly differentiated carcinoma, a melanoma and a variety of sarcomas with epithelioid differentiation. On IHC, the tumor cells showed a polyphenotypic expression, including positivity for epithelial markers i.e cytokeratin (CK), CK7, EMA and mesenchymal markers like vimentin and CD 34. Desmin was focally positive. CK20, CEA, S-100, HMB-45, SMA, LCA and CD31 were negative. A diagnosis of a proximal-type ES was formed. Six moths later, despite adjuvant chemo and radiotherapy (CT and RT), the patient continued to have the lesion and was referred again. In addition to the earlier histological features, sections from the persistent tumor mass showed an increased number of larger cells along with multinucleated tumor giant cells.The value of identifying this uncommon tumor from a list of differential diagnoses is in view of its aggressive behavior, as seen in our case. A wide excision with clear margins is imperative with options of post-operative CT/RT in individual cases during a close follow-up. Epithelioid sarcoma (ES) is an uncommon soft tissue sarcoma, first described as an independent entity by Enzinger . This isA 47-year-old male presented with a gradually increasing, painful soft tissue mass of 7–8 months duration, measuring 4 × 3 cm in the inner side of his right thigh. A computed tomogram (CT) scan revealed an abnormal enhancing lesion in the perianal region. All the visceral organs were found to be normal. No bowel wall thickening, free fluid or abdominal lymphadenopathies were noticed.Subsequently, he underwent a marginal excision for this mass, elsewhere. It was diagnosed as a poorly differentiated carcinoma on biopsy and referred to us in form of a single hematoxylin and eosin (H & E) stained micro section along with a paraffin block for review.At our Hospital, his tumor marker levels for carcino embryogenic antigen (CEA) were normal i.e. 1.8 ng/ml On review histology, a diagnosis of a "proximal-type" epithelioid sarcoma was offered. The patient was recommended a wide excision post a magnetic resonance imaging (MRI) for fear of residual disease. However, he was lost to follow-up.Six moths later, he presented with an enlarging mass at the same location. During this time gap, he revealed a history of having undergone adjuvant chemotherapy (CT) and radiotherapy (RT) that were well tolerated. However, he developed difficulty in walking as a result of the persistent lesion. He underwent a chest X-ray along with CT scan and a real time B-mode high frequency ultrasonographic (USG) examination for the persistent mass that was re-excised and re-submitted to us for review.An oval, hypoechoic, solid lesion measuring 2.9 × 2.2 × 2.1 cms was seen in the deep subcutaneous region of the right, upper, inner thigh up to 5–10 mm superior and anterior to the scar of the earlier excision. Other, two, small oval hypoechoic solid lesions of sizes 10 and 8 mm were seen in the subcutaneous layer. The main lesion showed an ill-defined echogenic wall with peripheral echogenic linear echo entering the lesion, suggestive for a lymph node. Eccentric cortical parenchyma showed a distorted architecture. The overlying skin was normal with slightly prominent subcutaneous fat lobules. No significant flow lesion was seen in the color Doppler. Radiologically, the differential diagnoses included a tumor mass vs a lymph node mass.  it is mostly seen in the distal extremities (commonly hand) of young adults. On histomorphology, it displays a typical "granuloma-like" pattern of tumor cells surrounding a necrotic focus .Lately, a non-conventional subtype of ES has been identified with a differing clinical profile and a polyphenotypic differentiation, termed as the "proximal-type" ES. It is more frequently seen in the pelvis, perineum and genital tract of young to middle aged adults. On histology, it usually lacks the conventional "granuloma-like appearance". In addition to presence of oval to polygonal cells, it shows characteristic large cells with 'rhabdoid' traits .The present case was identified as a perineal mass in a middle-aged male. The outside slide on review revealed a multi nodular tumor comprising oval to polygonal cells arranged predominantly in cohesive sheet like pattern. Interspersed were large cells with abundant cytoplasm, vesicular nuclear chromatin and prominent nucleoli. A wide panel of IHC makers was performed to sort out a diagnosis from an equally wide range of differentials, including metastasis from a poorly differentiated carcinoma, a rhabdomyosarcoma, a melanoma, an epithelioid leiomyosarcoma, an epithelioid angiosarcoma, an epithelioid malignant peripheral nerve sheath tumor (MPNST), an epithelioid gastrointestinal stromal tumor (GIST), a conventional ES, an anaplastic large cell lymphoma (ALCL), a synovial sarcoma, an extra-renal rhabdoid tumor and a mesothelioma. In the clinico-radiologic metastatic work-up, no definite mass was identified in the solid organs as well as in the gastrointestinal (GI) tract. In addition, CEA levels were found to be normal. No glandular or squamoid areas were seen in the tumor. Further, on IHC, lack of CK20 and CEA expression made a possibility of a metastatic adenocarcinoma from the GI tract as less likely. CD 34 positivity further substantiated a diagnosis of an ES over a carcinoma, as well as a synovial sarcoma, the latter which is known to express epithelial and mesenchymal markers ,6. AparthSNF5/IN11 on chromosome 22q11.2 [An interesting observation in the tumor was a 'pseudoangiosarcomatous' pattern, as has been noted in some cases by Guillou et al . Lack of 22q11.2 . Similar 22q11.2 . It has  22q11.2 . There h 22q11.2 , who repThe importance of identifying this subtype lies in its aggressive behavior was seen in our patient who, despite adjuvant CT/RT did not get rid of the local lesion. Although, fortunately he did not develop any metastatic lesions in view of the adjuvant treatment, the persistence of the tumor suggests a possibility of unclear margins of the first excision that was performed elsewhere.Certain prognostic factors, including tumor size have been recognized in proximal-type ES . CurrentThe importance of identifying this tumor relates to its aggressive behavior. A range of differentials needs to be kept and ruled out on the basis of the clinical profile, morphology and a wide panel of relevant IHC markers. A timely diagnosis, optimal treatment in form of a wide-excision with clear margins and a close-follow up can be helpful in anticipating recurrences and a fatal outcome that can be seen with this aggressive tumor.The author(s) declare that they have no competing interests.BR: Involved in the diagnosis of the case; design, preparation and drafting of the manuscript.BDG: Collected references, clinical and follow-up details.RFC: Overall supervision and has given the final approval of the manuscript.


