Congenital midline cervical cleft (CMCC) is a rare congenital anomaly. CMCC and its complications and treatment have been well described in ENT, dermatology, and pediatric surgery literature. However, to our knowledge, the imaging work-up has not been reported in the literature thus far. We present a case of CMCC in a neonate with description of clinical presentation and imaging features. Congenital midline cervical cleft was first described in 1848 by Luschka  explaineImaging may be requested to differentiate CMCC from these lesions or to determine any coexisting lesions . MagnetiWe present a case of CMCC in a neonate with description of clinical presentation and imaging features.A full term male neonate was born at 38 weeks of gestation with birth weight of 3465 grams. The infant's mother was a 20-year-old African American female with a history of obesity and pregnancy induced hypertension. Otherwise there was no history of tobacco, drug, or alcohol abuse and all the routine prenatal laboratory tests were normal.At birth, the child was examined and noted to have a linear craniocaudal pink track in the anterior neck with a skin tag arising from its superior aspect . No discBecause of the presence of a cervical cleft an MRI was requested to evaluate the cleft and to look for its internal extension and associated anomalies in the neck.MRI with contrast was performed which demonstrated a defect in the cutaneous/subcutaneous soft tissues in the midline anterior neck, anterior to the strap muscles. The defect measured 2 cm in craniocaudal dimension and 0.5 cm in its width. It demonstrated low T1 and T2 signal and extended from the level of hyoid bone caudally to the level of manubrium Figures , and 4. Congenital midline cervical cleft (CMCC) is a rare congenital anomaly, which is thought to be due to failure of fusion of the first and second branchial arches during embryogenesis . Less thAn incidence of 1-2% of congenital cervical malformations has been described in various case series; these include absence of thyroid, ectopic thyroid or thyroglossal duct cyst, ectopic bronchogenic cyst, branchial cyst, midline hemangioma, midline abdominal web, ectopia cordis, clefts of the lip, chin, tongue, mandible, or sternum, absence of hyoid bone or thyroid cartilage, and rarely congenital cardiac anomalies . Our casAs the child grows or if there is inadequate repair, the fibrous cord becomes more conspicuous and can lead to contractures of the neck, torticollis, or limited extension . Due to Antibiotics may be required to treat infections or abscesses of congenital midline cervical clefts. Treatment is usually surgical excision and closure of the defect. Although exact time and technique of surgery are variable in different reports, most of the authors advocate earliest possible intervention for best results. Z-plasty incisions are recommended especially in cases of delayed presentation in order to prevent the development of scar and subsequent cicatrices contractures . PrimaryIn summary, a congenital cervical cleft is a rare anomaly. A small number of these are associated with other midline anomalies of the head and neck and the chest. MRI is a useful modality for demonstrating the extent of the cleft, determining if there are any other associated anomalies, and it may be useful for presurgical planning for repair.


Intussusception in adults is a rare cause of abdominal pain that is often associated with organic pathology. We describe a case of ileocolic intussusception revealing a cecal adenocarcinoma in a young woman successfully managed by laparoscopic-assisted surgery adhering to oncological principles.A 30-year-old woman with a family history of colon adenocarcinoma in a young brother presented to our emergency department with a 2-month history of intermittent colicky abdominal pain accompanied by nausea and vomiting. Physical examination showed a palpable mass in the right lower quadrant of the abdomen. Computed tomography showed a 3-layered structure giving the characteristic target-shaped appearance in the ascending colon, highly suggestive for an ileocolic intussusception associated with right colic parietal thickening and an adjacent lymphadenopathy.Patient was planned for laparoscopic exploration and eventually definitive surgery. Intra-operatively, we found an ileocolic intussusception with thickening of the colic wall and slight proximal intestinal dilation. Multiple lymphadenopathies along the ileocecal artery were observed. Laparoscopic right hemicolectomy was performed following strict oncologic principles with “en bloc resection” and lymphadenectomy given the risk of an underlying malignancy. Considering this risk, previous reduction of the invaginated segments was not attempted and primary extracorporeal anastomosis was performed using manual sutures.Macroscopic examination of the resected specimen revealed a tumor mass of the caecal wall .The histological analysis identified a moderately differentiated tubular adenocarcinoma invading the serosa (T3) without permeation of the lymphatic or venous capillaries .No lymphatic metastasis of 28 nodes removed was seen. Postoperative course was uneventful and patient was discharged 5 days after surgery.Postoperative chest, abdomen, and pelvis CT scan were normal .Therefore, tumor is classified as stage II A (T3N0 M0).There was loss of MLH2 and MSH6 protein expression on immunohistochemistry findings reflecting a microsatellite instability phenotype, and the patient was followed up without adjuvant chemotherapy.Ileocolic intussusception rarely revealed a cancer in young adults. Laparoscopic surgery has a special interest in the diagnosis and treatment in this pathology. Oncogenetic consultation should be required in malignant lesion. Intussusception in adults is a rare cause of abdominal pain. Unlike its pediatric counterpart, intussusception in adults is often associated with organic pathology. We describe a case of ileocolic intussusception revealing a cecal adenocarcinoma in a young woman successfully managed by laparoscopic-assisted surgery adhering to oncological principles.A 30-year-old woman with a family history of colon adenocarcinoma in a young brother presented to our emergency department with a 2-month history of intermittent colicky abdominal pain accompanied by nausea and vomiting. She denied any history of gastrointestinal bleeding, fever, or past abdominal surgery. Her appetite was good but she reported a 3-kg weight loss during the previous 3 months. Physical examination showed a palpable mass in the right lower quadrant of the abdomen, but digital rectal examination was unremarkable. This mass was firm, non-tender, and slightly painful with limited mobility, but no signs of peritoneal reaction were evident. All laboratory investigations, particularly tumor markers including carcinoembryonic antigen (CEA) and carbohydrate antigen (CA 19.9) were within the normal limits.Ultrasound revealed a prominent swelling of the intestinal wall with target signs and a hyperechoic mass over the right lower quadrant area. An abdominal computed tomography (CT) was carried out to confirm the ultrasound findings. This showed a three-layered structure giving the characteristic target-shaped appearance in the ascending colon, highly suggestive for an ileocolic intussusception Fig. . AdditioPatient was planned for laparoscopic exploration and eventually definitive surgery. Intra-operatively, we found an ileocolic intussusception with thickening of the colic wall and slight proximal intestinal dilation. In addition, multiple lymphadenopathies along the ileocecal artery were observed, but no signs of intestinal ischemia or peritoneal carcinosis were noted. Consequently, we performed a laparoscopic right hemicolectomy Fig.  followinMacroscopic examination of the resected specimen revealed a tumor mass of the caecal wall measuring 4 × 5 × 4 cm and occupying more than 3-quarters of the circumference . Microsatellite status and immunohistochemistry of four deoxyribonucleic acid (DNA) mismatch repair proteins, including mutL homologue 1, post meiotic segregation increased 2, mutS homologue 2, and mutS homologue 6, were checked.There was loss of MLH2 and MSH6 protein expression on immunohistochemistry findings reflecting a microsatellite instability phenotype Fig. , and the. The exact mechanism is still unknown, and it is believed that any lesion in the bowel wall or irritant within the lumen that alters normal peristaltic activity is able to initiate invagination. Unlike in children, the clinical presentation of adult intussusception is considerably various and non-specific. Most patients present with intermittent or chronic symptoms that are suggestive of incomplete intestinal obstruction. The classic pediatric triad of abdominal pain, palpable abdominal mass, and bloody stool is rarely seen [Intussusception is an uncommon disease in the adult population compared with that in children . First dely seen , 3, 4.The accuracy of computed tomography for diagnosis of adult intussusception is better than that of other examinations, including abdominal ultrasonography, barium enema study, and colonoscopy . TypicalIn this case, a preoperative diagnosis of intussusception was easily made by conventional imaging techniques including ultrasonography and CT scan despite longstanding digestive symptoms, but colonoscopy did not show the intussusception. Preoperative diagnosis of intussusception is infrequent in the adult with a rate reported to range from 32 to 70 % .The optimal therapeutic management of adult intussusception remains controversial due to the differing etiology between pediatric and adult populations. A pathologic lesion is found in 90 % of adult intussusception cases . TherefoConventionally, laparotomy should be considered for the treatment. Given that laparoscopic management of pediatric intussusception has been available for some time  and thatWe herein describe a laparoscopic treatment for adult colonic intussusception caused by cecum carcinoma. In the present case, laparoscopic right hemicolectomy was successfully performed. The experience gained in the present case suggests that laparoscopy may be a diagnostic or therapeutic tool for selected cases of young adult intussusception.The histological examination showed a cecal adenocarcinoma stage II A according to the TNM classification, for which the indication of adjuvant chemotherapy is subject of controversy. Our patient presented more favorable prognostic factors such that: tumor was pT3, moderately differentiated and without angiolymphatic, or perineural invasion. Node dissection was optimal, and surgical margins were negative. Microsatellite was instable, and patient can be spared adjuvant chemotherapy .Finally, many items are in favor of a lynch syndrome according to BETHESDA criteria; colon cancer was diagnosed before the age of 50 years, with one first-degree relative with an HNPCC-related tumor diagnosed under age 50 years. The abundant mucus is presented, and microsatellite was an instable phenotype with loss of expression of MSH2 and MSH 6 which often reflects a germline mutation and therefore an HNPCC. Lynch syndrome is suspected, and cancer genetics consultation is required .Ileocolic intussusception rarely revealed a cancer in young adults. It can detect a genetic predisposition. Laparoscopic surgery has a special interest in the diagnosis and treatment in this pathology. Oncogenetic consultation should be required in a malignant lesion.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.


