Context. Leiomyosarcomas are rare malignant smooth muscle tumors that may arise in any organ or tissue that contains smooth muscle, commonly within the gastrointestinal tract. They are most often found in the stomach, large and small intestines, and retroperitoneum. Primary pancreatic leiomyosarcoma is extremely rare, and to the best of our knowledge only 30 cases have been reported in the world literature since 1951. Our case represents the first to have a clear origin from the main pancreatic duct. Case Report. This case was diagnosed in a large, tertiary care center in Tampa, Florida. Pertinent information was obtained from chart review and interdepartmental collaboration. A mass in the tail of the pancreas was identified with large pleomorphic and spindle-shaped cells. Immunohistochemistry for vimentin, smooth muscle actin, and desmin was positive. All remaining immunohistochemical markers performed were negative. The tumor clearly originated from the pancreatic duct wall, filled and expanded the duct lumen, and was covered with a layer of benign biliary epithelium. Conclusion. Leiomyosarcoma of the pancreas is an extremely rare malignancy with few reported cases in the literature. The prognosis is poor, and treatment consists of alleviating symptoms and pain management. To our knowledge, this represents the first reported case demonstrating clear origin of a leiomyosarcoma from the pancreatic duct. Leiomyosarcomas are rare malignant tumors of smooth muscle origin that may arise in any organ or tissue that contains smooth muscle and comprise less than 1% of all cancers and 2%–9% of sarcomas [An 83-year-old woman presented with a 3-month history of left-sided abdominal pain and weight loss. Her history was pertinent for a total abdominal hysterectomy with bilateral salpingo-oophorectomy in 1963 for benign leiomyomas with dysmenorrhea, a cholecystectomy in 1978 for cholelithiasis, and a hernia repair in 1993. No mass lesion was identified on physical examination. Abdominal CT scan and MRI revealed an 8 cm mass within the tail of the pancreas with no evidence of invasion into adjacent structures. At this time the presumed diagnosis was pancreatic adenocarcinoma. Ultrasound-guided biopsy of the lesion was attempted but yielded no diagnostic tissue. A distal pancreatectomy with splenectomy was performed and revealed an intrapancreatic tan, nodular mass measuring 8.5 × 7.0 × 6.2 cm with focal hemorrhagic areas suggestive of necrosis. The margins were grossly free of tumor. Histological examination showed large pleomorphic and spindle-shaped cells, focal necrosis, and a mitotic count of > 20 per 10 high power fields, with negative surgical margins Figures and 2. MuPrimary pancreatic sarcomas of any type are rare although many have been reported in the literature including fibromyxoid sarcoma, follicular dendritic cell sarcoma, Kaposi's sarcoma, leiomyosarcoma, fibrosarcoma, liposarcoma, angiosarcoma, and rhabdomyosarcoma –14. EpidLeiomyosarcomas are malignant smooth muscle neoplasms that can arise in any anatomic location containing smooth muscle, most commonly the stomach, but can be found anywhere along the gastrointestinal tract, as well as the uterus and retroperitoneum. They have also been reported in the skin, bladder, ovaries, salivary glands, larynx, gallbladder, adrenal glands, broad ligament, diaphragm, breast, vulva, penis, scrotum and testis, and, of course, the pancreas –37. LeioLeiomyosarcoma of the pancreas is an extremely rare malignancy with few reported cases in the literature. The prognosis is poor, and treatment consists of surgical resection with chemotherapy and/or radiation for alleviating symptoms and pain management. To our knowledge, this represents the first reported case demonstrating clear origin of a leiomyosarcoma from the pancreatic duct.


Herein is reported the case of a mantle cell lymphoma (MCL) with synchronous double intestinal location. A 74 - year old male presented with mild abdominal pain. CT scan imaging indicated invasion of lateral intestinal cavity by large mass formation. Exploratory laparotomy was performed and two solid extra-mural masses were isolated and excised. Histology revealed non- polypoid double synchronous lymphoma of mantle cell origin, an unusual presentation of the disease. It isollicles . A transollicles . Mantle ollicles ,2.At the time of diagnosis most patients have signs of multiple lymphatic involvement, including spleen, red bone marrow, cervical lymph nodes, liver, and gastrointestinal tract, usually under a condition known as "multiple small intestine polyps" -8. MCL cA 74- year-old Caucasian male Greek patient, presented with mild abdominal pain and a history of recurrent gastrointestinal bleedings over the last few years.At the time of admission at the hospital his general state was not indicative for an emergent situation. The patient complained for insisting abdominal discomfort, moderate flatulence and anorexia. Physical examination revealed abdominal distension with flat sounds at percussion, moderately decreased intestinal sounds, without signs of localised sensitivity or peritoneal irritation.3) with inverted cellular type , moderate decrease of Hematocrit and Hemoglobin  whereas Platelet count was noticeably elevated at 662.000 cells/mm3. Biochemistry was within normal levels, as well as coagulation time exams, except for elevated CRP count (at 45 mg/L) and decreased Albumin/Globulin ratio. Tumour marker CA 125 was also increased .Blood tests demonstrated normal WBC count  administered in a single day every 3 weeks for 6 cycles. In addition to chemotherapy the patient received treatment with monoclonal antibody Rituximab which acts against CD20, a special molecule on the surface of B-cell Non-Hodgkin Lymphomas.A complete physical and imaging evaluation 21 months later revealed complete recovery and no signs of remaining disease.Mantle cell lymphoma is an entity of B- cell malignancies, belonging in the non- Hodgkin lymphomas ,2,6. IntPrognosis for intestinal malignant lymphoma is poor due to its accelerated proliferation and the non- specific clinical presentation of the disease. Most of the patients have advanced stages of the disease at the time of diagnosis, being also delayed by the difficulty in direct visualization of small intestine. Patients with obstructive tumour masses require surgical resection.Chemotherapy is the treatment of choice, including regimens with (1) Cyclophosphamide, Hydroxydaunorubicin, Oncovin, Prednisone or Prednisolone (CHOP), (2) Cyclophosphamide, Vincristine, and Prednisone (COP), and (3) Doxorubicin, Tteniposide, Cyclophosphamide, and Prednisolone (AVmCP). Reports after application of AVmCP regimen, reach response rate of 80%, with a 5- year survival rate of 59%. Less aggressive regimens, such as COP, appear to be less effective, with a response rate of 30% and a mortality rate of 100% 3 years after treatment ,9.The case of extra- luminal augmentation of the tumour is extremely seldom and double synchronous presentation of such masses even rarer. Few cases are reported of synchronous colonic tubular presentations ,11. In nNon-polypoid lymphomas of the small bowel can be a rare place of occurrence for mantle cell lymphomas. In such cases of lymphomas presenting as small bowel individual tumors, with single or multiple location, the treatment of choice is surgical resection with appropriate anastomotic procedures. Following surgery, additional treatment with chemotherapy is also proposed, according to the classification of malignancy.MCL: mantle cell lymphoma; CT: computed tomography; WBC: white blood cells; CRP: c-reactive protein; MLP: multiple lymphomatous polyposis;Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the journal's Editor-in-Chief.The authors declare that they have no competing interests.SN, AK, EN have had an equally substantial contribution to the clinical diagnosis, surgical management and post-op follow-up of the patient. DM and PT analysed the specimen and confirmed the diagnosis, MSC and SN drafted the manuscript. SN and EN are guarantors of the paper. All authors read and approved the final manuscript.


Case 1: A 60-year-old man had a 2 cm carcinoid of the stomach and underwent partial resection. Involvement of the muscularis propria and lymph nodes metastasis were observed microscopically. Follow-up gastroscopy revealed another carcinoid lesion and total gastrectomy was performed. Case 2: A 67-year-old woman with multiple carcinoids of the entire stomach underwent antrectomy. No growth of residual tumors has been detected so far. Case 3: A 61-year-old man had a tumor near the esophagogastric junction and underwent total gastrectomy. Carcinoid component was diffusely intermingled with adenocarcinoma in the tumor and invaded into the subserosa. In all 3 cases, the serum gastrin level was high and atrophic gastritis was microscopically observed. Carcinoid tumor in Case 3 was different from those in Cases 1 and 2 and interestingly, gastric carcinoid with hypergastrinemia showed various types of appearance.We report 3 cases of gastric carcinoids with hypergastrinemia.  Gastric carcinoids originate from the foregut and are derived from enterochromaffin-like (ECL) cells, which are the main neuroendocrine cells in the gastric mucosa. Gastrin acts directly on ECL cells to induce hyperplasia, dysplasia, and, eventually, neoplasia or carcinoid . In patiA 60-year-old man, who suffered from cerebral palsy due to measles encephalopathy at the age of 6 months, became unable to eat and underwent gastroscopy. A round tumor was detected at the anterior wall of the middle gastric body, and biopsy revealed a carcinoid . No otheA 67-year-old woman experienced epigastric pain and nausea for the past 2 months. Gastroscopy revealed lots of elevated lesions  in the whole stomach , and theA 61-year-old man was asymptomatic except for severe anemia (Hb: 4.5 g/dl) that was detected at a medical checkup. Gastroscopy revealed a large gastric tumor near the esophagogastric junction , and bioA gastric carcinoid is a relatively rare neoplasm. Previously, carcinoids have been classified according to the embryologic origin ; a gastric carcinoid originates from the foregut. Recently, carcinoids have been categorized according to their pathobiological behaviors into 3 different types . Type 1 A type 1 gastric carcinoid is usually small, multifocal, and located in the gastric fundus with a low potential of malignancy . It growThere seems to be some differences in the characteristics of type 1 gastric carcinoids based on Japanese and Western reports. The incidence of gastric carcinoids is 2.4%–8.7%  among alIn Japan, total gastrectomy with lymph node dissection has been the preferred surgical procedure for multiple gastric carcinoids associated with type A gastritis. The merit of this procedure is that the fundic gland area, which could be the origin of the carcinoid and ECM, is completely resected along with excision of the majority of G cells. Okada et al. also suggested that total gastrectomy should be considered as one of the treatments for multiple gastric carcinoids with hypergastrinemia . In caseSome authors have recommended a conservative approach without removal of the tumors. Hosokawa et al. proposed a follow-up program without surgical resection for multiple gastric carcinoids that are associated with type A gastritis . They reIn the present study, we observed atrophic gastritis in all 3 cases. The development of adenocarcinoma or endocrine tumors in atrophic gastritis is widely documented . In caseWe showed 2 cases of multiple gastric carcinoids and 1 case of a carcinoid-adenocarcinoma tumor. All 3 cases were diagnosed with atrophic gastritis, which is associated with an increased risk of gastric carcinoma. We could have selected antrectomy with wedge resection of the large tumor for case 1 and we chose it for case 2. As shown in the previous studies, larger (>10 mm in diameter) or deeper infiltrating (than submucosa) tumors may, although rarely, be associated with regional lymph node metastasis,  a progno