Spindle cell tumors of the larynx are rare. In some cases, the dignity is difficult to determine. We report two cases of laryngeal spindle cell tumors.Case 1 is a spindle cell carcinoma (SPC) in a 55 year-old male patient and case 2 an inflammatory myofibroblastic tumor (IMT) in a 34 year-old female patient. A comprehensive morphological and immunohistochemical analysis was done. Both tumors arose at the vocal folds. Magnified laryngoscopy showed polypoid tumors. After resection, conventional histological investigation revealed spindle cell lesions with similar morphology. We found ulceration, mild atypia, and myxoid stroma. Before immunohistochemistry, the dignity was uncertain. Immunohistochemical investigations led to diagnosis of two distinct tumors with different biological behaviour. Both expressed vimentin. Furthermore, the SPC was positive for pan-cytokeratin AE1/3, CK5/6, and smooth-muscle actin, whereas the IMT reacted with antibodies against ALK-1, and EMA. The proliferation (Ki67) was up to 80% in SPC and 10% in IMT. Other stainings with antibodies against p53, p21, Cyclin D1, or Rb did not result in additional information. After resection, the patient with SPC is free of disease for seven months. The IMT recurred three months after first surgery, but no relapses were found eight months after resurgery.Differential diagnosis can be difficult without immunohistochemistry. Therefore, a comprehensive morphological and immunohistochemical analysis is necessary, but markers of cell cycle (apart from the assessment of proliferation) do not help. The most common type of malignant laryngeal tumors is the classical squamous cell carcinoma (SCC). Benign tumors of the larnyx are divided in two groups: mesenchymal and epithelial lesions. The latter harbours particular papillomas, whereas simple vocal cord polyps, Reinke edema, or e.g. leiomyomas have a mesenchymal origin. A further group are tumorous inflammatory lesions, such as granulomatous polyps. Most of these different entities show a characteristic histomorphology, so that the diagnosis might be unproblematic for the histopathologist.Spindle cell lesions of the larynx are rare 1.3%) [.3% [1]. We demonstrate a spindle cell carcinoma (SPC) and an inflammatory myofibroblastic tumor (IMT), two laryngeal spindle cell tumors with complete different dignity, and discuss the differential diagnosis focusing on the immunohistochemical results.A 55 year-old male patient with relapsing dyspnoe and five pneumonias within the last four years was referred to our ENT hospital with progressive dyspnoe and dysphonia for five months. The patient was a smoker with 30 pack years, no alcohol abuse. He did not show any other systemic symptoms. Flexible transnasal laryngoscopy showed a laryngeal mass without visible glottis. Subsequently, microlaryngoscopy with laser resection of the ulcerated tumor (diameter 3 cm) was performed. The tumor originated from the right vocal fold. Histologically, a spindle cell carcinoma (SPC) was diagnosed. In a second surgery no remnants were found. The cervical lymph nodes were unsuspicious in ultrasound and computertomographic investigation. Therefore, neck dissection was not carried out. The patient is free of disease seven months after surgery.A 34 year-old female patient with increasing dysphonia for one month was referred to our ENT hospital. She had neither a history of smoking nor alcohol abuse. Magnified laryngoscopy showed a polyp (0.8 cm) of the right vocal fold. Examination was followed by microlaryngoscopy with macroscopically complete resection. In a postoperative control ten days after surgery, a small nodule was found and suspected as a granulomatous polyp. Logopedic therapy led to a subjective voice improvement within the next three months. However, further magnified laryngoscopy showed an increasing size of the nodule. The following resection of a round tumor with 1.2 cm diameter was macroscopically and histologically complete. Eight months after surgery the patient is free of disease.After surgical resection, routinely processed paraffin blocks were cut at 2 μm and put on 3-aminopropyltriethoxysilane (APES) coated slides. Sections were first stained with hematoxylin-eosin (HE) and periodic acid Schiff (PAS) reaction.Cuts for immunohistochemistry were air-dried over night, dewaxed, rehydrated in descending concentrations of ethanol before being heated for antigen unmasking in 10 mM citric acid (pH 5.5) for five minutes. After rinsing with distilled water, slides were washed in phosphate buffered saline (PBS). For staining, the Histostain-Plus bulk kit (Zymed) was used according to the manufacturer's protocol: 15 min blocking reagent, primary antibody incubation for one hour, rinsing with PBS (pH 7.4), biotinylated secondary antibody incubation for 20 minutes, rinsing with PBS, streptavidin peroxidase 20 minutes, and rinsing with PBS. Staining was performed by adding 3,3'-diaminobenzidine  with subsequent counterstaining using haemalaun. The antibodies, sources, and dilutions used are indicated in table All stainings are used in routine investigations and the antibodies were tested in suitable positive controls.spindle cell carcinoma was diagnosed. Together with the clinical information, the tumor stage was pT2, cN0, cM0, and (after resurgery) R0.In HE staining, an ulcerated spindle cell tumor with widely myxoid stroma changes was found. Tumor cells were arranged in an irregular pattern with different cell density figure . No PAS inflammatory myofibroblastic tumor, immunohistochemistry revealed an expression of ALK-1 , the second an inflammatory myofibroblastic tumor (IMT). Because of similar morphology, only comprehensive immunohistochemical investigations allowed the correct final diagnoses and the aligned assessment of biological behaviour.th and 7th decade are predisposed  [According to the recent WHO definition, laryngeal SPCs represent biphasic tumors, composed of a squamous cell carcinoma (SCC), either in situ or invasive, and a malignant spindle cell component with mesenchymal appearance , but of is 10:1) ,5. The vis 10:1) . Some SPis 10:1) , but a sis 10:1) . Five yeis 10:1) .The differential diagnosis includes, apart from rare laryngeal sarcomas, reactive or benign spindle cell proliferations, such as nodular fasciitis, IMT, or low grade myofibroblastic sarcoma.IMTs are neoplasms with borderline dignity . They arThe etiology of IMTs is unknown. The idea of a reactive lesion was refuted by genetic studies. The clonal origin is evident ,14 and cClinically, IMT can mimic a neoplastic process. Patients suffer from hoarseness, dysphonia, or