Klippel-Trenaunay-Weber syndrome (KTWS) is a rare syndrome in which patients usually present with cutaneous hemangiomas, venous varicosities, and bone and soft tissue hypertrophy of the affected limb. Intracranial lesions in patients with KTWS are extremely rare, and are generally reported as single cases in the literature. We describe a rare case, where a patient with KTWS was found with a hemorrhagic grade IV astrocytoma. Although central nervous system abnormalities such as intracranial aneurysms and cerebral and spinal cord cavernomas have been described in patients with KTWS, to the best of our knowledge, this is the first report of an association between glioblastoma multiforme (grade IV astrocytoma) and KTWS in the English-language medical literature.A 61-year-old white Caucasian man with a history of KTWS presented with seizures. Left upper and lower extremity hypertrophy, left foot, leg and ear gigantism and left-sided abdominal capillary hemangiomas were noted in the physical examination. Cranial computed tomography (CT) and magnetic resonance imaging (MRI) were obtained, showing a heterogeneous lesion in the cingulate gyrus, with peripheral and central areas of T1 hyperintensity and layering T2 hypointensity consistent with a hemorrhage. A right parasagittal frontal craniotomy was performed with an interhemispheric approach. We had difficulty controlling the bleeding with bipolar electrocautery during surgery and finally were able to stop the bleeding using surgicel and gelfoam. Postoperative cranial CT and MRI scans showed intraparenchymal hemorrhage centered within the medial right frontal lobe. There was no increase in hematoma size in consecutive CT scans.Co-occurrence of vascular abnormalities with KWTS should be taken into consideration to avoid perilous preoperative and postoperative complications. Klippel-Trenaunay-Weber syndrome (KTWS) is a congenital vascular disorder characterized by cutaneous capillary hemangiomas (port-wine stains), venous varicosities, and osseous and soft tissue hypertrophy. Many theories have been developed since the discovery of KTWS; however, the etiology of the syndrome is still unknown. The general assumption outlines a polygenic etiology involving defects in angiogenesis and growth regulation of vessels and tissue . IntracrA 61-year-old white Caucasian man with history of KTWS presented with seizures. Our patient had been diagnosed with KTWS at the age of seven after an episode of rectal bleeding. Upon neurological examination, our patient was able to follow commands and move all extremities, and exhibited mild left drift. Left upper and lower extremity hypertrophy, left foot, leg and ear gigantism and left-sided abdominal capillary hemangiomas were noted upon physical examination Figure . CranialKTWS is a rare congenital phakomatosis, characterized by asymmetric limb hypertrophy, cutaneous hemangiomas and varicosities. Capillary malformations are the prevailing lesions (seen in 98% of patients), which include port-wine stains. Venous anomalies are seen in 72%, and include hypoplasia or aplasia of veins, persistence of fetal veins, varicosities, hypertrophy, tortuosity and valvular malformations. Soft tissue hypertrophy is present in 67% of cases [Astrocytoma grade IV is the most malignant form of central nervous system tumors, and current therapies are largely ineffective at treating the cancer. Although a common brain tumor, astrocytoma grade IV in KTWS has not been previously reported.In regard to neurovascular comorbidities, it should be noted that KTWS has been reported with accompanying central nervous system abnormalities in the literature. Aneurysmal dilatation is common, but cerebral or spinal arteriovenous malformations, persistent embryological veins, orbitofrontal varices, multiple aneurysms, meningiomatosis, epidural or vertebral hemangiomas, multiple cerebral and spinal cord cavernomas, venous malformations and duplications, hypo and aplasia have also been reported ,5,6,9,10It must be noted that an abundance of these vascular pathologies leads to an increase in the surgical complication rate. It was reported preoperatively 27% of patients had a history of recurrent bleeding . The undet al. [Mason et al.  reportedet al. . For sevet al. .Association of KTWS with intracranial pathologies that may require surgical intervention poses a significant challenge to the managing team both preoperatively and postoperatively. In these patients, a full coagulation profile should be studied during the preoperative period. Since there is not enough experience with any specific thrombolytic agents that may stop hemorrhage, we recommended all potential agents such as platelets, cryoprecipitate, fresh-frozen plasma and tranexamic acid be readily available intraoperatively. In addition to these preoperative and perioperative precautions, postoperative care is of great importance in monitoring hemorrhagic complications.Additional vascular pathologies should be considered during the surgical planning for comorbidities accompanying KTWS, and necessary pre- and postoperative measures should be taken accordingly.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.


Seminomatous and non-seminomatous Germ Cell Tumors (GCT) of the testis are a rare cancer, with an estimated incidence of 56.3 per million white males and 10 per million black males in the United States.The association between non-seminomatous GCT and horseshoe kidney is a rare event and is seen in about 1.3% of patients requiring retroperitoneal lymph node dissection. To our knowledge, no cases have been reported in which replacement of the IVC was also necessary.We report the case of a 22-year-old man with horseshoe kidney and metastatic non-seminomatous germ cell tumor involving the wall of the inferior vena cava.Following post-chemotherapy retroperitonal lymph node dissection, the inferior vena cava was replaced with an expanded polytetrafluoroethylene graft.At 2-years follow-up, the patient was in good health and the graft was patent. No clinical or diagnostic signs of renal impairment or recurrence of neoplastic disease were noted.Radical surgery is warranted in patients with non-seminomatous germ cell tumor metastasizing to the retroperitoneal lymph nodes. When vena cava replacement is required, and the situation is further complicated by horseshoe kidney, as in this case, surgical technique will rely on multidisciplinary surgical treatment planning by a team composed of urologists, vascular surgeons and oncologists. The annual incidence of seminomatous GCT is about 32 cases per million and that of non-seminomatous GCT about 27 cases per million [Testicular cancer is the most frequent type of testicular cancer in males between 20 and 35 years of age; the 5-year survival rate of seminomatous GCT is 72-80% and that of non-seminomatous GCT is 48-92% depending on prognostic class [Surgery with either post-chemotherapy lymph node dissection or residual tumor resection has become a mainstay in the treatment of non-seminomatous GCT presenting one or more residual masses after chemotherapy. As post-chemotherapy surgery poses particular challenges and often requires ad hoc vascular intervention, e.g., vena cava or aortic graft replacement, patients should be referred to a specialized surgery center with expertise in hepatic resection, vessel replacement, spinal neurosurgery, and thoracic surgery. The benefit to patients treated at such interdisciplinary centers is a significant reduction in perioperative mortality from 6 to 0.8%  and locaThe concurrent presentation of non-seminomatous GCT with retroperitoneal metastasis involving the inferior vena cava and horseshoe kidney, a congenital disorder, is a rare event that further complicates surgical treatment of the tumor. To our knowledge, this is the first such case to be reported.A 22-year-old man underwent right radical orchiectomy for a testicular mass; the histopathological diagnosis was pure teratoma of the testis. Computed Tomography (CT) with contrast material of the abdomen, chest and head for tumor staging showed metastases to the liver, lungs, retroperitoneal lymph nodes, and brain. The imaging studies also revealed a right laterocaval retroperitoneal mass (largest diameter 7 × 9 cm) invading the iliopsoas muscle but without clear signs of caval wall infiltration Figure . An inciThe patient subsequently underwent three cycles of chemotherapy with etoposide and cisplatin; a fourth cycle of EP was suspended due to the occurrence of a bacterial endocarditis. After the improvement of clinical conditions, the patient underwent a salvage chemotherapy with four cycles of etoposide, ifosfamide and cisplatin, during which the BHCG level decreased to 86 IU/L. On Positron-Emission Tomography (PET), elevated metabolic activity was absent in the lung and liver lesions but present in the laterocaval retroperitoneal lymph node mass.On AngioCT prior to surgery for removal of the residual retroperitoneal mass, the mass (largest diameter 5-6 cm) was found to be continuous with the inferior vena cava and extend to the inter-aorto-caval area. The images also showed, besides the known horseshoe kidney, two right renal arteries, one left renal artery, and one renal vein on each side draining into the inferior vena cava.The retroperitoneal mass was removed via transperitoneal surgical access. After a puboxiphoid incision and V opening of the retroperitoneal cavity, the right side of the horseshoe kidney and the two right renal arteries were isolated. The retroperitoneal lymphatic mass (largest diameter about 6 cm) was found to infiltrate the psoas muscle and the lateral wall of the inferior vena cava along its entire thickness for about 5 cm, from the segment underneath the renal isthmus till 3-4 cm from the iliocaval confluence Figure .The right external iliac lymph nodes, laterocaval, interaortocaval and presacral lymph nodes were removed. The right side of the horseshoe kidney was dislodged from the posterior planes of the abdominal cavity, flipped superomedially en bloc with its vascular structures to expose the mass Figure . The lymControl CT at 1 month postoperative showed graft and inferior vena cava patency, a reduction of about 20% in the volume of the lung lesions as compared to the CT scan obtained before chemotherapy, and no recurrence of neoplastic abdominal tissue. The graft patency and absence of abdominal neoplastic tissue was confirmed at 1 year AngioCT Figure .The patient then underwent removal of the brain metastases secondary to GCT with choriocarcinomatous features, followed by one cycle of chemotherapy with paclitaxel, ifosfamide, and cisplatin. At 18 months the patient underwent resection of bilateral lung lesions. At 2 years follow-up, the patients was in good health and virtually disease free . Control AngioCT confirmed transformation of the liver lesions into cysts. The inferior vena cava and the graft were both patent, and no signs of renal insufficiency were noted. The patient is still under oral anticoagulant therapy. Renal function was unchanged as compared to pretreatment status.The International Germ Cell Cancer Collaborative Group (IGCCCG) classifies GCT as a tumor having good, medium, or poor prognosis on the basis of variables including primary tumor site, presence or absence of metastases, levels of AFP (Alpha-FetoProtein), BHCG, and LDH . In viewRecent studies have shown that about 7% of patients who had received post-chemotherapy retroperitoneal lymph node dissection will require resection of the IVC  because An alternative to grafting may be primary ligation of the IVC without replacement of the removed segment; however, this is associated with a higher post-operative complications rate, a mortality rate of 9%, and severe lower limbs oedema in 36% of cases. Symptoms secondary to chronic venous insufficiency, including secondary varicose veins of the lower limbs, scrotum and abdomen, have been reported in up to 50% of cases. A recent study in 47 patients undergoing caval replacement for infiltrating tumor showed that graft replacement of the IVC prevents venous complications in the extremities and probably contributes to improving quality of life and survival .The IVC can also be replaced with autologous veins, but this option is often impractical because of the difficulty in harvesting enough homologous material to substitute a long venous segment. Spiral vein grafts are not recommended owing to the elevated thrombogenicity of the surface exposed at the suture sites and the high risk of veins collapsing due to the positive intra-abdominal pressure . CurrentIn the case presented here, caval replacement was carried out in the context of a particular anatomic setting: horseshoe kidney. Central fusion of the kidneys occurs when the embryo is 5 to 12 mm, i.e., when the kidneys lie in the true pelvis. Horseshoe kidney is usually located between L3 and L5, lower than normal, because its ascent is impeded by the root of the inferior mesenteric artery. Also, its normal medial rotation is impeded, frequently resulting in an anomalous course of the ureters, which are often ectopic or retrocaval. The kidneys are fused together by a thick isthmus, generally parenchymatous and very often situated anterior to the aorta and the IVC. More rarely it may be found posterior to these vessels or in the interaortocaval space.The incidence of horseshoe kidney is between 1/400 and 1/800 persons, with a male-to-female ratio of about 2:1. In 90% of cases, the kidneys are fused at the lower poles, either symmetrically (midline fusion) or asymmetrically (L-shaped fusion) .The vascular network of horseshoe kidney varies widely:1. One renal artery per side (20%)2. One renal artery per side with a branch from the aorta to the isthmus (30%)3. Two arteries per side with an aortic branch to the isthmus (15%)4. Two arteries per side with one or more arteries from the iliac arteries to the isthmus (15%)5. Multiple renal arteries originating from the aorta, the iliac and the mesenteric arteries (20%) Because embryogenesis of the kidney occurs contemporaneously with its venous drainage system in the vena cava, there is a correlation between the presence of horseshoe kidney and venous abnormalities:1. Double IVC (0.2-3%)2. Postrenal left IVC (0.2-0.5%)3. Retroaortic left renal vein (2.1%)4. Circumaortic left renal vein (8.7%)5. Retrocaval or circumcaval ureters (0.001-0.1%)6. Preaortic iliac vein confluence (very rare) The association between non-seminomatous GCT and horseshoe kidney is a rare event and is seen in about 1.3% of patients requiring retroperitoneal lymph node dissection . To our As described by Evans et al.  and SogaScheduled caval replacement will entail careful preoperative imaging to evaluate the morphology and functionality of the isthmus of horseshoe kidney, its arterial vascularization and anatomic relationships with the ureters and venous structures. In young patients, the surgical goal is to achieve radical removal. Winter et al.  reportedResidual tumor removal is associated with high rates of mortality and comorbidity. In patients with horseshoe kidney and advanced stage non-seminomatous GCT (NSGCT) and post-chemotherapy retroperitoneal residual disease amenable to resection, the aims of surgical treatment are to achieve radical tumor removal, restore venous drainage, and preserve renal function without worsening the precarious health of a patient already severely debilitated by disease and treatment. These objectives can be reached with scrupulous evaluation of the individual case by a multidisciplinary team composed of an urologist, a vascular surgeon, and a medical oncologist. Caval replacement with a ringed PTFE should be performed without resecting the renal isthmus, owing to the frequent vascular anomalies associated with this disorder, and thus avoid the risk of bleeding complications.Written informed consent was obtained from the patient for the publication of this case presentation and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.AFP: Alpha-fetoprotein; BHCG: Beta-human chorionic gonadotropin; GCT: Germ cell tumor; IGCCCG: International Germ Cell Cancer Collaborative Group; IVC: Inferior vena cava; LDH: Lactate dehydrogenase; NSGCT: Non-seminomatous germ cell tumor; PET: Positron-emission tomography; PTFE: Polytetrafluoroethylene.The authors declare that they have no competing interests.PR, PG and PD wrote the manuscript. PR, DF, PD and PG performed surgery. BL and LC followed clinically the patient in the pre-and post-operative. GV was involved in the final editing. CC and PL still maintain contact with the patient and are aware of information necessary for the review of the work: their contribution to the revision of the work was essential and extremely valuable. All authors approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2490/14/40/prepub