Thorough examination with computerized tomography and magnetic resonance imaging revealed a hemangioma extending to the posterior third of the vertebral body, compressing the spinal cord at the level of 8th thoracic vertebra. A percutaneous vertebroplasty was performed. The post-operative computerized tomography scan demonstrated cement leakage. After thorough cement removal combined with extensive decompression and posterior stabilization, the patient reported gradual improvement of his symptoms and was able to return successfully to his work a few months later.We present the case of a 50-year-old male with consistent back pain, not resolving with conservative treatment. Plain radiograms demonstrated a lytic lesion at the level of the 8 Hemangiomas represent benign tumors, originally described by Virchow in 1867. These common lesions, found in 10% to 12% of autopsy specimens, are defined as vascular spaces lined with endothelial cells. They are usually detected in the fourth or fifth decade of life and are twice as likely to be present in women as in men [Microscopically, hemangiomas are distinguished as: capillary, cavernous, arteriovenous and venous with the first two groups being the most prevalent in osseous tissue. Clinically, they are subdivided into three main subcategories: the most common asymptomatic hemangioma, the compressive vertebral hemangioma and the rare symptomatic hemangioma .Diagnosis is mainly based on CT and MRI findings. In CT-scans, hemangioma is presented with a characteristic “polka dot” appearance, while in MRI it is detectable in both T1 and T2 sequences, with T2 being the most diagnostic .We describe the case of a 50-year-old Greek Caucasian male with a free medical history, who visited his primary care physician for back pain. Since initial conservative treatment did not resolve his symptoms, he was referred to our department.On examination, he complained of consistent back pain. Clinical examination did not reveal increased sensitivity or any neurological deficit. Plain radiograms and CT scan of the thoracic spine demonstrated a hemangioma at the level of T8 vertebra  and 2.On a following examination, the new CT scan indicated a fracture of T8 while MRI revealed an unspecific image of the paravertebral space, the fracture of the T8 vertebra and spinal stenosis at the same level  and 4.In order to retain vertebral height and relieve our patient from his symptoms, a percutaneous vertebroplasty at the level of T8 was performed . Post opHemangiomas represent benign, silent lesions that usually remain undetected. Symptoms arise only when spinal cord compression occurs.Treatment of such symptomatic lesions includes several options. Embolization, radiotherapy and ethanol perfusion of the hemangioma present good results and can be used therapeutically to reduce the vascularity of the region and diminish bleeding. Laminectomy can also be performed, in case of spinal compression . PercutaHochegger et al.  reportedThese serious complications have troubled most of the surgeons dealing with the technique. In an effort to decrease the possible risk of spinal canal compression, Wenger M et al.  proposedNevertheless, in our case despite of the use of special cement, the anteriorly placed needles, the initial injection of small amount of cement and the continuous radiological evaluation, a cement leakage occurred.Percutaneous vertebroplasty is a well documented treatment option for asymptomatic hemangiomas. Nevertheless, the possibility of cement leakage should always be taken into consideration when dealing with such a minimal invasive technique and extreme consciousness and continuous radiographic evaluation are mandatory in order to be able to detect cement leakage and proceed to immediate decompression and cement removal.


Insulinoma is the most common endocrine tumor of the pancreas. Accurate preoperative detection and localization of insulinomas is essential for the appropriate selection of candidates for surgery. We present two cases with benign pancreatic insulinoma.Preoperative evaluation for patients with suspected insulinomas has been controversial. Endoscopic ultrasonography (EUS) has a sensitivity of 95% in well skilled operators and well tolerated preoperative imaging method.We have detected the insulinomas with EUS before surgery in our patients but other imaging modalities did not help us for localization of them. Patients have been asymptomatic postoperatively with no hypoglycemia on repeat fasting. We reviewed here the different modalities for preoperative localization of insulinoma. Insulinoma is a neuroendocrine tumour derived mainly from the pancreatic islet cells producing excessive amounts of insulin. Insulinoma is the most common form of the pancreas islet cell tumors (60%). It can be seen at every age but mostly seen in females above age 50 years old ,2. NinetIn cases that were clinically suspected for insulinoma, C-peptide suppression test or prolonged supervised fast tests are used for diagnosis. In these cases, suspected tumor localization has been done with different laboratory and scanning procedures. Because they are small tumors the sensitivity of transabdominal ultrasonography (TAUSG) and abdominal computerized tomography (CT) scanning are low (between 23–63% and 40–73% respectively). Angiography and transhepatic venography have higher sensitivity rates (60–98%) but they are expensive and invasive procedures [2, initial glucose level was 48 mg/dl (70–11 mg/dl), plasma insulin level was 16 μIU/ml (5–25 μIU/ml), C-peptide level was 2.7 ng/ml (1.6–3.6 ng/ml). Prolonged supervised fast test was applied and at the sixteenth hour of the test hypoglycemic symptoms were occurred. Simultaneously taken blood samples showed the glucose 23 mg/dl, plasma insulin 12.7 μIU/ml, insulin/glucose ratio was 0.52. The TAUSG and CT scan showed no pathology. In the EUS scanning at the pancreas uncinate process, there was a hypoechoic 13 mm nodular lesion  was 27 kg/mmg/dl 70–1 mg/dl, 2, initial glucose level was 41 mg/dl (70-1-mg/dl), plasma insulin level was 17 μIU/ml (5–25 μIU/ml), C-peptide level was 12.9 ng/ml (1.6–3.6 ng/ml). Prolonged supervised fast test was applied and at the sixth hour of the test hypoglycemic symptoms were occurred. Simultaneously taken blood samples showed the glucose 28 mg/dl, plasma insulin 28.1 μIU/ml, insulin/glucose ratio was 1. The TAUSG and CT scan showed no pathology. In the EUS scanning at the pancreas uncinate process, there was a hypoechoic 11 mm nodular lesion  was 24 kg/mDiagnosis of insulinoma may delay because symptoms are nonspecific. Sometimes neuroglycopenic symptoms are chiefly seen in patients and these groups of patient are misdiagnosed as epilepsy or neuropsychiatric disease. In patients with insulinoma, diplopia, sweating, palpitation is seen at a rate of 85%; confusion or abnormal behavior 80%; unconsciousness and amnesia 53% and epilepsy 12% [It is diagnosed with hypoglycemic symptoms and hypoglycemia and hyperinsulinism simultaneously. If the blood glucose level is under 40 mg/dl and the insulin level is over 6 μIU/ml, it is diagnosed as hypoglycemia due to hyperinsulinism . If it i80–90% of insulinomas are solid and benign. However they localize on corpus and head mostly, they rarely localize ectopically on pancreas 5–10% of cases are with multiple endocrine neoplasia type I (MEN I) and in this cases the adenomas are generally multiple . One of A USG, CT, EUS, percutaneous transhepatic portal vein sampling (PTPVS), selective intra-arterial pancreatic stimulation (SIAPS), and also somatostatin receptor scintigraphy (SRS) have been used for tumor localization. But any of the procedures have enough sensitivity. Some of the investigators believe that when insulinoma diagnose clinically and biochemically there is no need to preoperative imaging because most of the lesions were located on pancreas and dimensions were small and also IOUSG and palpation procedures were enough for localization . The locThe sensitivity of USG on insulinoma patients were about 29 – 63% because of small dimensions of the tumor and also the fatness of patients as well. In both cases which we underwent, there was no adenoma on pancreas showed in TAUSG, CT is a kind of technique that can determine tumors which are smaller than 1 cm and it is told its' sensitivity is about 40–73% also [Our two cases were underwent CT. Although one of the adenomas diameter was 15 mm – that can be measured by CT – no adenomas were showed in CT anyway. Tumors can be determined with well skilled operators 80 – 90% by EUS . This meThe localization of adenoma can be determined by measuring the hormones from the branches of portal veins in PTPVS. The diagnostic value is about 60–98%. One of the other techniques is SIAPS. The technique can be explained measuring of hormone from synchronize venous catheter after injection calcium to arterials which are feeding pancreas. Diagnostic value is 90% . NeitherIOUSG's sensitivity is about 75–90%. IOUSG and palpation are complementary techniques. Gouyo et al reported that they have diagnosed the tumors 62.5% with inspection and 96.4% with palpation and also they have reported that 20 of 21 patients diagnosed by IOUSG . IOUSG mSurgery is recommended therapy in insulinomas as the tumors are often soliter and related to benign adenomas. For small and benign characteristic adenomas enucleation is enough and distal pancreatectomy may be suitable for the lesions which are located on the corpus and on the tail of the pancreas. Distal pancreatectomy is suggested for the lesions which can not diagnose by palpation and IOUSG. Total pancreatectomy is suggested for the malign, multiple and large tumors. But medical treatment is suggested when there is contraindication for surgery and in uncontrolled preoperative hypoglycemia. Streptozocin, Diazoxide, Verapamil, Pheytoin, somatostatin can be used for the medical treatment .Both of our cases underwent enucleation. Adenomas have been detected in histopathologic analysis. And both cases were underwent 50 μg (tid) subcutan octreotide treatment.When EUS is applied by well skilled operators, it can help the chosen operation type as well as there will be no need invasive diagnostic methods. And also intraoperative palpation must be done carefully. However insulinomas localization were pancreas and the tail part of pancreas, they can be diagnosed any other parts of pancreas during the operation. So palpation is much important in these situations. Our findings indicate that a diagnosis can be suggested by biochemical evaluation, clinical symptoms and by EUS.If there is only one small adenoma enucleation is the most effective method. In this situation complications like diabetes mellitus and exocrine pancreas function disorders can be seen so rarely.Written informed consent was obtained from the patients for publication of this case reports and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.EUS: Endoscopic ultrasonography; TAUSG: Transabdominal ultrasonography; CT: Computerized tomography; IOUSG: Intraoperative ultrasonography; BMI: Body mass index; MRI: Magnetic resonance imaging; PTPVS: Percutaneous transhepatic portal vein sampling; SIAPS: Selective intra-arterial pancreatic stimulation; SRS: Somatostatin receptor scintigraphy.The authors declare that they have no competing interests.MO carried out the patient's diagnosis, drafted the manuscript. MFY performed the cases management, drafted the manuscript. EC, BC and MO participated in the patient's management. MFY participated in the writing of the case report. All authors read and approved the final manuscript.