foreign body sensations in the troath. Constitutional or systemic signs  are usually missing in extrapulmonary IMTs. Systemic alterations are only reported in one laryngeal IMT .The diagnosis of IMTs can be difficult due to the wide morphological spectrum. Coffin et al. have described three morphologic patterns:1. spindle cells in a myxoid background with a vascular and inflammatory component (nodular fasciitis like)2. compact spindle cells in a solid confluent area or as irregular foci in areas of dense collagen (fibrous histiocytoma like)3. collagen dense pattern similar to desmoid fibromatosis .Our case of IMT first presented as variant one with relapse as variant two. Alaani et al. described in a relapsed IMT a progressive diminution of inflammatory cells, reduction of atypia and more collagenous, less myxoid stroma up to osteoblastic changes . A transAccording to Coffin et al., immunohistochemical investigations could reveal an expression of vimentin in 99%, sm-actin in 92%, focally desmin in 69%, cytokeratins in 36%, CD68 in 24%, and CD30 in 6% . The repThe differential diagnosis of IMT comprises low grade myofibroblastic sarcomas as well as a long list of benign, reactive, or neoplastic spindle cell lesions, such as leiomyoma, solitary fibrous tumor, spindle cell carcinoma, nodular fasciitis, and peripheral nerve sheet tumor . Morpholth/6th decade) [As mentioned above, SPC is also an important differential diagnosis. But if the diagnosis cannot be made on the basis of conventional histomorphology, it becomes even more difficult. Typically, SPCs contain pleomorphic malignant spindle cells with mitoses . Most of them are associated with epithelial dysplasia or common SCC . Our cas decade) ,29. TherHussong et al. described an overexpression of p53 in one of six IMT with recurrence and in one of two with malignant transformation, but in none without . Even ifIn summary, the differential diagnosis of SPC and IMT can be difficult, particularly in cases with uncommon immunohistochemical profile. Therefore, a comprehensive morphological and immunohistochemical analysis is necessary, but markers of cell cycle (apart from assessment of proliferation) do not help.The author(s) declare that they have no competing interests.HUV and MS drafted the manuscript, HUV, SH, PS, and ME evaluated the immunohistochemical stainings and confirmed the diagnoses, MS and RH compiled the clinical data, HKM and ME mainly contributed to the discussion. All authors read and approved the final manuscript.


Interferon-beta has been reported to suppress MGMT in an experimental glioma model. Here we report a patient with TMZ-refractory anaplastic astrocytoma (AA) who was treated successfully with a combination of interferon-beta and TMZ.Malignant gliomas recur even after extensive surgery and chemo-radiotherapy. Although a relatively novel chemotherapeutic agent, temozolomide (TMZ), has demonstrated promising activity against recurrent glioma, the effects last only a few months and drug resistance develops thereafter in most cases. Induction of O6 international units/body, followed by 5 consecutive days of 200 mg/m2 TMZ in cycles of 28 days. After the second cycle the tumor decreased in size by 50% (PR). The tumor showed further shrinkage after 8 months and the patient's KPS improved from 70% to 100%. The immunohistochemical study of the initial tumor specimen confirmed positive MGMT protein expression.A patient with recurrent AA after radiation-chemotherapy and stereotactic radiotherapy was treated with TMZ. After 6 cycles, the tumor became refractory to TMZ, and the patient was treated with interferon-beta at 3 × 10It is considered that interferon-beta pre-administration increased the TMZ sensitivity of the glioma, which had been refractory to TMZ monotherapy. Since surgery or local radiotherapy can be applied only to limited patients, a more systemic approach such as chemotherapy is used in most cases. Until recently, however, chemotherapy has had only a limited impact for control of recurrent glioma. A relatively novel chemotherapeutic agent, temozolomide (TMZ), has demonstrated promising activity against recurrent glioma in some patients, however the effects last only a few months and drug resistance develops thereafter in most cases,2. Resishe tumor . Interfehe tumor ,5. Here A 51-year-old man was found to have a diffusely infiltrative tumor in the bilateral frontal lobe and right thalamus Figure . The pat2 for the first 5 days, escalated to 200 mg/m2 in the following cycles. Although the TMZ chemotherapy seemed to have some effect, the tumor continued to grow. After 6 cycles, the patient developed dysarthria and hemiparesis, and Karnofsky performance status (KPS) decreased from 100% to 70%. From the clinical course, the serial MRI findings /body interferon-beta (Feron®) was given intravenously followed by 5 days of 200 mg/m2 TMZ (Days 2 – 6). To differentiate radiation necrosis from recurrence, a fluorodeoxy glucose (FDG) PET study was performed. As the FDG-PET findings strongly suggested recurrence, TMZ chemotherapy was started. The patient was treated with the usual 5-day protocol repeated in cycles every 28 days, i.e. TMZ 150 mg/ms Figure  and the The patient's neurological symptoms improved after this first cycle, and MRI after the second cycle showed shrinkage of the tumor Figure . The patThe MGMT protein expression of the tumor specimen taken at the initial surgery was performed using immunohistochemical method as described previously . BrieflyMore than 40% of the tumor cells were positive for this staining thus it is confirmed that this tumor moderatelyexpressedWe have reported a case of TMZ-refractory glioma that was treated successfully with interferon-beta and TMZ combination therapy. Although TMZ is one of the most effective chemotherapeutic agents for treatment of glioma recurrence, not all patients benefit from the drug. The response rate of glioblastoma (GBM) or AA to the standard TMZ protocol of 5 days in a 28-day cycle is 7–30% ,2,7,8. Tin vivo and in vitro [8 IU/body is far less than that needed for adequate control. However, this dose may allow attainment of a serum concentration about one fourth of that shown to suppress MGMT activity in an in vitro setting [Interferon-beta has been used in Japan as an adjuvant therapy for some patients with malignant glioma . It has in vitro ,5. We adThe MRI enhancement after stereotactic radiotherapy might have been due to a radiation effect. MRI is often useless for differentiating recurrence from radiation necrosis , and falThis is the first clinical report of effective treatment using a combination of interferon-beta and TMZ against TMZ-refractory glioma. This combination warrants further testing in a larger-scale clinical study.Combined treatment with interferon-beta and TMZ achieved a response in a case of TMZ-refractory recurrent AA. This approach may be a useful salvage therapy for patients with recurrent malignant glioma.The author(s) declare that they have no competing interests.TF: Involved in the diagnosis of the case, design of the treatment, submission of the protocol to the IRB, and drafting of the manuscriptHI: Involved in the treatment of the caseAM: Performed immunohistochemical study of the surgical specimenHA: Involved in the surgery of the caseTN: Involved in the treatment of the case, and submission of the protocol to the IRB