Of the most common odontogenic cysts, radicular cysts represent cystic lesions of inflammatory origin and are managed either by surgical enucleation or by marsupialization. This article aims to report a clinical case of radicular cyst of a huge proportion treated with a conservative management. An illustration of possible complete healing of such a cystic periapical lesion in mixed dentition with conservation of vital structures is covered.How to cite this article: Somani R, Garewal R, Bhandari PP, Kumar D. When Nature plays upon an Ailment: A Case Report. Int J Clin Pediatr Dent 2012;5(1):61-63. Most radicular cysts seen in the primary dentition are associated with mandibular molars.3Radicular or residual cysts, also called as periapical cyst, are the most frequent odontogenic cysts of teeth bearing areas. Radicular cysts are rare in primary dentition,4 and is the conversion of a cyst into a pouch,5 it requires considerable aftercare and patient cooperation in keeping the cavity clean whilst it resolves and heals by relieving the internal pressure. This technique is indicated when cyst is in close proximity to vital structures and where there is significant risk of injury with enucleation. Several odontogenic developmental processes take place in a mixed dentition, hence, the surgical technique of choice should be the one with the least likelihood of iatrogenic damage. A case is presented for attempting the conservative treatment of odontogenic cyst and a workable protocol for this is applied.Marsupialization represents synonymous with Partsch’s operation,A 13-year-old male was reported to the Department of Pedodontics and Preventive Dentistry, DJ College of Dental Sciences and Research, Modinagar with a complaint of painless swelling in the right lower back tooth region since 2 months. Extraoral examination revealed a nontender and firm swelling on mandibular right side of face. Intraoral examination revealed a grossly decayed 85. Buccal vestibular expansion extending from distal aspect of 44 to mesial aspect of 46 was noticed. Panoramic radiograph revealed a single well-defined periapical unilocular radiolucency in relation to 85 region, with a radiopaque rim, of about 3 × 2 cm in size, extending from distal aspect of 42 to mesial root of 46 anteroposteriorly, involving tooth bud of 45 and displacing developing tooth bud of 43 inferiorly and mesially. The cyst caused tipping of the developing root of 44 and pushed it mesially causing the crown to incline distally . OcclusaAfter the blood investigations and consent of the guardian of the patient, the case was posted for a surgical management of the lesion under local anesthesia. Crevicular incision was made from mesial of 44 to distal of 46 along the gingival margin and mucoperiosteal flap was raised. The socket margins were widened to gain access to the cystic cavity and cavity was decompressed . The linThe surgical defect was packed with ribbon gauze soaked in betadine ointment. An obturator was prepared to promote bone healing, to prevent food accumulation and to maintain a patent surgical opening .Ribbon gauze dressing, dipped in betadine and glycerine to keep the cavity moist was replaced every alternate day for a period of 6 months. A bimonthly panoramic X-ray aided in keeping a track on the healing of site. A substantial reduction in the size of the cystic cavity as well as uprighting and promotion of eruption of 43 was seen in a 6 months follow-up panoramic X-ray .Histologically, a nonkeratinized stratified squamous epithelium, arcading pattern of proliferation and dense chronic inflammatory cell infiltrate were evident which were consistent with the diagnosis of radicular cyst .A lingual arch space maintainer was planned and placed to prevent mesial migration of 46 and to promote eruption of 44 into normal occlusion .Cysts are usually enucleated, where the cystic lining is separated from its inner bony surface and removed and cavity is allowed to fill with blood clot. However, a cyst can be marsupialized to relieve the internal pressure as well.In our case, the marsupialization technique applied with extraction of associated primary tooth and developing permanent tooth bud of 45 and preservation and promotion of erupting tooth bud of 43 appeared to be the most suitable treatment option due to lower morbidity and faster healing process, with a healing period of 6 months to our first phase of treatment. Placement of a lingual arch fixed space maintainer was our second phase of treatment.46 marsupialization consists of deroofing the outer wall of a cyst by surgical incision and establishing a permanent opening by suturing the remaining cystic wall to the mucosal surface followed by an obturator treatment.According to Neaverth,The main intention of obturator placement was to promote healing of the wound, followed by placement of lingual arch to prevent migration of 46 and provide ample space for 44 to erupt in normal occlusion.7An operator should maintain constriction with conservation of vital structures as much as he can when operating such cases.Marsupialization is favored because of lower morbidity and the fact that bony ingrowth occurs as the lesion shrinks in size, resulting in more normal bony contour. It is very likely that reduction of intracystic pressure is a key factor in the healing process. Although it is not known what percentage of radicular cyst can be expected to heal with only marsupialization, it is a viable treatment modality that bears consideration when treating large cystic lesions.


A 26-year-old man, previously healthy, presented with a 6-month history of bilateral sciatic pain and frequent constipation but no urinary symptoms. Physical examination revealed tenderness of the lumbosacral region with radiating pain along the sciatic nerve and the perinea without motor weakness of the inferior legs. No cutaneous lesions were noted especially no café-au-lait macules. Magnetic resonance imaging of the lumbosacral area showed multiple intracanalar lesions of the lumbosacral spine causing scalloping of posterior parts of all the sacral vertebrae and neural foraminal widening with bilateral and symmetric extension to the pelvic region. Biologic data were normal. Chest radiography followed by computed tomography revealed an axillary thoracic tumor (5 cm on diameter) which was biopsied and histologically identified as benign neurofibroma. Brain MRI showed no other cranial nerve tumors and there was no family history of neurocutaneous lesions. Because the patient had mild symptoms without neurological deficits, no surgery was performed for the sacral neurofibromas. Neurofibromas are one of the major characteristics of type 1 neurofibromatosis. They can develop from the Schwann cells or fibroblasts of any peripheral nerve. Massive intra-extraspinal cases characterized by dumbbell neurofibromas as in our case are rare and maybe asymptomatic for a long time. It is crucial to screen the entire spinal axis to detect all neurofibromas. Symptomatic cases justify surgical treatment but the decision for surgery and the surgical approach is quite complex because neurofibromas occupy a large spinal and extraspinal regions.