In the last decade, several sophisticated and accurate imaging methods such as positron emission tomography have been developed in order to evaluate malignant potential in enlarged mediastinal lymph nodes. This case illustrates an unusual presentation of sarcoidosis that mimicked lymphatic metastases of non small cell lung carcinoma. The reported high specificity and sensitivity of positron emission tomography-Computer Tomography regarding mediastinal staging could lead in same cases of false positives to a delaying of stage adapted therapy of non small cell lung carcinoma, showing that despite the recent advances of imaging techniques, such as positron emission tomography-computer tomography, several limitations of this imaging technique are still existing. Accurate staging of mediastinal and hilar lymph nodes is a critical factor determining operability in patients with NSCLC. Positron emission tomography with has recently been reported to be more effective in detecting tumor involvement in mediastinal and hilar lymph nodes than computed tomography. We report about an interesting case of false-positive PET finding in mediastinal and hilar lymph node staging.An 80-year-old man  with the medical history of Parkinson’s disease consulted his neurologist because of paresthesia in his lower extremities. The patient had 40 pack-years smoking history but had quit 26 years earlier. His medical history showed mitral valve insufficiency grade 2, hypertension and hypercholestererolemia. The social history, family history and review of systems were otherwise unremarkable. The physical examination revealed a generally light reduced state of health, normal vital signs, no fever, with bilaterally clear lung fields and no lymphadenopathy. His routine lab values, including complete blood count and chemistries showed elevated creatinine (1.87 mg/dl) and urea (93 mg/dl) as well as normochromic normocyteric anaemia. After readjustment of medical therapy, chest X-ray was done to exclude a paraneoplastic cause of the above-mentioned symptom. Chest X-ray revealed a solid contrast-enhancing mass in the right upper lobe (RUL). Based on these finding CT-scan was ordered and demonstrated a 1.4 × 3.1 cm pulmonary mass in the lateral segment of the RUL associated with 2.2 cm node in precarinal and 2.4 cm node paraaortal position . A flexiIn recent years several sophisticated and accurate imaging methods have been developed in order to evaluate malignant potential in solitary pulmonary masses associated with enlarged mediastinal lymph nodes.Multiple studies have demonstrated that PET-CT is more reliable as a single test for predicting malignancy in a radiologically undifferentiated nodule and in enlarged mediastinal lymph nodes than CT scan -3. PositThe reported sensitivity and specificity of PET regarding mediastinal staging is reported to be 74-84% and 85-89% respectively with a negative predictive value of 93-95% . On the In our case a whole body PET-CT scan was performed to assess mediastinal nodal disease, and showed high FDG uptake in precarinal, infracarinal, paraaortal and contralateral left main bronchus lymph nodes corresponding to N3 disease . Transbronchial biopsy of a parabronchial lymph nodes and CT-guided needle biopsy of the pulmonary mass showed no evidence of malignant tumor. Afterwards we decided to proceed to thoracotomy in order to assess the high uptake lymph nodes and lesion in the RUL. The lymph nodes found to be positive on PET were discovered to be not malignant by the surgical biopsy. Numerous epitheloid cell granulomas were found in all resected lymph nodes in accordance with sarcoidosis. Pathologic staging resulted in significant downstaging relevant for further therapy, as our patient based on current ATS guidelines would have been a candidate for palliative chemotherapy according to PET staging.Despite of sarcoidosis, false positive PET results have been reported to occur, most notably in association with further active lung diseases such as aspergillomas, active tuberculosis and abscesses . Also faThis case illustrates an unusual presentation of sarcoidosis that mimicked metastatic NSCLC. The high reported specificity and sensitivity of PET-CT regarding mediastinal staging could lead in same cases to a delaying of stage adapted therapy of NSCLC, showing that despite the recent advances of imaging techniques, such as PET-CT, several limitations of this imaging technique are still existing. Histological confirmation of positive PET findings have to remain the golden standard of NSCLC staging.


Neoplasms of the placenta are uncommon. Tumors arising from the placental tissue include two distinct histological types: the benign vascular tumor, chorangioma, and very rarely, choriocarcinoma. Benign leiomyomas, in contrast, are very common tumors of the uterine wall and occur in 0.1% to 12.5% of all pregnant women. However, the incorporation of uterine leiomyoma into the placenta is exceptional and raises the question of its origin. This case is possibly the first report on this kind of a placental tumor which has been examined using both immunohistochemistry and chromosome analysis.A 34-year-old G4P3 Caucasian woman was followed up antenatally because of a stillbirth in her previous pregnancy. At 36 weeks' gestation, a hypoechoic, 3.6 × 4.2 cm rounded mass was noted within the placenta on ultrasound examination. Histologically, the tumor was a benign leiomyoma and this finding was supported by immunohistochemistry. The newborn infant was male. Chromosomes of the neoplasm were studied by the fluorescence in situ hybridization technique and the tumor was found to carry XX chromosomes.A rare benign smooth muscle neoplasm involving the placental parenchyma is presented. The tumor was a uterine leiomyoma of maternal origin, which had become entrapped by the placenta. This case report is of interest to the clinical specialty of obstetrics and gynecology and will advance our knowledge of the etiology of placental tumors. Neoplasms of the placenta are uncommon. Tumors arising from the placental tissue include two distinct histological types: 1) chorangioma, a benign vascular tumor; and (2) choriocarcinoma, which is exceedingly rare. Benign leiomyomas, in contrast, are very common tumors of the uterine wall and occur in 0.1% to 12.5% of all pregnant women . Two. Two9]. Leiomyomas are the most common tumors in women and are found in at least 25% of women in active reproductive life. Leiomyomas are known to be estrogen responsive and may undergo a rapid increase in size during pregnancy. Three theories about the origin of placental leiomyomas have been introduced. First, it may arise from feto-placental mesenchymal tissue. Second, it may represent an intra-endometrial leiomyoma. Third, it may have been a pedunculated uterine leiomyoma incorporated into the placenta during its development and growth.Abdominal or transvaginal high resolution color Doppler ultrasound has recently made it possible to identify the waveforms obtained from intrauterine masses such as uterine leiomyomas. Knowledge of the characteristics of the expected fetal or maternal waveforms is important to differentiate placental tumors from other intrauterine abnormalities. For instance, in cases of undetermined intrauterine tumors, demonstration of the fetal waveforms strongly suggests a chorangioma, while maternal waveforms suggest a uterine myoma .It is interesting that the situation presented in this report is so rare although leiomyomas are known to be very common in fertile years. One explanation could be that submucosal leiomyomas are often associated with impaired fertility due to implantation failure.In our patient, the tumor was not entirely inside the placental parenchyma. In line with other reported cases, the tumor was easily separated from the decidua, which has been thought to support a putative origin from feto-placental tissues. Because the newborn was male, it was possible to study the chromosomes of the tumor to find out whether it was of maternal or feto-placental origin. As in the case of Ernst , the tumThe tumor was a uterine leiomyoma of maternal origin which became entrapped in the placenta and finally lost its attachment to the uterine wall entirely during pregnancy. Uterine leiomyomas can influence the course of pregnancy . In our PEP: polymorphic eruption of pregnancy.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.KM, NH and EP were the major contributors in study concept and design and writing the manuscript. SH has been involved in drafting the manuscript and revising it critically for important intellectual content. NH was responsible for the care of the patient. EP performed the histological examination of the placenta. MK has made substantial contributions to data analysis and performed the chromosome studies of the tumor. All authors read and approved the final manuscript.