Clear cell sarcoma of tendons and aponeuroses (CCSTA) appears usually in the extremities and rarely in the trunk.We present an unusual case of CCSTA overlying the scapular region and with secondary osseous extension in the lower scapula. The patient underwent a wide local excision with removal of the tumor and the lower two thirds of the scapula. He had no local recurrences but he developed lung metastases after 5 months in spite of postoperative chemotherapy. He finally died ten months later.The patients with CCSTA have a variable unpredictable course. Despite treatment the overall prognosis is poor. Clear cell sarcoma of tendons and aponeuroses (CCSTA), first described by Enzinger in 1965. It has A 61-year-old man presented with a painful mass overlying the lower left scapula of four months duration. His pain deteriorated the last three weeks and was accompanied by limitation of the range of motion of the left glenohumeral joint.Physical examination revealed a subcutaneous tender uniformly firm mass in the lower left scapular region measuring 4.5 × 4.5 cm in diameter. There were no signs of superficial skin inflammation. The patient had not a previous history of melanoma and/or other skin tumors.Radiological examination showed an osteolytic lesion in the lower left scapula while bone scanning was positive with increased uptake in the same area. CT scans and MRI images of the thorax and shoulder showed a soft tissue mass 45 × 45 mm localized in the lower scapular region between infraspinatus and teres minor muscles with bone invasion of the lower part of the scapula Figure , 2, 3. TThe open biopsy specimen consisted of a rubbery, homogenous, white tissue with no attachment to the skin, implying deep location of the tumor. There were compact nests and fascicles with large rounded and spindle-shaped cells separated by connective tissue septa. The large rounded cells had pleomorphic nuclei and large amounts of clear cytoplasm while the spindle-shaped cells had palely staining eosinophilic cytoplasm. Mitoses were moderately numerous. There were areas of necrosis and hemorrhage and tumor spreading into the surrounding muscles and scapula. Immunohistochemical stains were performed with negative staining for CD68, actin and desmin antigens and with positive staining for tumor markers S-100 protein, HMB-45, NSE, EMA, cytokeratins and myosin. Histochemical stains for melanin presence were negative. Pathologic findings were compatible with a CCSTA with osseous extension into the lower scapula  consisting of ifosfamide, vincristine and epirubicin. The patient had no local recurrences but he developed lung metastases after 5 months. Despite chemotherapy, he died 10 months later with progressive lung disease.CCSTA is an aggressive rare soft-tissue tumor with approximately 300 cases totally reported in the English literature . In our Commonly the presenting symptom is a painless mass or swelling. Pain and/or tenderness above the tumor site may be found in 33–50% of all the patients suffered from CCSTA. Typically CCSTA has the appearance of a palpable deep-located soft tissue mass bound to an adjacent tendon or aponeurosis. The overlying skin may occasionally appear with discoloration ,5. IntraFrequently it arises in the extremities with a predilection for the lower limbs (78–97%). Both foot and ankle are the commonest sites of tumor appearance accounting 33–65% of all cases. Next most common sites are the knee, thigh, hand, forearm, elbow and shoulder in descending order of frequency; rarely the tumor arises in the head, neck or trunk ,11.et al [Primary CCSTA overlying the scapular region, as in our case, is very uncommon with only a few case reports have been described ,12. ThisUsually CCSTA has the appearance of a lobular or multinodular, circumscribed or encapsulated mass with a gray-white appearance on cut surface. Hemorrhage and necrosis may be present in large tumors. Intralesional calcifications are rarely seen . HistoloImmunohistochemically, the tumor cells are usually positive for S-100 protein, HMB-45 and vimentin and/or microphthalmia transcription factor . CytokerCCSTA is associated with chromosomal abnormalities consisting of changes in chromosome number and chromosome rearrangements ,14,16. CDistinction between CCSTA and metastatic malignant melanoma may have diagnostic difficulties. Both tumors have similar histological features. However tumor presentation  and histological features help to make a differential diagnosis between these tumors . Both CCThe treatment of choice for patients with CCSTA is wide excision of the tumor or amputation with excision of regional lymph nodes. Adjuvant radiotherapy and/or aggressive multiagent chemotherapy seem to be ineffective but probably could be given -5,21,22.The patients with CCSTA have a variable unpredictable course. Despite treatment the overall prognosis is poor and subsequent wide spread dissemination of disease lead patients with CCSTA to death. The 5-year survival rate with radical excision and adjuvant chemotherapy and/or radiotherapy ranged from 60 to 67% and the 10-year survival rate is about 33% ,5,10,11.Usually there is a median interval time between the primary treatment and first local recurrence ranged from 6 months to 1 year ,12. TherThe most common sites of metastases are the regional lymph nodes and the lungs and less common skin, bones, liver, heart and brain ,5,11. 60The author(s) declare that they have no competing interests.CJK: Preparation of manuscript, operation, VGG: Preparation of manuscript, operation, collection of clinical data and review of the literature, AG: Collection of data, carried out the pathologic findings DJV: Proofreading of manuscript, operation, collection of clinical data; ES: carried out the pathologic findings, proofreading of manuscriptAll Authors read and approved the final manuscript.