A baby girl referred from the newborn unit at day 1 of life with a pedunculated soft tissue 3x3.5x 3 cm mass located pre-axially on the palm at the base of her pollex finger.  The rest of the local and systemic examination was normal. Ultrasonography of the abdomen did not reveal any other congenital anomaly. The lesion was excised. Histopathological examination reported the lesion to be a fibrolipoma. The child was followed up for six months and no signs of recurrence were noted. Fibrolipoma is a benign variant of the family of lipomas which consist of other varieties such as angiolipoma, chondroid lipoma, myolipoma, spindle cell/pleomorphic lipoma, diffuse lipomatous proliferations (lipomatosis) and hipernoma. A pedunculated lesion, as reported here, on the palmar aspect in a newborn is rare and to date no reports have been found to exist in the literature. Other reported congenital sites of occurrence of a fibrolipoma include cornea, anal canal, nasopharynx, mesencephalic protuberation area and the middle ear. The consistency of this lesion varies from soft to firm, depending on the percentage of fibrous tissue and the depth of the tumor. The etiology and possible genetic role in the development of fibrolipoma is still unknown. It is characterised by adipose tissue interposed in a background of fibrous tissue. Treatment of fibrolipoma is entirely surgical and complete excision is the primary treatment of such lesions. [1-3]Source of Support: NilConflict of Interest: None


Cavernous angiomas usually occur in the parenchyma of both the supra and infratentorial compartments. At times, they can both clinically and radiologically mimic other dural-based lesions. We present a case of a patient with chronic occipital headaches, initially thought to have a meningioma, but proven to be a cavernoma with histological analysis. Cavernous angiomas, also known as cavernous malformations or cavernomas, are benign vascular lesions usually found in the brain parenchyma. With no gender predisposition, they affect between 0.4% to 0.6% of the population . ParenchA 42-year-old man presented with longstanding occipital headaches since childhood, which progressively worsened over the preceding six months. He denied diplopia, ataxia, nausea, or weakness in his arms or legs. On physical exam he was neurologically intact. Evaluation included computed tomography (CT) scan as well as magnetic resonance imaging (MRI) of the brain. The MRI revealed a 2.8 cm, enhancing, extra-axial mass along the floor of the right posterior cranial fossa that is demonstrated in Figures It was recommended to the patient that he undergo elective craniotomy and resection. Following a right suboccipital craniotomy, the lesion was noted to be encapsulated and crimson in color. There were no significant adhesions or evidence of invasion of the brain parenchyma and the lesion was easily removed en bloc and without complications. Histological analysis of the tumor demonstrated back-to-back, thick-walled, endothelial-lined, venous channels consistent with a dural-based cavernous angioma shown in the hematoxylin and eosin stain in Figure Postoperatively, the patient did very well and was discharged home on postoperative day two. At three months follow-up, he continues to do well and has complete resolution of his preoperative symptoms.Cavernous angiomas are benign vascular lesions comprised of enlarged, clustered, sinusoidal vessels without intervening parenchymal tissue and that are lined with epithelium . CavernoCavernous angiomas have a very distinctive appearance on MRI and on CT, often have punctate calcifications . The chaA few cases of dural-based cavernous malformations mimicking meningiomas or other neoplastic processes have been reported in the literature -13. The Previous cases have described lesions similar to ours with cavernous angiomas being mistaken for neoplastic lesions such as meningiomas and also neoplastic lesions mistaken preoperatively for cavernous angiomas -13, 16. With the advancement of radiological techniques, the preoperative diagnosis of intracranial lesions continues to improve. However, as in the case reported herein, the definitive diagnosis continues to be the pathological specimen.


Focal epithelial hyperplasia (FEH), or Heck’s disease, is a rare disease of the oral mucosa associated with infection by some subtypes of human papilloma virus, especially subtypes 13 or 32. The disease is predominantly found in children and adolescents with indigenous heritage, but other ethnic groups can be affected worldwide. To the best of the authors’ knowledge, it has not been reported in Brazil’s elderly population. This article describes a case of FEH in a 57-year-old Brazilian patient presenting since childhood, with multiple lesions in the lips, buccal mucosa and tongue. The solitary tongue lesion underwent excisional biopsy and the histopathological analysis showed parakeratosis, acanthosis, rete pegs with a club-shaped appearance, koilocytosis and the presence of mitosoid cells. These microscopic findings in conjunction with clinical presentation were sufficient to establish the accurate diagnosis of FEH. Polymerase chain reaction (PCR) was performed, but no one human papillomavirus (HPV) subtype could be identified. Clinicians must be aware of this rare oral disease, which can even affect elderly patients, as we described here. Treatment may be indicated in selected cases due to esthetic and/or functional problems. Focal epithelial hyperplasia (FEH), also known as Heck’s disease, is a rare benign disease first described in the English literature by Archard et al 1965) as multiple oral lesions affecting children belonging to the Navajo and other Native American tribes 965 as mu. FurtherThe concentration of FEH cases in some geographical areas with familial occurrence suggests environmental influences and genetic predisposition in its etiology. However, Heck’s disease is primarily considered an infectious illness caused by human papillomavirus (HPV), especially 13 and 32 subtypes , 8.FEH is predominantly found in children and adolescents with indigenous heritage and has a variable female predilection . ClinicaThe diagnosis is based on clinical grounds, and treatment is usually unnecessary since most of lesions regress spontaneously and there is no tendency to malignant transformation , 12. TheA brown-skinned 57-year-old Brazilian male complaining of numerous lumps in his upper lip and tongue was referred to the oral medicine center at the municipal health service of Palmas-Tocantins, Brazil. The patient reported that he had noticed such lesions since childhood and that they were painless. However, the lingual lump was often traumatized during mastication, causing bleeding and difficulty in feeding. His past medical history was non-contributory and the patient denied the presence of similar lesions in other family members.Extraoral examination did not show abnormalities. Intraoral examination revealed multiple lip lesions presenting themselves as normochromic, sessile and scattered papules and nodules, with a tendency to coalesce into plaques yielding double lip appearance . On the The clinical diagnosis was FEH. The patient denied indigenous descent, although he was born in the northern region of Brazil, in which there are many Indian tribes. With the purpose of diagnostic confirmation and elimination of chewing discomfort, the excisional biopsy of the tongue nodule was performed under local anesthesia. There were no complications during the surgery nor during the postoperative period .Histopathological analysis showed stratified squamous epithelium with parakeratosis, acanthosis and horizontal fusion of elongated epithelial rete ridges. Some rete pegs got a club-shaped appearance. The spinous layer exhibited individual keratinocytes with nuclear fragmentation resembling a mitotic figure (mitosoid cells) and other groups of cells with pycnotic or absent nuclei and clear cytoplasms (koilocytes). The underlying connective tissue consisted of abundant and wavy collagen bundles interspersed with fibroblast nuclei and blood vessels, some of which congested. Scarce inflammatory cells could be seen in the subepithelial area . These dIn other examinations to identify the presumed HPV subtypes in the specimen, paraffin-embedded block was submitted to polymerase chain reaction (PCR) analysis. First, DNA was isolated from the 5 μm sections of formalin-fixed, paraffin-embedded tissue specimens. The samples were deparaffinised in microcentrifuge tubes, then digested by 400 mg/mL proteinase-K in 200 mL TE10E1 buffer at 55 °C for 24 - 48 h. After heat inactivation of the enzyme, DNA was isolated by using the PureGene Extraction kit  according to the manufacturer’s protocol. PCR amplification with the L1 consensus primers Gp5+/Gp6+ gave an expected PCR product with approximately 140 bp. These primers allow the detection of a broad spectrum of mucosotropic HPV genotypes . However, no HPV DNA could be detected in the examined material.The patient reported significant improvement in chewing after removal of the tongue nodule and there was no sign of recurrence after 36-month follow-up . As the FEH is a benign oral disease caused by HPV, especially 13 and 32 subtypes , 8, 12. The occurrence of FEH in patients older than 45 years is extremely rare in the Americas. In 1994, Carlos and Sedano , 13. TheOn the other hand, Axell et al  studied As there are many indigenous groups in Brazil and there is great miscegenation, more FEH reports among adults and the elderly in the Brazilian population would be expected. Indeed, the patient reported here may have unknown or denied indigenous descent, since he is from northern Brazil where there are many Indian tribes. It is noteworthy that Indian heritage is still regarded with a certain prejudice in Brazil. Factors that could explain the lack of FEH reports in adults and the elderly in Brazil include a particular tendency to spontaneous regression and the difficulty of access of poor people to health services, since FEH usually affects individuals who live in poverty or are of low socioeconomic status , 9, 11. The identification of Heck’s disease is strongly suggested by the clinical findings , but hisAlthough PCR analysis is a highly specific method and considered the gold standard for the detection of HPV , 21, our2 laser, electrocoagulation, chemical agents  and immunostimulants  [Treatment of FEH is not always required since the lesions are asymptomatic and may undergo spontaneous remission and there is no tendency to malignant transformation , 24. We erferon) , 24.In conclusion, FEH is a rare benign disease of the oral mucosa associated with HPV infection. The disease is usually diagnosed in young people of indigenous heritage, but other ethnic groups and older patients may be affected. Herein, we reported a case of FEH in a 57-year-old Brazilian male, presenting with extensive lesions in the lips, buccal mucosa and tongue. Hence, clinicians must be aware of such rare presentations and accomplish a prompt diagnosis and appropriate treatment according to esthetic and functional purposes.