Pleomorphic adenomas are the most common benign tumour of the major salivary glands. In addition, they may also occur in the minor salivary glands of the hard and soft palate. Intranasal pleomorphic adenomas are unusual and may be misdiagnosed because they have greater myoepithelial cellularity and fewer myxoid stromata compared to those elsewhere.We present the case of a 61-year-old man who presented with a 2-year history of left nasal obstruction, occasional epistaxis and facial pain. Radiological examination demonstrated well pneumatised paranasal sinuses and a soft tissue mass in the anterior aspect of the left nasal cavity. In this patient, an intranasal approach was used to achieve a wide local resection.Pleomorphic adenomas are rare tumours of the nasal cavity and have been shown to be misdiagnosed in over half of cases leading to more aggressive treatment than is necessary. If unilateral nasal obstruction is the main presenting complaint, we suggest consideration of this diagnosis. In view of the potential for tumour recurrence, long-term follow-up and careful examination of the nose with an endoscope are necessary. Salivary gland tumours constitute about 3%  of all nSeveral benign lesions of the septum such as leiomyoma, osteochondroma and transitional cell papilloma have been reported in literature. The other differential diagnoses may include malignant tumours such as melanoma, adenoid cystic carcinoma and squamous cell carcinoma. The majority of these tumours arise from the mucosa of the bony and cartilaginous septum.Nasoseptal swell body is a discrete area of erectile tissue in the submucosa over the anterior nasal septum. In some individuals, it can present as a suspicious lesion. It does not have a significant relevance when considering the differential diagnosis in this patient given the enormous size of the septal mass. However, in smaller septal swellings, it could be given consideration.A 61-year-old man presented with a 2-year history of left nasal obstruction, occasional epistaxis and facial pain. There was no history of visual defect, atopy or previous trauma to the nose. His weight was stable and his general health was satisfactory.Rigid endoscopy of the nose revealed a grossly deviated septum to the right and a large polypoid mass filling the left nasal cavity. There was no evidence of rhino-sinusitis and his postnasal space was normal. There were no palpable neck nodes.Radiological examination (CT scan) demonstrated well pneumatised para-nasal sinuses and a soft tissue mass in the anterior aspect of the left nasal cavity. This was located anterior to the inferior turbinate and arising from the septum. The smooth surface, preservation of mucosal lining and the localised nature of the mass were consistent with a benign lesion Figure .In this patient, pre-operative incisional biopsy of a smooth, rounded and firm mass arising from the septal mucosa established the diagnosis of a pleomorphic adenoma. A submucous resection was used as an approach to the tumour and as a method of excising the mass with the segment of septal cartilage attached to it. This was deemed necessary during surgery due to evidence of partial thinning of the septal cartilage adjacent to the lesion. A 1 cm margin of normal ipsilateral mucosa and the surrounding perichondrium were also excised. The septal mucosa of the opposite side was preserved.Histological analysis of the tumour confirmed a benign pleomorphic adenoma with no focus of malignant change; the resection margins were clear. The patient was discharged on the same day, and the postoperative course was uneventful. After 4 years, the patient had experienced no further problems with the nasal airway, and repeated nasal endoscopic examination revealed no recurrence of the disease.et al. [The most common tumours of the major salivary glands are pleomorphic adenomas, but in rare instances, they can occur in the respiratory tract . Cases have been reported in the nasal cavity, paranasal sinuses, nasopharynx, oropharynx, hypopharynx, and larynx. In the upper respiratory tract, the most favoured site of origin is the nasal cavity, followed by the maxillary sinus and the nasopharynx . The firet al. ,5.The majority of tumours present between the age of 30 and 60 years and are slightly more common in women. Typical presenting features include unilateral nasal obstruction (71%) and epistaxis (56%). Other signs and symptoms include a mass in the nose, nasal swelling, epiphora, and mucopurulent rhinorrhoea .Pleomorphic adenomas are characterised by epithelial tissue mixed with tissues of myxoid, mucoid or chondroid appearance. Histologically, pleomorphic adenoma of the aerodigestive tract may resemble aggressive epithelial tumours because of the high cellularity and lack of a stromal component Figure . ImportaWide local resection with histological clear margin is generally agreed as the treatment of choice for benign salivary gland tumours. Postoperative radiotherapy has been advocated by some authors in circumstances where residual disease was apparent . In the In their reported series of 40 patients, Compagno and Wong used the lateral rhinotomy approach for excision of tumour in the majority of the patients. Only three patients had a recurrence of disease after 3 years of follow-up. The recurrent lesions constituted more stroma than cellular elements and the former is thought to provide the focus for recurrence [The outlook for intranasal mixed tumours is better than for those in other ectopic sites, because they show early symptoms leading to an early diagnosis. Involvement of the surrounding structures such as bone is rare since the tumours have sufficient space to expand within the nasal cavity .A neoplasm originating from the nasal septum has a higher risk of malignancy compared to other sites in the nose . OccasioIn summary, pleomorphic adenomas are rare tumours of the nasal cavity. They have a higher epithelial and lower stromal component compared to their major salivary gland counterparts and may be misdiagnosed at an early stage leading to more aggressive treatment. We suggest consideration of this diagnosis if the patient has unilateral nasal obstruction or epistaxis as a presenting complaint. In view of the potential for tumour recurrence, long-term follow-up and careful examination of the nose with an endoscope are necessary.CT: computed tomography.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.PG and LM both contributed to conception and design, and carried out the literature research, manuscript preparation and manuscript review. Both authors read and approved the final manuscript.


It has been stated that regulation of the development of the iliac bone is different from that of the ischium and pubis. There are well-known clinical syndromes concerned with hypoplasia of ischiopubic bone, such as small patella syndrome, nail-patella syndrome, ischiopubic-patellar hypoplasia, and ischiopubic hypoplasia.A fit and otherwise healthy 35-year-old woman presented with pain in the left lower limb of 6 months duration. She sought advice from an orthopedic surgeon and was referred for exclusion of a primary soft tissue neoplasm. There was no history of trauma, chronic medical illness or surgical operations. Full systemic examination, laboratory investigations and whole body imaging showed no soft tissue swelling or any other bony defects. Isolated loss of the left inferior pubic ramus and thinning of the superior pubic ramus were detected, raising the question of whether the lesion was a secondary osteolytic lesion, a primary osteolytic lesion or due to endocrine disease.Isolated loss of the inferior pubic ramus with no concomitant bony or soft tissue anomalies is previously unreported. To the best of the author's knowledge, this finding has not been described previously. The development of the pelvic girdles has been poorly investigated and reported evidence suggests that the regulation of ilium development is different from the development of ischium and pubis .An extensive study was carried out to investigate the prenatal development and mineralization of ossification centers in the pelvic bone  using radiography and optical density measurements on human fetuses. The mineral density of the pelvic bone increases with age and the mineralization rate changes throughout fetal life . The hipDelayed ossification of limbs and girdles is an expression of several congenital syndromes and dysplasias, and major morphological abnormalities can arise at any time during the fetal period, with deformation more frequent in the third trimester when the fetus is subjected to greater constraint . There aA fit and otherwise healthy 35-year-old woman presented with pain in the left lower limb of 6 months duration. She had sought advice from an orthopedic surgeon and was referred for exclusion of a primary soft tissue neoplasm. She complained of pain in the left hip joint that worsened with walking and long periods of standing; there was no complaint regarding either knee. She denied any history of trauma, violence or abnormal muscular overload. There was no history of chronic medical illness or surgical operations. There was no family history of congenital defects or similar conditions.On examination she was apparently well-built, fit and otherwise healthy, and full systemic examination with special attention to both breasts and thyroid showed no soft tissue swelling or any other bony defects. There was no evidence of any congenital anomalies especially in the genitalia, hip bones or the long bones of the lower limbs or chest. Laboratory investigations were performed and showed normal organ functioning. Serum calcium and phosphorus and a parathyroid hormone assay were carried out. All were within the normal ranges.Whole body imaging started with plain X-ray films which showed isolated loss of the left inferior pubic ramus and thinning of the superior, with no other bony anomalies Figure . ComputeIsolated loss of the left inferior pubic ramus and thinning of the superior were detected, raising the question of whether the lesion was a secondary osteolytic lesion, a primary osteolytic lesion or due to endocrine disease.Review of human limb malformation syndromes revealed a number of clinical syndromes associated with hypoplasia of ischiopubic bone: small patella syndrome, nail-patella syndrome, ischiopubic-patellar hypoplasia, and ischiopubic hypoplasia. All described pubic bone defects as well as other bony or soft tissue anomalies .Small patella syndrome (SPS) is characterized by patellar aplasia or hypoplasia and by anomalies of the pelvis and feet, including disrupted ossification of the ischia and inferior pubic rami . BilaterHypoplasia of the ischiopubic region together with spinal dysraphism and scoliosis as well as bilateral aplasia of the patella is an extremely rare anomaly . GenitopAll of these clinical syndromes show multiple bony and soft tissue anomalies -10, but The patient presented here remained free from any complaint for 35 years, a fact that reflects reported data of patients aged from 20 to 70 years, where the main complaint at consultation was with the knees due to patellar instability and pain . Many paHypoplasia of the ischiopubic region is described in some syndromes together with other bony and soft tissue anomalies. However, to the best of the author's knowledge, isolated loss of the inferior pubic ramus without any concomitant bony or soft tissue anomalies has not been reported previously.If the lesion in this case is congenital, it is unusual in being isolated with no other visceral or bony manifestations. Alternatively, the lesion may be secondary to an eroding traumatic hematoma or vascular insult. The etiology of this lesion in this patient remains unknown.The author declares that they have no competing interests.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Patients who present with a co-existing connective tissue disorder add a degree of complexity to operative intervention. We present an unusual case of a 53-year-old Caucasian female patient with Ehlers Danlos syndrome who presented with an occult perforation of the distal ileum. The patient had known small bowel diverticulae yet the perforation occurred within the normal bowel wall. The pre-operative CT only showed malrotation of the large bowel and did not correlate with the intra-operative findings. Our case has highlighted that although small bowel perforation is a rare occurrence, it may be more common in Ehlers Danlos and may present with atypical features. Perforation may also occur alongside normal bowel as well as diverticulae within the bowel. Where diverticulae exists within a patient with Ehlers Danlos syndrome and there is some diagnostic uncertainty, there should be a lower threshold for operative intervention. We present in the discussion a number of salient features and learning points. Patients who present with a co-existing connective tissue disorder add a degree of complexity to operative intervention. Ehlers Danlos syndrome is a group of disorders that affect connective tissue and in type IV leads to the absence of type III collagen in the bowel wall . This caA 53-year-old Caucasian female with Ehlers Danlos syndrome presented with abdominal pain and vomiting to the Emergency Medicine Department. The pain was described as aching in character, initially beginning in the left iliac fossa and radiating to the left upper quadrant. The patient was unable to keep food down and had several episodes of vomiting over the course of the previous 24 hours. She felt distended but the bowel motion was unchanged with normal stools on the day of admission and no rectal bleeding. The patient recounted feeling feverish prior to admission. She had a previous history of diverticular disease and had seen her general practitioner (GP) four days earlier with abdominal pain. Her GP had started her empirically on oral Cefalexin and Metronidazole. Her past medical history consisted of diverticulosis within both the small and large bowel diagnosed on contrast studies Figure . She alsOn examination the abdomen was soft and minimally tender with no guarding, rigidity or rebound tenderness. Bowel sounds were present. Rectal examination revealed soft stool in the rectum. Chest radiograph was normal and abdominal films showed distended bowel loops. The patient's bloods on admission showed a normal white cell count of 6.9 and a raised C - reactive protein of 491. The other bloods were normal. The surgical team organised admission and converted her to intravenous antibiotics and fluids.Over the next 48 hours, the abdominal pain continued to increase and was associated with intermittent vomiting with an inability to eat or drink. C - reactive protein remained elevated. The patient continued to feel distended yet bowels continued to open normally. A repeat abdominal radiograph revealed progressive bowel distension so a CT scan was requested. This was performed the same day and suggested malrotation of the large bowel with a dilated caecum lying under the diaphragm on the left hand side and the ascending colon lying to the left of the midline Figure . MultiplIn view of the continuing deterioration in conjunction with the imaging, a laparotomy was performed. Malrotation of the bowel was found as per the CT findings and the caecum was fixed in the left hypochondrium Figure . NumerouThe small intestine segment with the multiple perforations was resected. A hand sutured double-layer anastomosis was performed. The mesenteric defect was also closed. Haemostasis was obtained and an abdominal lavage performed with two litres of warmed normal saline. A corrugated drain was brought out through the right iliac fossa. Mass closure was performed with loop 0 Nylon. Wound lavage was performed and clips used to appose skin.Post-operatively, intravenous antibiotics were prescribed, and the patient was admitted to the intensive care unit. Total parenteral nutrition was started and the patient kept nil by mouth. Her bloods showed a decreasing CRP from 175 to 32 and her WCC remained stable. The patient's haemoglobin dropped to 6.0 gm/dl post-operatively while her platelets climbed to 1532. The patient refused a blood transfusion. Also during the post-operative period there was a developing mild reduction in peripheral oxygenation noted incidentally with accompanying blue hands and lips.The patient made a good post-operative recovery with discharge to the ward on day 4 with commencement of a normal diet by post-operative day 7. Resumption of normal bowel habit began and patient was discharged home. Follow-up over the next three months confirmed no other complications.Histology revealed a segment of small intestine measuring 16 cm in length × 3 cm in diameter. The serosal surface was noted to be haemorrhagic, with several perforations, the largest measuring 1.0 × 0.5 cm with associated white exudate on the serosa. The mucosa shows a submucosal haemorrhage measuring 1.0 × 1.0 cm, which was 4 cm from its closest margin. The second specimen revealed an omental mass with an appearance of white tissue measuring 2.5 × 1.5 × 1.0 cm.On microscopy, the sections of small bowel show submucosal oedema and vascular dilatation with focal haemorrhage and perforation and organising inflammation in the serosal surface. The mucosa was preserved and showed no significant abnormality. The resection margins showed viable small bowel. There was no dysplasia or malignancy and there was no thrombus in the sections of the mesenteric vessels.Further sections of the omental mass show organising inflammatory debris and granulation tissue. No malignant cells were seen. Within the small bowel resection a submucosal haemorrhage and focal perforation was noted. On microscopy of the omental mass, inflammatory debris was seen.Ehlers Danlos syndrome type IV is an often lethal disease caused by various mutations of type III collagen genes . AffecteIn Ehlers Danlos syndrome type IV, one of the genetically distinct collagens (type III) normally found in skin, aorta, and intestine is missing from the tissues of patients  and onlyIn a case series of 125 patients reported by Beighton et al , two devIn our case, the patient developed perforations, associated with symptoms of mild gastritis and had no episode of GI bleed. In the literature, a permanent stoma is recommended as the operative approach due to the high incidence of anastomotic leak. In a review in Japanese literature, when colonic perforation occurs in this syndrome, total colectomy and ileo-rectal anastomosis is reasonably indicated . The recOur case has highlighted that although GI perforations can occur in Ehlers Danlos it can be a difficult initial diagnosis, and small bowel perforation is a rare occurrence. Perforation may also occur alongside normal bowel as well as diverticulae within the bowel. It is also important to realise that diverticulae can spread from the colon to the stomach and so symptoms of perforation may be varied or atypical. Where jejunal diverticulae exists and there is some diagnostic uncertainty, there should be a lower threshold for operative intervention. It is also important to highlight the significance of minimal bowel handling and to be aware of the complications of primary anastomosis in patients with Ehlers Danlos Syndrome.ED: Ehlers DanlosWritten informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.TL and AC performed literature search and prepared case report, TS, SD, AH, FS contributed to case report and discussion while AA helped in reviewing the literature. All authors read and approved the final manuscript.