Peritoneal carcinomatosis from a gastrointestinal carcinoid tumour is rare and the long-term management and prognosis have not been clearly defined. The natural history is different from gastrointestinal adenocarcinoma, although its capacity to invade regional lymph nodes and generate distal metastasis can make the management more complex. Whilst the development of carcinomatosis is uncommonly reported, it may be higher than expected.A 63 years-old woman underwent emergency surgery in 1993 for right iliac fossa pain and a mass that was found to be an ileal carcinoid tumour. Over the next ten years, further surgery was required for disseminated disease with peritoneal carcinomatosis and liver metastasis. Systemic chemotherapy had little effect, although Somatostatin was used effectively to relieve symptoms caused by the disseminated disease (flushing and diarrhoea).Peritoneal carcinomatosis from carcinoid tumours is not well documented in the literature. Aggressive surgery must be performed in order to control the disease since chemotherapy has not been reported to be effective. With repeated surgery long-term survival can be achieved in these patients. Intestinal carcinoid tumours can be found in all parts of the gastrointestinal tract, with the highest incidence being in the distal ileum. They present as a spectrum from small 'benign' tumours to the classical 'carcinoid syndrome' of local tumour invasion with liver metastases and systemic symptoms. However, compared to adenocarcinoma, they have a better prognosis and long-term survival is reported. This case reports peritoneal dissemination of the carcinoid tumour and a long follow up of the disease.A 63-year old woman presented in 1993 with a short history of right iliac fossa pain, localised peritonitis, and an elevated white cell count. She reported a two-year history of intermittent colicky abdominal pain. At operation, a 5 cm ileal tumour was found, situated 10 cm proximal from the ileocaecal valve. This was resected. Histopathological assessment confirmed a carcinoid tumour with locally involved nodes (three positive lymph nodes). Nine years later, she re-presented with right-sided abdominal pain, a two-week history of diarrhoea, and a two month history of episodes of her face getting "hot and blushing". A computed tomography (CT) scan revealed a liver nodule (30 mm diameter) and a mass (60 × 40 mm) in the right iliac fossa, which was in contact with a small intestinal loop Figure . In addiAt operation in January 2003, peritoneal carcinomatosis was discovered with tumoural implants over the greater omentum, the parietal peritoneum, the left ovary, the terminal ileum (with stenotic and fibrous lesion), and a liver metastasis in segment IV. The peritoneal deposits, left ovary and fallopian tube, the involved distal ileum, and the liver metastasis were surgically removed. All deposits were histologically metastases from the original carcinoid tumour, although the distal ileal tumour metastasis only affected the serosal and muscular layers. Following an uneventful postoperative recovery, she commenced six cycles of chemotherapy with Streptozotocine and 5-fluorouracil.In October 2004, she was admitted with abdominal cramps and vomiting. A CT scan revealed dilated intestinal loops that appeared to be attached to each other and with the anterior abdominal wall. In view of the previous surgery and the history of carcinomatosis, she underwent a further laparotomy. However, at operation, the cause was adhesional obstruction, although some foci were found in the parietal peritoneum and in the Pouch of Douglas. These implants were less than 4 mm in length and were excised. All the biopsies were positive for carcinoid tumour and after an uneventful recovery she was discharged. Further therapy with Somatostatin with the aim to control the progression of the disease and because she reported occasional flushing, achieved a good quality of life. At review in March of 2006, the patient reported some abdominal pain and infrequent flushing, but is otherwise well. A further Octreotide scan revealed no disease activity.The first reported case of a carcinoid tumour was published in 1888, but the molecular basis of the syndrome was not elucidated until 1954 and the following year the presence of serotonin was confirmed .There is no globally accepted classification for carcinoid tumours although most series and reports are based upon the embryologic origin of the organ and its blood supply. Hence, they can be divided into; foregut , midgut , and hindgut  carcinoid tumours -4. DepenThe incidence of carcinoid tumours is reported to be 0.5 – 8.4 cases per 100,000, although they have been reported to be present in 1% of autopsies ,7. The pThe symptoms of intestinal carcinoid tumour are non-specific and vague, generally consisting of periodical abdominal pain as a major symptom, along with intestinal obstruction, intermittent diarrhoea, and gastrointestinal bleeding ,5,10. InCarcinoids are usually discovered during laparotomy for intestinal obstruction or pain, and the existence of multiple primary tumours is remarkable in 26–28% of cases ,12. The With hepatic metastasis, the ten-year survival is reported to be 20%, however, recent study shows 5-year survival between the groups with non-aggressive and aggressive liver resection to be 25% versus 72% ,14. The Peritoneal carcinomatosis from carcinoid tumours has been scarcely reported in the form of a limited number of case reports. Recently, in a series of 116 cases of intestinal neuroendocrine tumours , thirty In another large series of 111 endocrine carcinomas that were surgically treated , 81%  declare that they have no competing interests.GG: Documented and prepared the draft.IRD: Revised, edited most of the manuscript and helped in preparing the draft.AG: Surgeon who performed the operations and helped in preparing the manuscript specially in describe the findings and the follow-up.JMR: Literature search, revision of bibliography and helped with the editing of the manuscript.All authors read and approved the final manuscript for publication


The term melanosis in the gastrointestinal tract refers to the accumulation of pigment deposits in the mucosa. Melanosis of the colon is not uncommon and has been associated with certain conditions, however melanosis of the small intestine is extremely rare. Herein, we describe a case in which we observed melanosis not only in the colon, but in the terminal ileum as well, associated with the use of anthraceneline laxatives. The clinical significance of this condition is not clear, however Gastroenterologists and Pathologists should be aware of its existence. The term melanosis coli was initially proposed by Virchow . "MelanoA 74-year-old man presented complaining of abdominal cramps and bloating. He had a long history of constipation and extensive use of anthraceneline laxatives. Endoscopic examination followed with no remarkable findings and multiple biopsies were taken and sent to the Pathology laboratory for histological evaluation. On microscopic examination, abundant pigment-containing macrophages were noted at the lamina propria of the colon biopsies Figure . InteresMelanosis of the colon is not uncommon and has been associated with the ingestion of anthraceneline laxatives, although it can be observed in patients without such history . MelanosThe author(s) declare that they have no competing interests.AB, JP and NJA have been involved in conceiving the manuscript. AB and JP have been involved in the literature search and in drafting the manuscript. All authors read and approved the final manuscript.