Background: Histiocytic sarcoma (HS) is a rare hematologic neoplasm with a few hundred cases having been described to date.Case Presentation: We report the case of a 56-year-old woman with a history of hepatitis C infection and chronic kidney disease (CKD), submitted to a kidney transplant in 1984, under maintenance immunosuppression with prednisone and azathioprine. Patient presented with a relentlessly growing mass on her right front thorax. It was painless, smooth, and adherent to the deep muscle. Laboratory studies were unremarkable. Ultrasonography and computerized tomography (CT) scan revealed a highly vascularized heterogeneous mass (8×9 cm), with a necrotic centre. Positron emission tomography (PET) scan demonstrated multiple thoracic, abdominal, and pelvic nodules. Histology revealed a highly undifferentiated HS . In spite of having started treatment with etoposide and thalidomide, no clinical response was achieved and the patient died three months later.Conclusions: To the authors’ knowledge, this is the first described case of HS in a solid organ transplant patient. A World Health Organization (WHO) expert panel stated histiocytic sarcoma is likely to be underdiagnosed. The case herein presented provides evidence to the existence of this rare disease in solid organ transplant recipients. Therefore this diagnosis should be considered in the presence of an undifferentiated sarcoma (vimentin and CD68 positive). Moreover, the presented case highlights the importance of positron emission tomography (PET) scan for the diagnosis of disseminated disease. Post-transplant lymphoproliferative disease (PTLD) incidence among solid organ recipients ranges from 0.56%-1.8% , and is Histiocytic sarcoma (HS) is a rare hematologic neoplastic disease with only a few hundred cases reported worldwide, with a wide range of clinical presentations and manifestations described -7. This Herein we present a report of a case an HS in a kidney transplant recipient. To the best of authors’ knowledge, this is the first report of an HS in a solid organ recipient not related to Epstein-Barr virus (EBV) infection.A 56-year-old woman with a history of chronic hepatitis C virus (HCV) infection diagnosed with chronic kidney disease (CKD) of unknown aetiology at the age of 27 years, was submitted to a deceased-donor kidney transplant in 1984, and maintained under immunosuppression with prednisone and azathioprine. Post-transplant period was complicated with new-onset diabetes after transplantation (NODAT), and noninvasive cutaneous squamous cell carcinoma diagnosed in 2004 – surgically removed and without evidence of relapse. The remaining transplant period was unremarkable with a serum creatinine of 0.8 mg/dL in December 2011.Staphylococcus aureus, successfully treated with cephazolin.Patient presented in 2012 to the emergency department due to fever of unknown origin. She also complained of a painless, smooth, and adherent to the deep muscle relentlessly growing mass on her right front thorax. Physical exploration was otherwise unremarkable – no other masses, palpable lymph nodes, or splenomegaly were found. Normal leucocyte count and haemoglobin levels were found, as were lactic dehydrogenase (LDH) and bilirubin levels. EBV serology was positive for IgG but negative for IgM. Both ultrasound and computerized tomography (CT) scan  and B reHistology of the biopsied lesion revealed a malignant undifferentiated large cell neoplasm. No lymphoid tissue was identified. The immunohistochemical study was positive for vimentin, CD68, CD99, and CD4, and negative for all other tumour markers , consistA positron emission tomography (PET) scan, later performed, revealed a large axillary mass, as well as multiple thoracic, abdominal, and pelvic nodules C. EvidenThe cumulative incidence of PTLD has been reported to approach 1.8% , and repHS is a rare neoplastic disease of unknown aetiology, accounting for less than 1% of all hematolymphoid neoplasms . It mostPreviously known as “true histiocytic lymphoma,” HS is a diagnosis of exclusion. The classification according to the 2008 World Health Organization (WHO) requires the verification of the histiocytic lineage and the exclusion of other, poorly differentiated, large-cell malignancies. Even though, the presence of B-cell markers no longer excludes its diagnosis .HS immunohistochemical profile most often expresses lysosome-associated (macrophage or histiocytic) markers, CD68 (in 100% of cases) and lysozyme (in 94% of cases) . AdditioSteroids, a cornerstone of immunosuppression in solid organ transplantation, inhibit gene induction by blocking the translocation of nuclear factor-κB (NFκB) from cytoplasm to nucleus. As a result, gene transcription and release of inflammatory cytokines is impaired . AzathioImmunosuppression vintage was over 25 years in the presented case. To the authors’ comprehension, the prolonged gene transcription inhibition due to long-term treatment with steroids enhanced the risk for mutation in the B and/or T-cells, possibly causing a differentiation into histiocytes or macrophages. Moreover, the association with azathioprine could have enhanced the risk for translational mutations and proliferation of a mutated monoclonal clone. Nevertheless, a large number of kidney transplant recipients are under an immunosuppression scheme similar to the one reported, and still the incidence of HS is very low.To the best of authors’ knowledge, this is the first report of HS in a solid organ recipient without evidence of EBV infection. In 1985, Kramer et al  describeThis report highlights two important features: first, the importance of PET scan for the diagnosis of disseminated disease. Neither CT scan nor bone scintigraphy were able to identify the presence of thoracic, abdominal, or pelvic metastasis. Second, the need for increased awareness for HS, since an overconfident diagnosis of the other more frequent neoplasms likely results in a reduced recognition of HS’s true incidence.PVA conducted manuscript redaction, CD, histological diagnosis, and picture selection. PA; Prepared draft. HNS; Patient management. MA; Patient management. SP; Manuscript revision. LSM; Manuscript revision and patient management. LD; Manuscript revision. AR; Patient follow-up. RV; Histological diagnosis, and pathology report. AC; Manuscript revision. ACH; Manuscript revision.None of the contributing authors have any conflict of interest, including specific financial interests or relationships and affiliations relevant to the subject matter or materials discussed in the manuscript.None.


A young adult presented with a four-month long history of hemoptysis. A computed tomography (CT) scan showed a mid-trachea lesion . A flexiLobular capillary hemangioma (LCH) is typically found on cutaneous and oronasal mucosa. Tracheobronchial LCH is a rarity . HemoptyBronchoscopic ablative intervention often results in a cure although past reports had a follow-up of one year or less. Ablative modalities included endoscopic resection with Nd-YAG laser, argon plasma coagulation, electrocautery, cryotherapy, and forceps. In one reported case of tumor recurrence, brachytherapy was applied.


Glioblastoma multiforme located in the posterior fossa is extremely rare with a frequency up to 3.4%. Compared with glioblastoma of the hemispheres the prognosis of infratentorial glioblastoma seems to be slightly better. Absence of brainstem invasion and low expression rates of epidermal growth factor receptor are described as factors for long-time survival due to the higher radiosensitivity of these tumors.In this case study, we report a German female patient with an exophytic glioblastoma multiforme arising from the cerebellar tonsil and a secondary spinal manifestation. Furthermore, the tumor showed no O (6)-Methylguanine-DNA methyltransferase promotor-hypermethylation and no isocitrate dehydrogenase 1 mutations. All these signs are accompanied by significantly shorter median overall survival. A long-term tumor control of the spinal metastases was achieved by a combined temozolomide/bevacizumab and irradiation therapy, as part of a standard care administered by the treating physician team.To our knowledge this is the first published case of a combined cerebellar exophytic glioblastoma with a subsequent solid spinal manifestation. Furthermore this case demonstrates a benefit undergoing this special adjuvant therapy regime in terms of overall survival. Due to the limited overall prognosis of the disease, spinal manifestations of glioma are rarely clinically relevant. The results of our instructive case, however, with a positive effect on both life quality and survival warrant treating future patients in the frame of a prospective clinical study. Glioblastoma mulitforme (GBM) is the most common primary tumor of the central nervous system (CNS) comprising approximately 50% of all primary intracranial tumors . It is lExtracranial metastasis from GBM is rare, with a reported frequency of only 0.44%. In the reported cases, metastases occurred in regional lymph nodes, lungs, pleura, and occasionally in the bone and liver. Spinal metastases are clinically rarely detected . Spinal A 55-year-old German woman presented at our department with a short history of persistent headache, vertigo, nausea, and a generalized seizure. Magnetic resonance imaging (MRI) showed a well-defined tumor approximately 1.7 × 3.7 × 2.6 cm in size, located at the cranio-cervical junction with dorsal compression of the medulla oblongata, and in close relation to the right vertebral artery. The tumor was hyperintense on T2-weighted images and displayed an intense, nearly homogeneous gadolinium (Gd) enhancement. Due to its close contact to the dura, the initial tentative neuroradiological diagnosis was an infratentorial meningioma [Figure The histological examination on formalin fixed and paraffin embedded tissue confirmed the diagnosis of a glioblastoma multiforme WHO IV with a typical high cellular pleomorphism, microvascular proliferations, and necrotic areas Figure  but with2 body surface). Adjuvant temozolomide chemotherapy according to the Stupp regimen (200 mg/m2 body surface) [The postoperative course of the patient was uneventful. She underwent radiochemotherapy with an over-all dose of 54 Gy applied in fractions of 1,8 Gy twice a day over a period of 3 weeks and concomitant temozolomide chemotherapy  nodules throughout the whole spinal column [Figure Two weeks later, the patient described lower limb pain, reduced sensitivity, and micturition disorders accompanied by progressive dysphagia. The spinal MRI revealed a small tumor located dorsal to the 4n Figure . DiagnosRadiotherapy was administered to the lumbar spinal mass with an over-all dose of 54 Gy administered in 18 sessions. The cerebellar relapse was treated likewise with 30 Gy in 5 sessions. Irradiation was combined with continuous temozolomide chemotherapy . After therapy the patient presented in an improved overall condition. She still described dysesthesia in the lower limbs but showed a normal function of the bladder and she was also able to walk without aid.After radio-/chemotherapy the patient consented to off-label intravenous bevacizumab antibody-therapy with 15 mg/kg d1 q21d (according 800 mg bevacizumab) combined with a continuous application of temozolomide 100 mg per day over 25 bouts of therapy, administered by the treating physician team as part of a standard care in case of disseminated GBM.This procedure is in consistent with the institutional ethics committee instructions according to the Federal Institute for Drugs and Medical Devices. The use is permitted within therapeutic flexibility of the physician in cases of life-threatening diseases without alternative therapeutic option and evidence for therapeutic success (§35bSGBV).After 3 months of combined temozolomide/bevacizumab therapy the MRI scans revealed a decrease of cerebellar gd - enhancement and a stable clinical condition concerning the spinal manifestation such as dysesthesia of the lower limbs.33 months after the first operation and 12 months after spinal irradiation the, MRI scans still showed stable disease without evidence of further tumor growth or new CNS manifestations Figure  being awThe most recent MRI scan, 38 months after the first operation, revealed a progressive disease with extended meningeosis carcinomatosa around the brainstem and the cervical spinal cord. Because of the poor clinical condition, further chemotherapy and bevacizumab were stopped in favour of best supportive care.Brain stem and spinal metastases of supratentorial glioblastoma multiforme are well known in the late course of the disease, but are rarely symptomatic. In a hospital series of 600 GBM patients, the incidence of symptomatic spinal metastases was about 2% . To dateet al. described combined radiotherapy and bevacizumab in a case of supratentorial GBM with spinal manifestation. This patient died 6 months following diagnosis of metastasis and 3 months after the end of therapy [Fiorentino  therapy . No specA technical enquiry to Hoffmann-La Roche Ltd. Basel Switzerland by the corresponding author revealed that there is no data available concerning the bioavailability of bevacizumab in the cerebrospinal fluid (CSF).Despite the fact that the prognosis of GBM of the posterior fossa seems to be slightly better than GBM of the cerebral hemispheres, we confirmed in our case an absence of IDH -1 mutation, a negative MGMT methylation, and a meningeosis carcinomatosa as a “signum malum omnis”. Altogether, these are negative predictors of a good response to chemotherapy and long overall survival.On the other hand, an off-label-use of intravenous bevacizumab combined with temozolomide and radiotherapy arrested a further tumor growth at least for 33 months in the presented case.Exophytic cerebellar glioblastoma of the posterior fossa is a rare occurrence. This is also the first report of a secondary spinal manifestation of this entity.This report highlights the potential of a combined adjuvant therapy with bevacizumab to achieve a long-term tumor control in the case of spinal manifestation of an exophytic glioblastoma multiforme of the posterior fossa.Due to the limited overall prognosis of the disease, spinal manifestations of glioma are rarely clinically relevant.This is a single case study, thus we cannot postulate this therapy as standard therapy. The results of our instructive case, however, with a positive effect on both life quality and survival warrant treating future patients in the frame of a prospective clinical study.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.