Macrodystrophia lipomatosa is a rare cause of gigantism of limb which can be confused with other common causes like congenital lymphedema. It presents usually with loss of function and cosmetic problems. Four cases are described with emphasis on clinical presentation, differential diagnoses, imaging and treatment options.Four patients of macrodystrophia lipomatosa were thoroughly examined and subjected to investigations.Besides diligent clinical examination, imaging and histopathology are crucial in clinching the diagnosis. Macrodystrophia lipomatosa, hamartomatous enlargement of the soft tissue components leading to localized or generalized gigantism of a limb, is a rare congenital disorder which can present anywhere from infancy to late adulthood. There are various causes which can lead to increased size of one or several fingers or toes. Presentation in pediatric age group leads to considerable confusion and till such time when the diagnosis is reached, various descriptive terms are given to such an anomaly . A varieWe present here four cases of the anomaly with the aim to high lighten the clinical features, differential diagnoses and the treatment protocol to be followed in these patients.A 5-year-old girl had a history of steady enlargement of the right-lower limb since birth and mainly the medial three toes of the right foot. There was loss of function but there was no history suggestive of trauma, pain, skin changes or family history. The plain radiographs revealed perceptible increase in the soft tissues elements of the foot. The medial three short bones of the foot appeared a little bulkier but the cortex and their normal form was preserved. Biopsy demonstrated profuse amount of fatty tissue with proliferation of subcutaneous nerves Table .nd toe of the right foot. The 2nd toe of the right foot was unusually large with an increase in subcutaneous tissue which was visibly more prominent on the ventral aspect . Assessment of the other systems showed no abnormality. Radiological examination showed increase in length and diameter of the metatarsals and toes with appreciable increase in the soft tissue component. Punch biopsy revealed increase in the fatty component of the subcutaneous tissue.A two-year-old boy presented with increased size of the toes of left foot which were gradually increasing since 1 year of age. There was no history of increase since birth. On examination we could find that the boy had unusual hypertrophy of the left lower extremity from distal leg onwards with predominant involvement of the 2Macrodystrophia lipomatosa (ML) is a rare congenital anomaly characterized by an abnormal overgrowth of the mesenchymal elements resulting in gigantism of single or multiple digits or the entire limb. Sometimes it may have bilateral involvement of the limbs . AlthougMacrodystrophia Lipomatosa is a congenital anomaly but it is certainly not a hereditary disease . VariousFibrolipomatous hamartoma (FLH) is another heterogeneous group of lesions which produces digital overgrowth and can be confused with ML. It usually presents as an isolated nerve lesion and associated with intramuscular fat deposition . But in In ML, the most prominent histopathological finding is the increase in adipose tissue scattered in a fine lattice of fibrous tissue, which involves the bone marrow, periosteum, muscles, nerve sheaths, and subcutaneous tissues . Neural The problems associated with a patient of ML can be twofold, cosmetic and mechanical. Since overgrowth mainly involves the volar aspect, it can produce dorsal deviation of affected parts. This may lead to interference in normal day to day activities or make patient prone to repeated injury as was seen in our patients. However, mechanical problems may develop like secondary osteoarthritis and compression of neurovascular structures causing impairment of function . CosmetiTypical X ray findings of ML includes excessive growth of soft tissue as well as osseous tissue, presence of radiolucent areas due to presence of adipose tissue and degenerative joint disease. Excessive growth of the bone within the area innervated by nerve and fat tissue proliferation within muscle fibers are the characteristic findings detected on CT scan. The volar aspect of the fingers is disproportionately involved ,13. WideThere are certain conditions which can present with localized limb hypertrophy. The differential diagnosis of ML includes neurofibromatosis type 1 (plexiform neurofibroma), fibrolipomatous hamartoma (FLH), lymphangiomatosis, hemangiomatosis and Klippel-Trenaunay-Weber syndrome, Mafucci syndrome, Ollier disease and Proteus syndrome. But in all of these differential diagnoses there is a positive family history and are characterized by cutaneous or systemic manifestations . For exaUsually the intervention is usually carried out due to cosmetic reasons, but the guiding surgical principle in managing this condition should be improving cosmetic appearance as well as preserving neurologic function . SurgicaML is progressive hamartomatous enlargement of the fibrofatty tissue involving all the layers of soft tissue and even bone more commonly leading to localized gigantism Diagnosis is accomplished on the basis of clinical and radiological evaluation which can be confirmed on histopathological examination The management is mainly surgical but the outcome may not be very gratifying.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journalThe authors declare that they have no competing interests.RAK conceived the study and the design. SW conceived, participated in design and coordinated the study. SW involved in correspondence as well. IA and RSC were involved acquisition, analysis and interpretation of data. All authors read and approved the final manuscript.