Mediastinal pancreatic pseudocyst is a rare complication of acute or chronic pancreatitis.This case report describes the management of a difficult case of pancreatic pseudocyst with a mediastinal extension in a patient having chronic pancreatitis. Different management strategies were used until complete resolution of this complex pseudocyst occurred using open surgical cystogastrostomy.Despite the availablity of different minimally invasive techniques to treat pancreatic pseudocysts, management of complex mediastinal pseudocyst may still require open surgical drainage procedures. Mediastinal pancreatic pseudocyst is a rare complication of acute or chronic pancreatitis. Since its first description in 1951, approximately 50 cases have been reported in the world literature. We report a case of mediastinal pseudocyst associated with alcoholic pancreatitis successfully treated with cystogastrostomy.A 22-year-old female patient with a known history of chronic alcohol abuse was referred to hepato-biliary unit with recurrent upper abdominal related to chronic pancreatitis. Prior to this she has had multiple hospital admissions in previous one year with similar symptoms. In the past, ultrasonography had excluded gallstones and a CT scan 12 months ago had shown normal-looking pancreas. Her most notable symptomatic episode was 4 months ago when she was admitted with left lateral chest pain and dyspnoea. A CT-scan although excluded a pulmonary embolism but incidentally revealed a left gastric artery pseudoaneurysm  declare that they have no competing interests.All the authors have been involved in literature search, writing and final reviewing of this manuscript.


Retroperitoneal cystadenocarcinomas are rare lesions, the majority of cases presented as one-patient reports.We present two cases of retroperitoneal cystadenocarcinoma, both in women of reproductive age: one with aggressive behavior, and the remaining case, with a more indolent clinical evolution.One case presented as pelvic tumor, was treated with surgical resection of the disease, but manifested with recurrent disease a few months later despite use of chemotherapy. The second case involved a patient with diagnosis of abdominal tumor; during laparotomy, a retroperitoneal tumor was found and was totally removed. At follow-up, the patient is disease-free with no other treatment.The behavior and treatment of retroperitoneal cystadenocarcinoma are controversial. We suggest aggressive surgery including radical hysterectomy and bilateral salpingoopherectomy with adjuvant chemotherapy in these cases. Retroperitoneal primary cystadenocarcinomas are extremely rare entities . At presIn the majority of instances, preoperative diagnosis of these lesions is not possible because computed tomography (CT) scans or magnetic resonance imaging (MRI) is not able to distinguish the exact origin of the lesion. The most common presumptive diagnosis at surgery is abdominal mass.Due to its rarity, optimal treatment options, survival, and exact prognosis continue to be uncertain. We present two cases of primary retroperitoneal mucinous cystadenocarcinomas treated at a single institution; these had different clinical presentations and totally different outcomes with the most adequate treatment for each case according to clinical presentation. To the best of our knowledge, these are the first of such cases reported in Latin-American women.A 36-year-old Hispanic woman had an appendicitis-associated appendectomy at 6 years of age, and post-Cesarean section uterine atony-related total abdominal hysterectomy at age 26 years. Other medical, surgical, or family histories were unremarkable.The patient was seen with a 6-month history of abdominal distention and discomfort. At physical examination, no abdominal masses or ascites were detected. A pelvic ultrasound (US) was performed, and an adnexal cystic mass of 19 × 16 × 12 cm with solid component was observed in the area of the left adnexa. There was no ascites or any other abnormalities observed in the pelvic cavity. Serum CA-125 was 4.13 U/ml, and routine preoperative laboratory tests were obtained and reported as normal, as was the chest x-ray. A CT scan was conducted as part of the preoperative evaluation; the scan confirmed the US findings and revealed solid areas in a cystic tumor Figures  and 1b.2. CA-125 was obtained after the fifth chemotherapy course and was reported as 5.3 U/ml. A pelvic US was performed 8 months later, and a new pelvic mass measuring 7 × 6 × 6 cm at the same site was found. An exploratory laparotomy was conducted and a sigmoid resection was required due to a mass involving this. Infracolic omentectomy was also carried out due to multiple nodules. The surgery was considered optimal cytoreduction with no visible lesion remaining. The Pathology report stated mucinous cystadenocarcinoma.An exploratory laparotomy was performed with the diagnosis of a suspicious malignant adenexal mass. During the procedure, a tumor measuring 19 × 16 × 12 cm was found in the left retroperitoneal space, the tumor completely separated from the ovaries or other intrapelvic structures. Complete and intact removal of the lesion was obtained; this was sent for frozen section, and was subsequently reported as mucinous cystadenocarcinoma. At the time of the surgery, both ovaries and fallopian tubes were grossly normal, as were all intra-abdominal structures. The patient's postoperative course showed no complications or adverse events. Six courses of adjuvant chemotherapy were administered with carboplatinum at a dose of 6 area under the curve (AUC) and paclitaxel at 175 mgs/mSecond-line chemotherapy was initiated with oral etoposide (50 mg daily for 21 days). After one cycle, disease progression was identified and the patient decided to stop chemotherapy. Tamoxifen (20 mg/day) was instituted as palliative management.On gross examination, an ovoid, well-defined tumor with measurements of 19 × 16 × 12 cm with smooth grey surface was received. Internal surface comprised a multilocular mass with thin walls and mucinous material only, while a small area exhibited solid nodules in the wall Figure  and areaThe recurrent tumor's surgical specimen comprised a multiorgan pelvic resection with an ill-defined white mass with gross infiltration to muscle and fatty tissue of the pelvis wall, left ovary, fallopian tube, omentum, and in wall of the colon, without lesion in the mucosa. Microscopically, high-grade adenocarcinoma similar to high-grade areas of the previous lesion was identified, the ovary demonstrating direct infiltration from the abdominal mass.The patient, a 21-year-old female with no remarkable previous medical or surgical history complained of diffuse abdominal discomfort 1 month prior to the patient's presentation at the Emergency Service due to acute abdominal pain and intestinal occlusion-compatible clinical data. Abdominal and pelvic US was performed, revealing a cystic mass with solid areas Figure . An emerSystemic adjuvant chemotherapy was proposed; nonetheless, the patient refused this treatment. Therefore, close follow-up was advised, and the patient has been seen at the Medical Oncology Service over the last 6 months with serial CA-125 serum levels at latest measurement of 13.3 U/ml. An abdominal CT was performed, and no evidence of disease was found.Grossly, the tumor was a well-defined multilobular mass of 26 × 18 cm. Cut surface exhibited a multicystic tumor with thin walls and solid white areas. Microscopically, the lesion was a mucinous tumor with well-differentiated mucous glands with cribiform or papillary architecture, and cells had pseudo-stratified, large regular nuclei with mucinous cytoplasm, these areas