Carcinoid tumors are slow-growing tumors originating in the neuroendocrine cells, and occur most frequently within the gastrointestinal tract. Although the liver is the most common site for metastatic carcinoid tumors, primary hepatic carcinoid tumors are exceedingly rare and reports of the imaging findings have been very scarce. We herein report imaging findings with an emphasis on magnetic resonance imaging in two cases of primary hepatic carcinoid tumors. In both cases, the tumors showed cystic areas with hemorrhagic components and early enhanced solid areas. Carcinoid tumors are slow-growing tumors originating in the neuroendocrine cells, and occur most frequently within the gastrointestinal tract  imaging. To the best of our knowledge, there have been no report describing the MR imaging findings with the hepatocyte-specific contrast media like one of our cases.A 70-year-old male with past medical history of a squamous cell carcinoma of the skin underwent a computed tomography (CT) examination for the assessment of distant metastasis. He was asymptomatic. Unenhanced CT showed a heterogeneous hypodense mass mainly in the posterior segment of the right hepatic lobe. On enhanced CT, multiple cystic areas without contrast enhancement became clear by the enhancing solid areas in the hepatic mass. His laboratory data showed high levels of serum γ-glutamyl transpeptidase (75 IU/L) and leucine aminopeptidase 136 IU/L). Tumor marker assays showed high levels of serum carcinoembryonic antigen (5.7 ng/mL) and carbohydrate antigen 19-9 (154 U/mL). Abdominal ultrasound showed the mainly hyperechoic mass with multiple cystic areas. Abdominal MR imaging showed the hepatic mass with solid and multiple cystic areas. T2-weighted MR images showed multiple cystic areas with shading in the hepatic mass . Abdominal unenhanced CT showed a heterogeneous hypodense mass 12 cm in diameter, mainly in the right lobe of the liver  in diagnostic imaging, this may be difficult owing to the rarity of this tumor. However, hepatic masses including cystic areas with hemorrhagic components and early enhanced solid areas on MR imaging should thus lead to a suspicion of a primary hepatic carcinoid tumor.


Posterior reversible encephalopathy syndrome (PRES) is an unusual condition typified by acute visual impairment caused by sudden, marked parieto-occipital vasogenic edema. Thought to be inflammatory in origin, it has been described in patients undergoing chemotherapy, with autoimmune disease, and in some infections. We report a case of PRES that occurred one week after an episode of acute pancreatitis in an otherwise healthy 40-year-old female. There was progressive visual impairment over a 24-hour period with almost complete visual loss, with characteristic findings on magnetic resonance imaging. After treatment with steroids, the visual loss recovered. Clinicians should retain an index of suspicion of this rare condition in patients with visual impairment after acute pancreatitis. A 40-year-old female presented to our emergency department with sudden visual loss over a 24-hour period. She was otherwise healthy, but had been admitted two weeks previously with an episode of acute pancreatitis secondary to alcohol intake from which she had recovered uneventfully, and without any obvious sequelae. An urgent magnetic resonance imaging (MRI) scan was performed. This revealed symmetrical areas of hypoattenuation in both posterior parieto-occipital and cerebellar regions , 2. She PRES is extremely rare, and usually diagnosed by a history of sudden visual impairment in the presence of specific radiological changes on MRI. Bilateral symmetrical hypodensitities in the parieto-occipital areas and cerebellar hemispheres on imaging are characteristic. The condition has been associated with chemotherapy, hypertension, infection and autoimmune disease.1It is thought to occur from temporary impairment of the blood brain barrier causing vasogenic edema with symptoms of reduced consciousness, seizures, headaches, and typically visual problems.Our case is unusual, as PRES caused by pancreatitis has only been reported in very sick patients with other comorbidities. It probably occurred in this case as a result of the systemic inflammatory response.6Whilst pancreatitis itself can be life threatening, this case reminds clinicians of unusual complications that can occur after discharge of patients who seem to have recovered from the disease.


Around 10 per cent of catecholamine-secreting tumours can be found outside the adrenal medulla (paraganglioma). We report a case of a functional sporadic paraganglioma that was localized lateral to the prostate without causing lower urinary tract symptoms.A 76-year old male with an extensive history of cardiovascular disease suffered from hypertension and an unexplained hypochromic microcytic anaemia for years before the coincidental discovery of a 2.5 × 3.5 cm periprostatic mass upon abdominal contrast-enhanced CT scanning. Transrectal biopsies revealed a paraganglioma. The urinary levels of the catecholamine metabolites were found increased. The paraganglioma showed uptake of iodine-123-metaiodobenzylguanidine by SPECT scanning, indicating a solitary lesion. Successful preperitoneal endoscopic resection of the tumour was performed, which resulted in a decrease in blood pressure and a normalization of the urinary catecholamine metabolites. None of the to date known genetic mutations that have been shown to relate to the existence of paragangliomas were identified in the current case.An intra- or periprostatic localization of a paraganglioma is very rare. We reviewed the literature and found 6 other cases. Three of the described cases presented with lower urinary tract symptoms. In these three patients, the tumour had a size of 4 cm or larger and in 67 per cent of these cases the paragangliomas were situated within the prostate. The periprostatic region might be considered as a possible location for paragangliomas, especially in the presence of lower urinary tract symptoms even though they were absent in the current case. Pheochromocytomas and paragangliomas are characterized by a large diversity of clinical features and as a result can remain unrecognized for years. General features of these catecholamine-producing tumours, which originate from the sympathetic nervous system, are related to the response to stress, i.e. paroxysms of hypertension, paleness, headache and sweating attacks . PheochrIn this article, we describe a rare case of a 76-year old male who presented with a sporadic functional paraganglioma that was situated at a very unusual localization, namely lateral to the prostate. A review of the available literature on this specific topic is provided.A 76-year old man was referred to the outpatient clinic of the internal medicine department of our hospital because of persisting iron-deficiency anaemia that had been present since 2010. The patient had an extensive history of cardiovascular disease. In 1985 he suffered from an acute myocardial infarction for which he underwent percutaneous transluminal coronary angioplasty of the circumflex branch of the left coronary artery. He was treated for two thromboembolic events: a deep venous thrombosis of his right leg in 2008 and pulmonary embolism in 2009. Over the past years he developed a therapy-resistant hypertension, which was, which was presumed initially due to white coat phenomenon. Figure Upon arrival at the medical outpatient clinic, he complained of dyspnea on exertion that had been stable since he was diagnosed with decompensated heart failure. He also complained of paroxysmal headaches without aberrant palpitations, increased transpiration or paleness. In the context of the persisting anaemia, which was the initial reason for referral, he had not noticed a change in his defecation pattern over the past years and especially no melaena or bloody stool had been present. Further laboratory investigation showed a low mean corpuscular volume and hemoglobin concentration with a low serum iron and ferritin, but a high iron-binding capacity, indicative of hypochromic microcytic anaemia in the context of an iron deficiency Table . The abs123I-MIBG), a molecule similar to norepinephrine and taken up in adrenergic tissue showed pathological uptake solely within the paraganglioma  and a carcinoembryonic antigen (CEA) concentration of 3.3 μg/L (reference in non-smokers < 5.0 μg/L), medullary thyroid cancer became less likely. Since extra-adrenal pheochromocytomas have been shown to be more prevalent in patients with a hereditary paraganglioma syndrome, genetic screening was indicated. Various schemes for genetic screening based on risk factors for mutations have been proposed, which include screening for RET, SDHB, SDHD and VHL in case of abdominal paragangliomas [RET, MAX, SDHA, SDHAF2, SDHB, SDHC, SDHD, TMEM127 and VHL; MRC-Holland kit P226-B2), but none showed pathogenic mutations in their coding sequence or splice sites.When he presented at the urology department, his serum total prostate-specific antigen concentration was 2.5 μg/L . At that time, the patient had no lower urinary tract symptoms and his headache had not worsened during micturition. Cystoscopy revealed an impression of the right bladder wall with no mucosal pathology. Three transrectal ultrasonography-guided biopsies of the lesion where obtained and histological analysis of the material with hematoxylin staining showed partly tube-forming tumorous epithelial cells with intensely stained nuclei that differed in size Figure 23I-MIBG,The patiënt was scheduled for preperitoneal endoscopic resection of the paraganglioma and Figure Here we documented the case of a 76-year old male with symptoms of sustained therapy-resistant hypertension, unexplained microcytic anaemia and a catecholamine-producing tumour near the prostate that had remained unrecognized for years. The paraganglioma was discovered incidentally on a screening abdominal CT scanning made during the work-up for his unexplained and presumably unrelated anaemia.To the best of our knowledge, this is the seventh case in history reporting a paraganglioma that is localized in or in close proximity to the prostate -10. CompBesides the classical triad of paroxysmal palpitations, headache and sweating, which are direct signs of sympathetic activation , pheochrExtra-adrenal localization of catecholamine-secreting tumours is more often in the context of a genetic syndrome and it is therefore recommended to screen these patients for underlying genetic mutations . The curIn conclusion, we describe the case of a functional periprostatic pheochromocytoma, which is an unusual extra-adrenal site of presentation for this type of neoplasia. The tumour was recognized on a contrast-enhanced CT scan performed in the work up for unexplained and presumably unrelated microcytic anaemia. Although very rare, the periprostatic region should be considered as a possible location for paragangliomas, especially in the presence of lower urinary tract symptoms even though they were absent in the current case.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.NCAM: Neural cell adhesion molecule; PSA: Prostate-specific antigen; PSAP: Prostate-specificacid phosphatase; VMA: Vanillyl mandelic acid; MEN: Multiple endocrine neoplasia; SPECT: Single photon emission computed tomography; MIBG: Iodine-123-metaiodobenzylguanidine; PRRT: Peptide receptor radionuclide therapy; MIBI: Methoxyisobutylisonitrile; CEA: Carcinoembryonic antigen; RET: Ret proto-oncogene; MAX: Myc-associated factor X; SDH: Succinate dehydrogenase; TMEM127: Transmembrane protein 127; VHL: von Hippel-Lindau.None of the authors have competing interests to declare.JK: contributed to study conception and design, acquisition of the data, analysis of the data, drafting and critically revising of the manuscript and has given final approval of the version to be published. ZAC: contributed to acquisition of the data, drafting and critically revising of the manuscript and has given final approval of the version to be published. TAR: contributed to acquisition of the data, drafting and critically revising of the manuscript and has given final approval of the version to be published. APJH: contributed to acquisition of the data, drafting and critically revising of the manuscript and has given final approval of the version to be published. All authors read and approved the final manuscript.The authors have nothing to disclose.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1472-6823/13/55/prepub