Pseudomyxoma peritonei is a poorly understood and uncommon tumor that is known for its production of mucin in the abdominal cavity and mucinous implants, diffusely involving the peritoneal surfaces. A 60-year-old female presented to us with post-op complaints of diffuse abdominal pain and distension. On work-up, she was diagnosed as a case of Pseudomyxoma peritonei . She received chemotherapy in the form of oral capecitabine for residual disease. She was totally asymptomatic till the last follow-up. This case is being reported on account of its rarity and to emphasize a simple alternative treatment option as compared to the standard one. Pseudomyxoma peritonei, a syndrome first described in 1842, is an enigmatic, often fatal, intra-abdominal disease characterized by disseminated gelatinous ascites and multifocal peritoneal epithelial implants, secreting copious globules of extracellular mucin.A 60-year-old female presented in our department in Dec 2007 with post-op complaints of diffuse abdominal pain and distension of the abdomen. On history work-up, she told the investigator that she had pre-op complaints of severe abdominal pain associated with nausea and vomiting and bleeding P/V off and on since 2 months for which she was operated on in Nov 2007 at some private hospital. Her records were checked and the surgical details revealed that she was operated in view of intestinal obstruction and on exploration of the abdomen, bilateral ovarian cysts were present with an appendicle mass and few gelatinous material on the surface of the appendix. Therefore, she underwent surgery in the form of right ovarian cyst salpingoopherectomy, left ovarian cyst excision and appendicectomy. Histopathology on gross examination showed gelatinous material within the multiloculated cyst and the appendix. The cut-surface showed dilated appedicular lumen measuring 0.9 cm and surrounding mass with cystic area containing mucinous material. Microscopic examination was suggestive of invasive well-differentiated mucinous adenocarcinoma appendix with features of Pseudomyxoma peritonei and serosal rupture, borderline  mucinous tumor bilateral ovaries and unremarkable right fallopian tube. Base of the appendix was tumor free. The ovarian tumor probably represents metastatic tumor from the appendicial primary.The pre-op ultrasonography (USG) (26/10/07) finding revealed a 155 mm×175 mm cyst in the right ovary. The margins were well defined and multiple thick septa were seen. Small cysts were also seen in the left ovary.She was properly examined. On per-abdomen examination, no organomegaly was found; the abdomen was distended, but no shifting dullness or fluid thrill was present. On per-vaginal examination, no significant finding was present No lymphadenopathy was present. She was properly investigated. Complete hemogram and liver and renal function tests were within normal limits Figures –2.Serum CEA was 5.29 U/ml, S.CA-19.9 was 2 U/ml and S.CA-125 was 2.32 U/ml. USG-guided fine needle aspiration cytology from the cyst showed gelatinous material and, microscopically, revealed mucinous adenocarcinoma.Contrast-enhanced computed tomography (CECT) scan of the abdomen (17/12/07) showed a small left ovarian cyst of size 18 mm×22 mm with post-operative changes in the right lower abdomen .th cycle of chemotherapy on 20/04/08. On first follow-up after chemotherapy, her S.CEA-level was 3.33 U/ml and CT scan of the abdomen (17/05/08) showed that the hypodense lesion in the left adenexal region that was seen in the previous scan was not seen now [X-ray of the chest was within the normal limit. In view of residual diseases and patient’s symptoms, she was planned for adjuvant chemotherapy with oral capecitabine 2g/day×2 weeks (q-3 weeks) in three divided doses up to six cycles. She received the 6seen now .During chemotherapy, the patient developed incisional hernia for which herniorraphy was performed after completion of the chemotherapy cycles. The patient was kept on monthly follow-up .During follow-up, she was advised to undergo monthly S.CEA. It was noticed that S.CEA was on an increasing trend from July 08 and on 26/10/09, it reached up to 5.65 U/ml. But, her CT-scan of the abdomen was normal. In view of increasing serum marker levels, the patient was advised three more cycles of capecitabine. But, due to financial problems, the patient was planned for four cycles of Inj 5-FU 500 mg I/V d1-d5 along with Inj leucovorin 50 mg I/V d1-d5 (q-4 weeks). The patient has received three cycles of chemotherapy till date and her serum CEA is 4.64 U/ml (dated 28/1/10). At present, the patient is totally asymptomatic.Pseudomyxoma peritonei is a disease of the MUC expressing goblet cells that are specific for mucin production. Extracellular mucin accumulates dramatically in pseudomyxoma peritonei because the number of MUC2-secreting cells dramatically increases and because this MUC2 has no place to drain. The firIt has been found that in most of the cases, the appendix is affected; in females, the ovaries are usually also involved. Focal proliferation appears to be a prognostic factor.Measurement of the tumor markers S.CEA and CA19.9 is useful in evaluating therapy in patients with Pseudomyxoma peritonei treated with cytoreductive surgery and heated intraperitoneal chemotherapy (HIPEC). These markers also have a prognostic value for predicting recurrent disease.The prognosis of Pseudomyxoma peritonei has always been extremely guarded. Sugarbaker published data showing that peritoneal carcinomatosis, regardless of primary tumor type, has always been a lethal condition. Recently, special treatments using cytoreductive surgery with peritonectomy procedures combined with peri-operative intraperitoneal chemotherapy have resulted in long-term survival. Pseudomyxoma peritonei may be especially appropriate for these aggressive local regional treatments.Witkamp, while treating 46 patients of Pseudomyxoma peritonei observed that Pseudomyxoma peritonei remains a fatal disease. However, extensive surgical cytoreduction combined with intra-operative HIPEC has recently emerged as a new treatment modality, which might improve survival.2/L) plus mitomycin-C (3.3 mg/m2/L) for 60 min under hyperthermic conditions (42.5°C), and he concluded that cytoreductive surgery associated with IPHP permitted complete tumor removal with an acceptable morbidity and mortality for patients with Pseudomyxoma peritonei. This study confirms the efficacy of the combined treatment in terms of long-term survival and local disease control.[Deraco treated 35 patients of Pseudomyxoma peritonei. In all cases, cytoreductive surgery was performed with peritonectomy procedure. The closed abdomen technique was employed for intraperitoneal hyperthermic perfusion (IPHP) with the use of cisplatin (25 mg/mIn the literature, only a limited number of patients have received adjuvant systemic chemotherapy. There isOral capecitabine can be considered as an alternative chemotherapy in an adjuvant setting with acceptable toxicity thus minimizing the cost burden as well as the treatment complexity, and 5-FU and leucovorin can be kept as maintenance therapy for Pseudomyxoma peritonei cases.


VIPomas are rare neuroendocrine tumors poorly described in the literature. Aggressive resection of patients with advanced VIPoma neuroendocrine tumors has rarely been reported.A 46 year old women presented with abdominal pain and diarrhea. A three-dimensional (3-D) pancreas protocol computed tomography scan revealed an 18 × 12 cm pancreatic VIPoma abutting the liver, stomach, spleen, left adrenal, colon that also invaded the distal duodenum – proximal jejunum at the ligament of Treitz in association with sinistral portal hypertension. Following preoperative proximal splenic artery embolization, the patient with underwent successful en bloc resection of the locally advanced VIPoma in conjunction with a diaphragmatic resection, total gastrectomy, splenectomy, left adrenalectomy, as well as small and large bowel resection. The estimated blood loss was 500 ml. All margins were negative (R0 resection). The patient is alive and disease-free.This case illustrates the role of aggressive resection of pancreatic neuroendocrine tumors and highlights several key technical points that allowed for successful resection. VIPomas are rare neuroendocrine tumors with an annual incidence of about 1 per 10,000,000 individuals. As with Surgical resection of pancreatic VIPoma provides the only chance at long-term cure, as systemic chemotherapeutic agents are associated with poor response rates. NevertheA 46-year-old obese woman presented to an outside hospital in August of 2005 with significant abdominal pain and diarrhea. Computed tomography (CT) revealed a 17 × 13 cm mass in the left upper quadrant that appeared to arise from the body and tail of the pancreas. The patient was taken to the operating room at an outside institution, but the mass was deemed unresectable due to reported involvement of the SMA, stomach, and colon. Wedge biopsy of the mass was consistent with pancreatic VIPoma. Over the next 2 years, the patient was treated with long-acting somatostatin with some improvement in her symptoms. The patient, however, developed repeat episodes of upper and lower gastrointestinal bleeding with associated anemia and ongoing transfusion requirements. Repeat CT scan revealed thrombosis of the splenic vein with numerous large splenic and gastric varices consistent with sinistral portal hypertension. In the summer of 2007, the patient underwent a failed transjugular intrahepatic portosystemic shunt (TIPS) procedure at an outside institution. The patient was therefore referred to the Johns Hopkins Department of Interventional Radiology for variceal embolization.The patient's case was reviewed at the Johns Hopkins multi-disciplinary pancreas tumor board. A repeat three-dimensional (3-D) pancreas protocol CT scan revealed an 18 × 12 cm mass abutting the liver, stomach, spleen, left adrenal, colon and invading the distal duodenum – proximal jejunum at the ligament of Treitz. The splenic vein was occluded. Large collateral vessels surrounded the mass and were associated with extensive gastric collaterals  that highlights several important peri- and intra-operative concepts. Aggressive resection of VIPomas is warranted and may provide the only chance at long-term survival. When done at large volume, experienced centers even complex multi-visceral resections can be done with low morbidity and near zero morality. In the subset of patients with associated severe sinistral hypertension, proximal splenic artery embolization should be considered as a preoperative means to decrease blood loss and improve outcome. Only by utilizing a multi-modality approach that incorporates state-of-art cross-sectional imaging, interventional radiology, and surgery can these complex patients be managed successfully.The authors declare that they have no competing interests.TP collection of data, analysis of data, draft of manuscript, critical revisions of draft, final review of manuscript, DJ collection of data, analysis of data, draft of manuscript, critical revisions of draft, final review of manuscript, KH collection of data , analysis of data, critical revisions of draft, final review of manuscript, EF collection of data (radiology images), analysis of data, critical revisions of draft, final review of manuscript, JW collection of data (pathology images), analysis of data, critical revisions of draft, final review of manuscript. All authors read and approved the final manuscript.