demonstrating transition to cystic areas lined by a single line of mucinous cells without atypia. No capsular invasion was identified.et al., the first group in 1977[Retroperitoneal tumors of epithelial origin are rare, because no epithelial cells are found in this area; nevertheless, cases of lesions with Müllerian-type epithelium have been reported, Roth p in 1977. The exap in 1977. Anotherp in 1977,4. Anothp in 1977. Other aAge-at-presentation ranges from teenaged to elderly patients, and the most common complaint at presentation has been abdominal discomfort and a slow-growing pelvic or abdominal mass ,6. In onet al., in that the authors were solely able to find two reports in the literature with suspicious preoperative retroperitoneal tumor in their review of 16 cases from 1966 to the report in 2005 [Preoperative diagnosis is rarely suspected because of the non-specific symptoms and the scarce aid of imaging examinations. Although US, CT scan, and MRI clearly detect cystic masses in ovarian or pelvic organs, diagnosis of retroperitoneal tumor is extremely difficult. This was mentioned by Matsubara  in 2005 . AlthougTumor markers are not very helpful in differentiating the exact origin of the lesion, because CA-125, CA19-9 may or may not be elevated and may or may not have increased values in other neoplasms such as epithelial ovarian tumor (serous or mucinous). On the other hand, tumor markers help in detecting a recurrent tumor, as in ovarian cancer or colon cancer .et al., [et al., [et al., [et al., who advocate for laparoscopic excision of the tumor and complete evaluation of abdominal and pelvic organs, sparing fertility in these women [It is clear that surgical treatment of these tumors is the cornerstone, but the question remains: how extensive must the surgery be? Everyone agrees on total removal of the lesion, but some authors such as Gotoh et al.,  recommen[et al.,  recommen[et al.,  recommen[et al., . The onlse women .et al. [Adjuvant chemotherapy is not a standard treatment and is beneficial solely in cases in which the tumor has been ruptured during surgery , or whenet al. , who hadet al. ,12?The answers of these questions must wait for the moment until we can determine whether the patient is at risk for recurrence or not, and further study is needed to establish optimal-treatment protocols on these rare neoplasms. We are in favor of aggressive adjuvant chemotherapy with hysterectomy and bilateral oophorectomy only when macroscopic involvement of these structures is found during the procedure.The author(s) declare that they have no competing interests.DCL – Contributed to manuscript conception, writing, organizing, drafting and revision of the manuscript.DPM – Reporting pathologist, involved in discussion leading to manuscript preparation and revision.JCHV – Reporting pathologist, contribution on images of both cases, critical review of manuscript.ADG – Acquisition of consent, patient clinical managed and critical revision of manuscript.VVV – Follow up of patients, collection of data and revision of the manuscript.GZC – Follow up, collection of data and revision of manuscript.All authors have read and approved the final manuscript.


Mucinous cystic neoplasm (MCN) of the pancreas is basically cystic epithelial neoplasm, unilocular or multilocular, occurring almost exclusively in women.A 51-year-old female presented with a pancreatic mass incidentally found on the abdominal computed tomography. She underwent distal pancreatectomy. The sectioned surface of the pancreas revealed a circumscribed, whitish gray ovoid firm mass with some cystic spaces. Microscopically, glandular or small cystic structures were scattered in the predominant stroma creating a solid appearance. The subepithelial stromal component was composed of cytologically bland looking spindle cells, which resembled ovarian stroma. The stromal cells were reactive to CD 34, vimentin, progesterone receptor and calretinin. The microscopy was consistent with mucinous cystadenoma of the pancreas.This case of mucinous cystadenoma of the pancreas showed very interesting pathology: It was solid rather than cystic, and accompanied by abundant benign transitional epithelia, which was a very unusual and novel finding in the mucinous cystic neoplasm of the pancreas. Mucinous cystic neoplasm (MCN) of the pancreas is a cystic neoplasm, unilocular or multilocular, occurring almost exclusively in women. The overwhelming majority of cases occur in the body-tail of the pancreas. MCNs show two distinct components: an inner mucinous epithelial layer and an outer dense cellular ovarian-type stromal layer -5.The case reported here is an exceptional case of MCN of the pancreas with predominant stromal component creating a solid tumor, and abundant transitional cell differentiation.A 51-year-old female presented with a pancreatic mass found incidentally on the abdominal computed tomography for routine health examination. The mass was located in the body of the pancreas and was ill-defined with faint inhomogeneous low density at both the arterial and the venous phases of computerized tomography (CT) scan , high molecular weight cytokeratin , cytokeratin 7 , cytokeratin 20 , calretinin , estrogen receptor , progesterone receptor , chromogranin A , CD34 , vimentin , α-smooth muscle actin , desmin , S-100 protein , CD99 , CD99 , c-kit . Both mucinous and transitional epithelia were diffusely immunoreactive to pancytokeratin and cytokeratin 7, but not reactive to cytokeratin 20 except for goblet cells. Transitional epithelia were diffusely reactive to high molecular weight cytokeratin Figure , whereasMCNs of the pancreas are defined by cystic epithelial neoplasms composed of columnar mucin-producing epithelium. This tumor occurs almost exclusively in women and shows no communication with the pancreatic duct system. According to the grade of dysplasia, tumors may be classified as adenoma, borderline and non-invasive or invasive carcinoma -5.The present tumor was composed of mucin producing epithelium with an ovarian type stroma. The stroma was predominant overgrowing epithelial element and creating a solid tumor. From this point of view, the main differential diagnoses of the present case include MCN with sarcomatous stroma, benign mesenchymal tumors and solid-pseudopapillary neoplasm. MCN with sarcomatous stroma is a rare variant of MCNs associated with a malignant sarcomatous stroma. This sarcomatous stroma is extremely hypercellular, contains mitotic figures, and shows marked atypia and pleomorphism of stromal cells . Unlike The epithelial component of MCN is composed of columnar cells which can also reveal pseudopyloric, gastric foveolar, small and large intestinal, and squamous differentiation, as is also observed in ovarian MCN -4. In thThe stromal component of MCN is composed of ovarian-type stroma which express vimentin and in a high proportion, progesterone receptor, estrogen receptor, calretinin, and alpha inhibin ,13-15. CThis case is a pancreatic tumor showing very interesting and unusual pathology. According to the current WHO classification of pancreas, this case belongs to the mucinous cystadenoma. But this case was solid rather than cystic, and showed abundant benign transitional epithelia, which are very unusual and a novel finding that has not been described in MCNs of the pancreas. This histopathology is very similar to the mixed mucinous cystadenoma and benign Brenner tumor of the ovary ,12.The author(s) declares that she has no competing interests.WAL performed the pathologic examination, researched the relevant literature and prepared the manuscript.This research was conducted by the research funds of Dankook University in 2004.