Myofibroblastoma is a benign tumor composed of spindle cells in clusters and fascicles. To date, only three cases of intracranial myofibroblastoma have been reported. The present study reports the case of a 47-year-old female with meningeal myofibroblastoma. The patient had a history of ovarian cyst resection and presented with paroxysmal mild headaches that had been apparent for 4 years. Magnetic resonance imaging disclosed a well-circumscribed mass in the left frontal lobe. A resection of the mass was performed. Abundant fascicular clusters of spindle- and oval-shaped cells were found by conventional histopathology. Immunohistochemical staining demonstrated that these cells were strongly positive for smooth muscle actin, weakly positive for epithelial membrane antigen and negative for cluster of differentiation (CD)117, CD34, S-100 or desmin, with a Ki-67 index of >10%. These results supported the diagnosis of myofibroblastoma. No recurrence of the mass was found during the 24-month follow-up period. Overall, the patient exhibited a rare type of meningeal neoplasm. Resection of the tumor proved to be successful and no recurrence were found. Histopathological and immunohistochemical staining is crucial to form a diagnosis. To the best of our knowledge, the present study is the first to show the presence of myofibroblastoma in the left frontal lobe. Myofibroblastoma is a benign and rare mesenchymal neoplasm composed of spindle cells in clusters and fascicles, with interspersed bands of hyalinized collagen ,2. The mA 47-year-old female with a history of ovarian cyst resection presented to The First Affiliated Hospital  with paroxysmal mild headaches that had been apparent for 4 years. The headaches had increased in intensity over the past 6 days. Computed tomography (CT) revealed a low-density mass lesion in the frontal lobe. The physical examination was normal. The analysis of tumor markers showed no abnormal findings. A cerebro-spinal fluid examination disclosed a slightly elevated level of protein at 0.50 g/l , with no other abnormal findings. Magnetic resonance imaging (MRI) disclosed a well-circumscribed mass in the left frontal lobe . The cleA resection of the mass was performed; the mass appeared to be dusty pink color and had a resilient structure, with abundant blood supply. Significant adhesion to the surrounding brain tissue was present, together with severe edema surrounding the mass. The base of the mass was attached to the convex dura, with focal localization on the dura of the frontal basal section. Diagnosis of the frozen section performed at the time of surgery disclosed a suspected myofibroblastoma. The patient’s headache had resolved at 2 weeks post-surgery. CT revealed postoperative manifestation and no evidence of lesion recurrence. Anti-epilepsy therapy was recommended when the patient was discharged from the hospital. The patient recovered well, with no evidence of mass recurrence on MRI to date at 24-months post-surgery .The histopathological findings revealed abundant fascicular clusters of spindle- and oval-shaped cells, which were arranged in interlaced or swirled patterns. Elongated to oval-shaped nuclei with inconspicuous nucleoli were another distinguishing feature. Finely-dispersed chromatin and poorly-defined cytoplasm were also observed. Mitotic figures were rare. Mucinous degeneration and necrosis were observed in the eosinophilic area, while an occasional lymphocyte and neutrophil were seen between tumor cells .Immunohistochemical staining demonstrated that the majority of the tumor cells were strongly positive for smooth muscle actin SMA; , the Ki-Studies on myofibroblastoma in the central nervous system are extremely rare. To the best of our knowledge, only three cases of intracranial myofibroblastoma have previously been reported –9. The dMyofibroblastoma is a well-circumscribed benign tumor. Histopathological findings demonstrate that the tumor is composed of spindle cells in clusters and fascicles, with thick interspersed hyalinized collagen bands. It has features of fibroblasts and smooth muscle cells, with frequent mitoses. The cells are also characterized by elongated to oval-shaped nuclei, irregular nuclear contours, finely-dispersed chromatin and poorly-defined cytoplasm ,2,7,9,10The clinical manifestations of intracranial myofibroblastoma are similar to meningioma and include intracranial hypertension, skull destruction and the presence of systematic symptoms. Headaches caused by intracranial hypertension subsequent to the effects of a mass are extremely common. The masses may grow slowly, as all patients tend to have a long medical history prior to their admittance to hospital. Intratumoral hemorrhage may also be a feature of the mass .et al , fibrous meningiomas and hemangiopericytomas.SFTs are rare tumors that can also occur in the meninges. Histopathological findings demonstrate the presence of numerous monomorphic spindle- or oval-shaped cells and diffuse intercellular reticulin fibers. These findings are similar to those of myofibroblastoma. However, unlike for myofibroblastoma, branching hemangiopericytoma-like vessels and rare mitotic figures are characteristics that are also present ,13. ImmuFibrous meningioma is another type of meningioma. Unlike myofibroblastoma, fibrous meningiomas are glycogen-containing tumors. Additionally, a storiform pattern, psammoma body and collagen calcification are defining characteristics ,18. ImmuMeningeal hemangiopericytomas (HPCs) are also meningeal neoplasms, and are composed of oval- to spindle-shaped cells, with dense intercellular reticulin fibers. However, unlike myofibroblastomas, HPCs are prone to multiple recurrences and eventual metastasis. Another difference can be found in the existence of numerous small blood vessels ,19,20. HThe prognosis of meningeal myofibroblastoma is optimistic. The masses are slow-growing and the histopathology findings show no evidence of malignancy. In the present patient, the resection of the tumor proved to be a successful treatment and no recurrence was found, similar to the two previous cases ,9.The present patient exhibited a rare type of meningeal neoplasm. Histopathological and immunohistochemical staining is crucial to identify the diagnosis of a myofibroblastoma. Further study is required to identify the origin of the tumor and the association between the tumor and other meningeal neoplasms, such as SFT.


Rationale: Eyelids are very susceptible area for non-melanoma skin cancers; among that, basal cell carcinoma has the highest incidence  and 50-60% of eyelid basal cell carcinomas appear on inferior eyelid. Objective: To analyze clinical features of inferior eyelid basal cell carcinoma and to determine the efficacy of surgical treatment with frozen sectioncontrolled margins and methods of primary reconstruction of defects.Methods: A review of medical records of cases with primary inferior eyelid basal cell carcinoma treated by surgical excision with urgent histopathology controlled margins by FS technique, doubled by paraffin examination from October 2011 to October 2014. After histopathology confirmation of tumor free margins, proper inferior eyelid reconstruction was performed.Results: The review resulted in 36 patients with 36 lesions analyzed by clinical, histopatological and therapeuticalaspectswith a mean follow-up of 20 months. All lesions were primary BCC affecting inferior eyelid. There were no recurrence in the follow-up period. Inferior eyelid reconstruction techniques were direct closure for small defects and complex techniques for defects more than one third of eyelid length. Discussion: Appropriate eyelid examination is mandatory in any routine ophthalmic check-up. Clinical signs suggestive of BCC should be familiar to ophthalmologist in order to have an early diagnosis and treatment for these tumors. Surgical treatment with FS controlled excision followed by eyelid reconstruction is an efficient treatment for inferior eyelid BCC.Abbreviations: basal cell carcinoma (BCC); frozen section (FS);Mohs micrographic surgery (MMS). Left untreated, BCC can have not only serious aesthetic but also functional meanings because of invasion to the orbit and craniofacial structures.The incidence of non-melanoma skin cancer has rapidly increased in the last years .Five to ten percent of all skin cancers occur on the eyelid. BCC is the most frequent skin cancer in periocular region, accounting for 90% of eyelid tumors. [2] Surgical treatment offers best curability rates and remains gold standard for BCC. Surgical resections used are excision with predetermined margins, FS controlled excision and Mohs micrographic surgery (MMS) with cure rates between 80 and 99%. Reviewing published reports regarding the treatment of basal cell eyelid tumors we see that there are described many options of non-surgical or surgical treatment. The non-surgical treatment includes cryotherapy, photodynamic therapy, radiation, topical 5-fluorouracil, topical imiquimod, but non-surgical management has the disadvantage of absence of histopathologic confirmation for correct diagnosis of tumor type or for complete tumor eradication. Excisional biopsy with predetermined margin is an easy and popular technique but there is a lack of consensus regarding the need of tissue to be excised (2-5mm margins for periocular BCC). Hsuanet al reported that for a 2mm margin excision 10 patients out of 55 needed reexcision for free tumor margin, while Hamada et al found clear margins for 84% of lesions excised at 4mm margins;still because standardization of predetermined margins is not possible and in the eyelids the amount of tissue excised is essential, the need for histology clear margin confirmation remains an important issue. [8]. In the light of these numbers, there are practitioners that support the idea that MMS should be the first line treatment for BCC and is mandatory for recurrences and tumours with high chance of recurrence , tumours with lacrimal extension andtumours larger than 3cm.. MMS has disadvantages like cost, prolonged time of intervention and necessity of a specially trained personnel because histologic preparation and interpretation of microscopic examination requires skill and practice.MMS is a surgical technique that allows precise microscopic control of the margins with the most effective preservation of normal tissue . In 1986, Mohs showed a 99,4% cure rate at 5 years for primary periocular BCC in 1124 cases using MMS In FS-controlled excision, the cutting is done verticallyandprocessed by bread loafing technique, making some authors say that a smaller percent of margins are examined than with Mohs technique; despite that, studies showed that frozen section technique for BCC has comparable curability rates with Mohs surgery  GiordaniResti et al found an overall recurrence rate of 1.8%, with a mean follow-up of 72.4 months (range 30-167) from 110 malignancies concluding that FS excision of eyelid BCCs yields recurrence rates comparable to those of MMS at 5-year follow-up. [17] In our series of patient we found no recurrences in the follow-up period, but we need to continue monitor the patients to larger periods of time.Early diagnosis and correct treatment with complete tumor removal give high curability rate. Bernardini stated that Mohs surgery or complete surgical excision with frozen-section control of the margins offers the lowest tumor-recurrence rate. [18]Wu et al found in 1713 consecutive periocular BCC excision specimens that 52.7% involved the lower eyelid and the main histological subtypes identified were nodular (65.7%); our analysis showed nodular histopathological subtype in 66,67% cases and the infiltrative subtype appeared in patients aged less than 60 years old. [Fig. 5) shows photos of a patient before and after FS excision and reconstruction of entire eyelid with buccal mucosa, auricular cartilage and superior eyelid flap.We performed inferior eyelid reconstruction by direct suture in the majority of cases but complex reconstruction for entire eyelid was performed in 6 cases with separate anterior and posterior lamellar reconstruction; we used Hughes tarsoconjunctival flap or buccal mucosa and auricular cartilage for the posterior lamella and local flaps usually from the superior eyelid for the anterior lamella with good functional and aesthetic outcome.  followed for a period of time of less than five years, nevertheless we consider important that average lesion size (7.16mm) was bigger than other studies and still we had no recurrence during our follow-up period.In conclusion, BCC diagnosis should be done early and proper treatment recommended. Surgical treatment with histopathological margin control remains standard gold for the treatment of periocular BCC followed by eyelid reconstructionwith restoration of eyelid structure and function to as near normal possible.DisclosureNone