In the literature, 51 cases of primary retroperitoneal mucinous cystadenocarcinoma have been published. We report the fourth case occurring in a male patient. The 42-year-old patient presented with multiple retroperitoneal cystic masses causing abdominal discomfort without alteration of the global clinical state. The masses were totally removed by a two-stage surgery. No other treatment has been introduced. After a follow-up of 6 months, the patient is disease-free. This rare tumor most likely arises from the mucinous metaplasia of peritoneal inclusion cysts rather than from ectopic ovarian tissue or ovarian teratomas. The occurrence of such a tumor in a male patient supports this theory. Preoperative diagnosis is mostly difficult. Clinical behavior and treatment are still controversial. Primary retroperitoneal mucinous cystadenocarcinoma (PRMC) is an extremely rare tumor. To date, only 51 cases have been published. To our knowledge, this is the fourth case ever reported in a male patient. The pathogenesis remains unclear and controversial. Diagnosis can often be confusing preoperatively. Surgeons must be aware of this type of tumor and include it in their differential diagnosis. Due to its rarity, optimal treatment, survival and exact prognosis continue to be uncertain. We report a case of PRMC in a 42-year-old man. A two-stage surgical removal of all tumoral cysts has been performed.A 42-year-old Moroccan man was initially seen in a private structure for complaints of abdominal discomfort and distension that lasted a year and a half. A notion of smoking weaned 4 years ago was found in his history. Prior physical examination found a large indolent right hypochondrium mass. Both testes were descended and were clinically normal. Biological tests, including Ca 19-9, were strictly normal. On abdominal ultrasonography (US), the mass was thought to be hydatid cyst of the liver . At prioComputed tomography (CT) showed residual retroperitoneal masses with cystic and tissular components, spontaneously hyperdense . No enhath case and to our knowledge, it is the fourth male patient with PRMC published in the literature. The first male patient was reported by Motoyama et al. (1994), with little mention of clinical data and follow-up [To date, only 51 cases of PRMC have been described. All were females  but threollow-up . The secollow-up  presentePathogenesis of these tumors remains unknown. First hypothesis suggests that PRMCs arise from a retroperitoneal monodermal teratoma, with predominant columnar epithelium . Other aSimilarly to prior case reports, we were unable peroperatively to discover a pedicle or attachment to other organs. Histologically, these tumors can be perplexing because they present benign, borderline malignant and frankly malignant areas . The prePRMCs often develop earlier in women with a mean age at presentation of 42.4 years range: 17-86), whereas the diagnosis was assessed in the three previous male patients at 83, 63 and 64 years respectively -7. Our p-86, wherManagement of PRMC is not well established. Clearly, radical tumor excision is mandatory. Adjuvant chemotherapy has been attempted in some patients with limited success ,13. ChemPrognosis of these tumors remains uncertain because of their rarity and the short follow-up of the patients. The longest reported follow-up is 6 years in women  and 18 mPMRC usually presents with mass effects, have an indolent course but can become very large or possess aggressive clinical course. Diagnosis remains difficult preoperatively and surgeons must be aware of PRMC as a differential diagnosis of a large retroperitoneal cystic mass. Clinical behavior and treatment of PRMC still are controversial. To date, extirpative surgery appears to be the standard treatment and the role of adjuvant radiation or chemotherapy has yet to be determined. Further studies are needed to establish optimal treatment protocols.


We report the case of a 61-year-old man showing persistent erythematous macules, plaques, and partially confluent nodules with irregular borders, developed on his nose for one year. During that time the patient underwent several dermatological consultations, and all produced the same diagnosis: rhinophyma. So antibiotic and steroid treatment was carried out without any improvement while the lesions kept growing. When the patient came to our observation, physical examination revealed large, infiltrative, oedematous, erythematous plaques and rare nodules, with superficial telangiectatic vessels. Cervical lymphadenopathy was not detectable. Routine laboratory analysis was normal. Punch biopsy was performed, and histopathology and immunohistochemical studies were consistent with cutaneous angiosarcoma. This is the report of a face angiosarcoma with an unusual and very deceptive clinical presentation. Angiosarcoma (AS) is a rare malignant vascular tumour that arises in skin or superficial soft tissue in 60% of cases . It main The prognosis is poor because the tumour is aggressive with high local recurrence and early metastasis.A 61-year-old man came to our department with erythematous macules, plaques and also rare nodules with irregular borders, localized on the nose, developed for about one year .Before examination at our clinic, the patient was repeatedly treated for rosacea/rhinophyma with oral tetracyclines, metronidazole, and steroids without significant improvement. He also suffered of hypertension, ischemic heart disease, and benign prostatic hyperplasia.Histopathologic examination of skin biopsy, taken from a nodule, showed vascular proliferation lined by atypical and plump endothelial cells in the dermis; immunohistochemistry proved the positivity of neoplastic endothelial cells for CD31 and CD34 Figures . Based on the clinical and histopathological findings, diagnosis of angiosarcoma of the nose was established. Total body CT, showing no significant findings, was performed, and then, according to consolidated cooperation, and also following his own desire, the patient has been put under oncologists care, undertaking Rx therapy (electron beam).Angiosarcoma (AS) is rare, amounting for less than 2% of all sarcomas. Skin and superficial soft tissue are the most common locations (60% of cases) . AS of the skin is often classified asAS on the face and scalp of elderly population,AS either in association with chronic lymphedema or secondary to a prior surgery (Stewart-Treves syndrome),AS following chronic radiodermatitis or skin trauma and ulceration .Our case can be described as AS of the face, that, together with scalp AS, is the most frequent type, with a poor prognosis and a 5-year survival rate of 15% .The etiology of AS remains unknow, but, as many cases arise in the elderly and many patients have a long history of solar and/or chronic ultraviolet exposure, solar damage has been supposed but not confirmed as pathogenetic factor. Few cases have been correlated to other factors as radiation, vinyl chloride, arsenic, insecticides, androgenic steroids, and thorium dioxide exposure. Actually there are no known genetic defects or chromosomal abnormalities implicated in AS pathogenesis .The classical manifestation is characterized by erythematous plaque which expands rapidly involving large areas of the skin, forming blue-black nodules that often ulcerate. Facial oedema can develop too . SometimThe tumour often mimics many different conditions such as arteriovenous malformations, nodular melanoma, lymphoma, sarcoidosis, or facial granuloma . The clinical picture described in this paper strongly mimicked rhinophyma for a long period of time, to the point that it was repeatedly treated by steroids and oral antibiotics, further delaying the correct diagnosis.In the literature both AS developing on rhinophyma and AS resembling rhinophyma are reported. The former was described by Gallardo et al. (2000)  and the Some authors believe that nasal AS has a better prognosis due to the lower grade nature and to a shorter interval between diagnosis and treatment, but there are few reports accurately describing the survival periods, moreover, different survival rate can be related to differences in treatment  and evenTherapeutical approach comprises wide resection in localised neoplasms while for larger tumors radiotherapy or chemotherapy can be used, even if no standard therapeutic protocols have been yet established .The present case is reported because of its peculiar and rare clinical presentation, characterized by marked rhinophyma-like features making it a difficult but intriguing diagnostic challenge.


Secondary peritoneal hydatidosis is caused by spontaneous or iatrogenic rupture of hepatic echinococcal cysts. We describe the case of a 65-year-old Tunisian male patient with previous history of liver hydatidosis who presented to our attention with subocclusive status. Imaging revealed a retrovesical hydatid cyst, adherent to the sigmoid colon. The treatment of choice was surgical removal of the cyst and the sigmoid colon. The patient is now being closely followed up. Hydatid disease or Echinococcosis is endemic in Mediterranean regions. The liver and lung are the most frequently involved organs. Peritoneal hydatidosis accounts for 13% of all abdominal hydatidosis. It is almost always secondary to seeding from spontaneous rupture of hepatic cyst or spillage of cyst fluid during previous surgery . It may In February 2010, a 65-year-old Tunisian male patient in good general conditions presented to our attention complaining from moderate abdominal pain and subocclusive status. In 1994, he underwent an operation for liver echinococcosis; the description of surgical procedure was not available. On the ultrasound examination and computed tomography, a diagnosis of pelvic hydatid cyst of 6.5 cm in diameter was made. In the preoperative planning, we decided to perform a magnetic resonance scan in order to study the relation of the cyst with contiguous organs Figures , and it Peritoneal hydatidosis is almost always secondary to a hepatic disease, in particularly related to previous surgery for liver cysts. Peritoneal involvement typically remains silent for years and is usually undetected unless cysts are large enough to cause symptoms. The differential diagnosis is usually not a problem. Ultrasonography and computed tomography are both excellent imaging modalities for the detection of hydatid cysts. The usefulness of magnetic resonance imaging lays in the precise definition of the anatomic relationship due to the excellent resolution for soft tissues . The tre