Hemangiopericytoma is a rare mesenchymal neoplasm, accounting for about 1% of vascular tumors The tumor occurs most commonly in the skin, subcutaneous soft tissues, muscles of the extremities, retroperitoneum but rarely in the lung, trachea or mediastinum.A rare case of primary mediastinal hemangiopericytoma is presented. A 72-year-old woman was treated by complete surgical resection of the tumor. Details of the clinical and radiographic feature are presented. The patient's postoperative course was uneventful with no evidence of recurrence 9 months after the operation.Hemangiopericytoma is an uncommon, potentially malignant tumor originating from pericytes in the small vessels and surgical radical excision is the treatment of choice, although the criteria for determining the area of resection have not been established. International literature has demonstrated that recurrent disease usually occurs within 2 years and therefore a long-term careful follow-up is required. Hemangiopericytoma is a rare mesenchymal neoplasm, accounting for about 1% of vascular tumors . The tumA 72-year-old woman was referred to our institution complaining of dyspnea, cough and chest tightness for the last two months. Physical examination indicated tachypnea, orthopnea and use of accessory respiratory muscles. Laboratory studies were essentially within normal limits. A chest X-ray revealed a homogenous opacity occupying lower two-thirds of the left hemithorax and causing contralateral shifting of the mediastinum and heart Figure . Thoraceth intercostal space. During the operation, the mass appeared to have mediastinal origin and was highly vascularized. There was no involvement of vital mediastinal structures and the surrounding lung parenchyma was compressed by the tumor. Six hundred ml of sanguinous pleural fluid were aspirated. Despite the persistent bleeding during dissection the tumor was resected. The mass measured 7 × 3 × 2.5 cm and weighed 210 g. Grossly the resected specimen was a smooth, friable encapsulated mass with focal hemorrhages. The cut surface was smooth, elastic and pale brown. Microscopic examination showed round and spindle cells surrounded by thin-walled, endothelium-lined vascular channels, giving a "staghorn" appearance to the vessels as typically seen in hemangiopericytoma  declare that they have no competing interests.AC, NB, AE took part in the care of the patient and contributed equally in carrying out the medical literature search and preparation of the manuscript. IF participated in the care of the patient and had the supervision of this report. All authors approved the final manuscript.


Inflammatory myofibroblastic tumor (IMT) is a neoplasm of unknown etiology occurring at various sites. By definition, it is composed of spindle cells (myofibroblasts) with variable inflammatory component, hence the name is IMT.The present case is of a 46 years old woman presented with a history of flank pain, abdominal mass and intermittent hematuria for last 6 months. The initial diagnosis was kept as renal cell carcinoma. Finally, it turned out to be a case of retroperitoneal IMT. The patient was managed by complete surgical resection of the tumor.IMT is a rare neoplasm of uncertain biological potential. Complete surgical resection remains the mainstay of the treatment. Inflammatory myofibroblastic tumor (IMT) is a relatively rare neoplasm. The outlook of this disease has changed with time from a benign reactive process to a malignant neoplasm, based on the multiple case reports demonstrating recurrent and constant clonal genetic alterations -5. ThereA 46-years-old woman presented with history of flank pain, abdominal lump and intermittent hematuria of 6 months duration. She was diagnosed as renal cell carcinoma (RCC) elsewhere and referred. The investigations at our hospital revealed normal hematological and biochemical parameters. The urine microscopy showed deformed RBC. The computerized tomography (CT) scan of the abdomen showed a large irregular well defined heterogeneous lesion occupying the left hypochondrium, lumbar and supra umbilical regions, measuring approximately 16 × 13.6 × 12.1 cm, and showed presence of predominant cystic portions with no obvious calcifications . It has long been debated regarding the origin of IMT whether it was truly neoplastic or a post inflammatory process. The proposed etiologies included Epstein Barr virus (EBV), Human herpes virus (HHV8), and over expression of interleukin IL-6. Though other diseases like Kaposi's sarcoma and Castleman's diseases also have similar etiologies, molecular transcription form of open reading frame (ORF) -16, K13, 72 expressed in IMT are not expressed in the aforesaid diseases . MoreoveIt is more common in children without sex predilection, though extra pulmonary forms are more common in adult females. The patients often present with fever of unknown origin and other vague nonspecific symptoms. Usually it has a benign course and in most of the cases it is a slow growing, locally confined tumor with less metastatic potential. However, there are some predictors for aggressive behavior and metastatic potential of IMT which include presence of ganglion like cells, cellular atypia, aneuploidy, and p53 over expression ,12.The tumor commonly occurs in lung. Extra pulmonary IMT are rare. In the largest series of 84 cases of extra pulmonary IMT, only four retroperitoneal IMT were reported  The medlThough IMT will not make its place in the common differential diagnosis of retroperitoneal masses, it should be kept in mind as one of the possibilities. Typically the IMT is characterized by the expression of vimentin, smooth muscle actin, and cytokeratins, corresponded to that of myofibroblasts along with other inflammatory markers . In the The author(s) declare that they have no competing interests.SVSA have been involved in drafting the manuscript & revising it critically for important intellectual contentRCC acquisition of data, or analysis and interpretation of dataHKD revising it critically for important intellectual contentPPB revising it critically for important intellectual content and given final approval of the version to be publishedCRR interpretation of dataGA have been involved in drafting the manuscript & revising it critically for important intellectual content