Adamantinoma is a primary low-grade, malignant bone tumor that is predominantly located in the mid-portion of the tibia. The etiology of the tumor is still a matter of debate. The initial symptoms of adamantinoma are often indolent and nonspecific and depend on location and extent of the disease. Histologically, classic adamantinoma is a biphasic tumor characterized by epithelial and osteofibrous components that may be intermingled with each other in various proportions and differentiating patterns. To assure the histological diagnosis, pathologists should employ immunohistochemistry for demonstrating the sometimes sparse epithelial cell nests when the radiological features are suggestive of adamantinoma. There is paucity of compiled data over adamantinoma in the literature, hence authors tried to make a comprehensive review which must be of use to beginners and trained pathologists. Our objective is to further define the clinicoradiologic features and pathologic spectra of adamantinoma. Adamantinoma is a primary low-grade, malignant bone tumor, of unknown histogenesis. However recent opinion seems to suggest that adamantinoma is a tumor of epithelial origin, based on ultrastructural and immunohistochemical studies . The tumIn the past there has been much speculation about the origin of adamantinoma. Several hypotheses have been proposed for its histogenesis ,6-22 Ta. FischerAdamantinoma mostly occurs in the second to fifth decade. The median patient age is 25 to 35 years, with a range from 2 years to 86 years. It is slightly more common in men than women, with a ratio of 5:4 ,26.The tumor has a striking predilection for the long bones (97 percent of cases) and, specifically, the tibia (80 to 85 percent of cases), represents the most characteristic clinical feature of this tumor. In 10 to15%, the lesion is also found in the ipsilateral fibula . StatistThe initial symptoms of adamantinoma are often indolent and nonspecific and depend on location and extent of the disease. The onset is insidious and its course shows a slow, progressive character. The patient often tolerates symptoms for many years before seeking medical attention. A history of significant trauma has been noted in about 60% of 200 cases reviewed by Moon and Mori . Most paRadiographically adamantinoma of bone displays features that are somewhat characteristic and help in establishment of presumptive diagnosis because of tumor's classic location and appearance. In the tibia, it usually appears as a central or eccentric, multilocular because of the sclerotic margins of overlapping radiolucencies, slightly expansile, sharply or poorly delineated osteolytic lesion. As previously stated, in long bones it is found in the diaphyseal location although metaphyseal extension of lesions or isolated involvement of a metaphysis is seen occasionally. The metaphyseal involvement makes the diagnosis more challenging because other tumors have to be considered in the differential diagnosis. The lesions are well-circumscribed involving anterior tibial cortex, with septations and peripheral sclerosis. Multifocality in the same bone is regularly observed. These multifocal radiolucencies surrounded by ring-shaped densities producing the characteristic "soap-bubble" appearance. The lesion is commonly intra-cortical, but may destroy cortex and invade the extracortical soft tissues in about 15% of cases .Radiologically, the differential diagnosis of adamantinoma should include osteofibrous dysplasia and fibrous dysplasia. Distinguishing osteofibrous dysplasia and fibrous dysplasia from adamantinoma may be difficult; however, diaphyseal location, involvement of the anterior cortical bone with extension toward the bone marrow, single or multiple nodular lesions in one or more foci, and intense and homogeneous enhancement may contribute to the diagnosis of adamantinoma. OFD is specifically located in the cortex without involvement of the medullary canal .Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) have been used to study the lesions of adamantinoma and usually the findings are not specific. CT scan shows the cortical involvement and the soft tissue extension when it exist. However, does not show the intraosseous extension of the tumour. CT does play a role in detecting pulmonary metastases and as such plays a role in the routine work-up .MRI is pivotal for precise locoregional staging, especially for depiction of distant cortical foci, soft tissue, and intramedullary extension and thus is useful for determining tumor-free margins and strategies for reconstructive surgery. Van der Woude et al described two morphological patterns on MR imaging: a solitary lobulated focus versus a pattern of multiple small nodules in one or more foci. If MRI scanning is used to study adamantinomas, the tumors demonstrate low signal intensity on T1-weighted spin echo images and high signal on T2-weighted images. Because these appearances are also typical of most tumors, these findings are nonspecific .Use of nuclear medicine to study adamantinomas is a relatively new undertaking. The following findings correspond to adamantinomatous lesions: increased blood flow in the region of the tumor, increased blood pooling, and increased accumulation of technetium-99m methylene diphosphate in the area of the tumor. In addition, bone scan may show a coexisting fibular involvement .Histologically, the classic adamantinoma is a biphasic tumor and characterized by epithelial and osteofibrous components that may be intermingled with each other in various proportions and differentiating patterns. Adamantinoma is classified into 2 distinct types: classic and differentiated. Classic adamantinoma usually occurs in patients older than 20 years, whereas differentiated adamantinoma (Osteofibrous dysplasia (OFD) – like/regressive/juvenile/intracortical adamantinoma) occurs almost exclusively in patients younger than 20 years . In addiClassic adamantinoma is in general characterized by admixture of both epithelial and osteofibrous components that are associated with various proportions and differentiation patterns. OFD-like adamantinoma is characterized by predominance of osteofibrous tissues, in which small groups of epithelial cells are only detected by careful search or immunohistochemistry.The appearance varies but most often the tumor is yellow gray or grayish white and fleshy or firm in consistency. Some OFD like adamantinomas are more solid because of the presence of relatively large bone forming areas. Occasionally tumors show macroscopic cysts range from a few millimeters to centimeters in diameter. These cystic spaces contain a straw colored or blood like fluid on gross examination .The neoplastic cells of the classic adamantinoma range from small to large in size with finely dispersed chromatin and an overall bland appearance. A few cases from a large series have exhibited nuclear atypia. Mitotic figures are usually infrequent, most reporting 0–2 mitoses per 10 high power fields ,47. SeveFoci of calcification , giant cIt has been postulated that the predominance of an osteofibrous dysplasia-like pattern in differentiated adamantinoma is the result of a secondary reparative process overgrowing matured and regressing tumor tissue. It is possible that this process may lead to the total elimination of recognizable tumor cells from the lesion. Therefore, osteofibrous dysplasia, which has a similar anatomic location, age distribution, and radiologic appearance as differentiated adamantinoma, may, in some cases, represent the evolution of an underlying adamantinoma. Analysis suggests that long bone adamantinoma could be another member of the unique family of tumors that may regress spontaneously .Because some osteofibrous dysplasia-like tumors have progressed to classic adamantinomas, it is hypothesized that the former is a potential precursor of the latter, showing mesenchymal-to-epithelial transformation. There are two cases reported in the literature of progression from differentiated to the classic type . There hA probable histogenetic relationship exists between the three entities. Indeed, the only apparent difference between osteofibrous dysplasia and differentiated adamantinoma is the presence of an epithelial component readily identifiable on routine hematoxylin and eosin stained sections and their identification only by immunohistochemical means in about 80% of osteofibrous dysplasia lesions Table .de novo osteofibrous dysplasia not related to adamantinoma. Sweet et al [Czerniak et al  proposedet et al  studied et et al .Recently, the least common variant of adamatinoma has been described as Ewing's-like adamantinoma or adamantinoma-like Ewing's. The variant is characterized by anastomosing cords of small, uniform, round cells set in a myxoid stroma. These cells exhibit features of both epithelial cells and neuroendocrine cells on ultrastructural examination. Immunohistochemical studies have shown the tumor cells to contain both epithelial and neural antigens including the Ewing's sarcoma-related antigen ,57. BridRegardless of histologic subtypes, all adamantinomas have been uniformly positive for keratins 14 and 19 . ImmunohThere is still debate as to whether the fibrous part should be designed as a benign neoplastic element of a biphasic tumour or as a reactive non-neoplastic tissue next to an epithelioid bone tumour. Bovee et al  examinedMaki and Athanasou  correlatCytogenetic analysis has contributed greatly to the understanding of the histopathogenesis of adamantinoma and osteofibrous dysplasia. Unfortunately, because of their rarity, cytogenetic reports concerning these tumors are few. Review of previously reported cases reveal extra copies of chromosomes 7, 8, 12, 19, and/or 21 in classic and differentiated adamantinoma ,68-70. EThere are several publications that confer the radiologic, clinical, and histopathologic features of adamantinoma; on the contrary, only few cases of the cytologic findings of adamantinoma are found -76. CytoBecause of its rarity and differing clinicoradiologic and histological patterns, adamantinoma may resemble numerous conditions Table .Adamantinomas are locally aggressive tumor and are extremely slow growing with the potential to metastasize. Recurrence of tumor is frequent after inadequate therapy, and the behavior of the recurrent neoplasm resembles more and more that of a sarcoma. This low-grade, slowly-growing malignancy metastasizes in about 15–30 percent of cases by both hematogenous and lymphatic routes to other parts of the body, usually to the lungs or nearby lymph nodes; bone and abdominal viscera make up a minority ,27,65,77Local recurrence rate varies from 18–32% ,65. AlthCurrent treatment of adamantinoma, including en bloc tumor resection with wide operative margins with limb reconstruction and limb salvage, provides lower rates of local recurrence than has been previously reported . QuereshAlthough the incidence of adamantinoma is low, it is important to recognize this rare bone tumour, since in early stages of the disease adequate treatment will result in an excellent prognosis. The histologic features of primary adamantinoma are usually characteristic enough for a presumptive diagnosis; however, the rarity and the heterogeneity of the tumor could pose diagnostic uncertainty in some cases, especially those arising in non-tibial locations. Extensive sampling of the lesion is important, especially in the differentiated adamantinoma where the epithelial component may be seen only focally. To assure the histological diagnosis pathologists should employ immunohistochemistry for demonstrating the sometimes sparse epithelial cell nests when radiology is suggestive for adamantinoma. Correct diagnosis should lead to resection with wide surgical margins.The author(s) declare that they have no competing interests.DJ and VKJ are primarily responsible for drafting, literature search, and submission of the manuscript. RKV established the diagnosis of the case described in figures. PR and YK provided images. All authors have read and approved the final manuscript.


Solitary neurofibroma is a rare benign non-odontogenic tumor. Particularly in the oral cavity, neurogenic tumors are rare, especially if they are malignant. Neurofibromas may present either as solitary lesions or as part of the generalised syndrome of neurofibromatosis or von Recklinghausen's disease of the skin. Clinically, oral neurofibromas usually appear as pediculated or sessile nodules, with slow growth and mostly without pain. The diagnosis can be confirmed by histological examination. Neurofibromas are immunopositive for the S-100 protein, indicating its neural origin. Treatment is surgical and the prognosis is excellent. For illustration a rare case of a solitary neurofibroma in the mandible is presented. Neurogenic tumors are rare in the oral cavity, particularly so when malignant. Traumatic neuroma, although usually included with neurogenic tumors, is a reactive process rather than a true neoplasm . In contAlthough neurofibroma represents one of the most common neurogenic tumors it is an uncommon intraoral tumor  like somClinically, oral neurofibromas usually appear as pediculated or sessile nodule, with slow growth. They are usually painless, but pain or paresthesia may occur due to nervous compression. The most frequent location is the tongue, although they may occur at any site, especially on the palate, cheek mucosa and floor of the mouth ,11-14. EThe macroscopic appearance of the oral neurofibroma is characterized by a whitish consistent mass with shiny surface. Microscopically the tumor is composed of an irregular pattern of proliferative spindle cells. The stroma is composed of collagen fibers and mucoid masses. Small axons all over the tumoral tissue are demonstrated with silver staining. Neurofibromas are immunopositive for the S-100 protein in 85 to 100% of the cases, indicating its neural origin -23.Treatment of choice is surgical excision of the solitary lesions, trying to conserve the nerve from which the tumor originates . MalignaA 64-year-old male patient with a history of somewhat alcohol but no nicotine or any other diseases attended the department for Cranio- and Maxillofacial Surgery. Clinical examination revealed an exophytic tumor in the oral cavity extending all along the lingual aspect of the left mandible fig. . PanoramIn the presenting case the lesion occured at the lingual site of the left mandible presenting as painless pediculated exophytic tumor with slow growth. The localisation supports the lingual nerve as origin for the neurofibroma. The patient presented no signs of von Recklinghausen disease or poliglandular syndrome. Preoperative panoramic radiographs showed no serious abnormalities. Definitive diagnosis was based upon histological and inmunohistochemical findings. A clinical follow-up has been regularly performed for one year, there was no evidence of recurrence until now.All authors disclaim any financial or non-financial interests or commercial associations that might pose or create a conflict of interest with information presented in this manuscript.DS, JH, RD and NK made substantial contribution to the conception and design of the manuscript. PR carried out the pathohistological investigations and participated in creating this part of the manuscript.All authors were involved in revising the manuscript critically and have given final approval of the version to be published.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.