Myelolipoma is a rare neoplasm composed of yellowish adipose tissue and reddish-brown tissue corresponding to hematopoietic or hemorrhages. It typically occurs in adrenal glands as a solitary, well-circumscribed mass, and the thoracic location is extremely unusual.We present a rare case who is a 54 years old male with bilateral Myelolipoma of the posterior mediastinum. He underwent the surgery via video-assisted thoracic surgery both sides interval 3 months. Histological examination showed both tumors consisted of mature fat tissue and hematopoietic tissue, including myeloid, erythroid, and megakaryocytic elements surrounded. We discussed the etiology, histopathology, differential diagnosis and recommended management of extra-adrenal myelolipoma and analyzed the features of the thoracic myelolipoma including mediastinal and pulmonary location.Literature review showed 16 similar cases, with a 2/1 male/female ratio and a mean age of 58 years. Eight of sixteen cases were observed in the mediastinum and six of sixteen cases were displayed in the pulmonary and one showed on the chest wall. CT and MRI scans are able to indicate the presence of extra-adrenal myelolipoma. Pathological analysis is an effective method to clarify the diagnosis. Observation and surgery are two regular treatment methods. Small, asymptomatic tumors should be monitored, while large tumors that cause unendurable symptoms may be removed by surgery. Myelolipoma is encapsulated and composed of yellowish adipose tissue and reddish-brown tissue corresponding to hematopoietic or hemorrhages . It was A 54-year-old man was referred to our hospital for assessment of two posterior mediastinal masses that was detected on chest radiography during a routine health check. He denied the following symptoms including the presence of chest pain, hoarseness, hemoptysis, cough and dyspnea. He was a non-smoker and had no exposure to any environmental fumes or dust. Physical examination revealed normal breath sounds in both of the lung fields. Laboratory findings were within normal limits. In these examinations, hematology tests and biochemistry tests were within regular levels. His Pulmonary function tests and cardiovascular examination revealed normal performance. Plain chest computed tomography (CT) scans two paravertebral masses with well-defined margins, well demarcated from the descending aorta in the posterior mediastinum measuring 7.0 × 5.5 cm of the left one and 3.8 × 2.8 cm of the right one in size. There was no destruction of adjacent bone structures, no pleural effusion or infiltration of surrounding tissues and no evidence of intraforaminal or intraspinal tumor manifestation. Contrast-enhanced CT scans showed obvious enhancement in soft tissue masses Figure A, 1B. MaMyelolipoma is a relatively uncommon benign tumor, composed of mature adipose tissue and hematopoietic elements. Myelolipoma occurs at any age, which varied from 12 to 93 years , howeveret al.[The exact pathogenesis of myelolipoma is not clear at present. Historically, there are 3 theories of the pathogenesis of myelolipomas. The first is that myelolipomas are derived from bone marrow emboli that lodge in the adrenal gland or other sites. The second theory suggests that myelolipomas are derived from embryonic primitive mesenchymal cells. The third theory suggests that myelolipomas arise from metaplastic transformation of adrenal (or other sites) stromal cells in response to stimuli. As Dieckmann KP et al. reportedet al.. Once inet al.. Therefoet al..EAMs are typically asymptomatic. They tend to be incidentally discovered during radiological investigation of symptoms unrelated to myelolipoma. In our analysis, most of the patients are asymptomatic ,18,21,22Because these tumors are rare, criteria for diagnosing EAM radiologically do not exist. Characteristics of myelolipoma are deduced to apply to these structures which present in a variety of locations . Chest ret al.[There is no agreement on how to manage EAM. Daneshmand et al. suggesteet al., the 70-Grossly, EAM is a solitary circumscribed mass ranging in size from a few centimeters to 27 cm . From ouWhen the diagnosis of myelolipoma is considered, it should be differentiated from other tumors. Pulmonary myelolipoma should be distinguished from extramedullary hematopoiesis and hamartoma. Extramedullary hematopoiesis usually occurs as a manifestation of myeloproliferative diseases or is a compensatory phenomenon in various chronic anemias. In contrast to myelolipoma, extramedullary hematopoiesis of the lung frequently presents with multiple occurrences and ill-defined lesions. Microscopically, extramedullary hematopoiesis is composed predominantly of hematopoietic cells and erythroid hyperplasia. Lung hamartomas generally consist of cartilage, fat, and connective tissue . When myThe usual natural history of EAM is benign although they may enlarge and bleed. Some reports have shown stable lesions with follow up from 3 to 62 months . Small aAlthough primary extra-adrenal myelolipoma is rare, understanding its diagnosis and treatment are important. If a patient is suspected to have primary extra-adrenal myelolipoma by CT or MRI scans, this may be clarified it by pathological analysis. Subsequently, the tumor may be modified or removed by surgery, according to its effects on the body.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.CS was involved in drafting the manuscript. ZH was involved in acquisition of data and preparing the figures. GC designed and revised the manuscript. All authors have read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2482/14/42/prepub


The gastrointestinal stromal tumors (GIST) are mesenchymal tumors, most commonly affecting the stomach and small bowel. Only few cases of port-site recurrence after laparoscopic treatment have been reported. We herein report the case of a parietal recurrence on the extraction incision site, 7 years after laparoscopic surgery for small bowel GIST.A 47 years-old female patient was hospitalized in November 2007 for isolated pelvic pain. CT scan showed an intestinal tumor with a benign aspect measuring 50 mm. A laparoscopy-assisted resection was performed. Surgical exploration found a 7 cm small bowel tumor. It was extracted through a supra-pubic transversal incision without a wound protector and then resected. Histologic analysis revealed an intestinal GIST with high aggressive potential (five mitosis per field), with CD117 positive at the immunohistochemical examination. The patient had no adjuvant chemotherapy. Seven years later, the patient was readmitted for an abdominal mass at the site of the supra-pubic scar. Abdomino-pelvic CT scan showed a 10 × 7.5 cm solid mass of the abdominal wall. Percutaneous biopsies were done and the pathological analysis revealed a mesenchymal-cell tumor, positive to CD117 and DOG1 at the immunohistochemical examination. Final diagnosis was abdominal wall recurrence of GIST secondary to tumor-contamination during the first surgery.Abdominal wall recurrence of GIST after laparoscopic surgery is rarely reported. This complication should be avoided with preventive measures such as the use of extraction bags or wound protectors. Gastrointestinal stromal tumors are rare tumors secondary to a malignant proliferation of mesenchymal cells. They affect more frequently the stomach and the small bowel in comparison with other segments of the digestive tract  with a safety margin of 1–2 cm is actually the only potentially curative treatment. Imatinib based adjuvant chemotherapy is complementary to the surgery to improve the survival and the disease free survival. Laparoscopy has been used successfully in the treatment of digestive GIST with minor complications. Particularly, very few cases of port sites recurrences have been reported , positive to CD117 at the immunohistochemical examination. An Imatinib adjuvant therapy was indicated, but the patient did not receive it because of financial considerations.The patient had a postoperative follow-up  every 3 months during the first year, then every 6 months during the second and the third year. After that, the patient stopped her follow-up on her own.Seven years after the surgical resection, the patient was hospitalized for a pelvic mass underneath the old scar. The abdominal examination found a mass measuring 60/70 mm. The abdominal pelvic CT scan showed a 100/75 mm solid mass of the abdominal wall alongside to the pubic symphysis Fig. .Fig. 2CoPercutaneous biopsies of the tumor were made and histologic examination showed an aspect of low-grade malignancy GIST. An enbloc surgical resection of the tumor was performed, with a safety margin of 1 cm. The wall defect measured 6 cm in diameter and it was closed by direct suture. The pathological examination revealed a tumoral proliferation of spindle atypical cells with mitotic signs. The immunehistochemical examination showed an important positivity to CD117 and DOG1. The patient received adjuvant chemotherapy (Imatinib).We herein report the case of parietal recurrence on the extraction site of a GIST, after a laparoscopy-assisted resection. This is a rare event and only few cases have been reported in the literature, mainly port-site metastasis   (Martinez et al. This complication is preventable with simple measures such as: the extraction of the specimen in a wound protector, careful manipulation of the tumor to avoid breaking; peritoneal washing with cytocidal or anti-adhesive solution (Kaczmarek et al. The influence of the surgeon was also suggested responsible of 0–21 % of port-site recurrences after a laparoscopy performed for cancer. An experimental study on a porcine model showed a tumor recurrence in 63.8 % in the control group versus 13.8 % in the protective measures group (Schneider et al. Although our patient had a primary resectable localized GIST, a complete resection with negative microscopic margins do not prevent recurrences. The estimation of recurrence and death risks related to the GIST is based on the tumor size, mitotic rate and the site of the tumor. This estimation allows the selection of patients for adjuvant Imatinib and identifying their prognosis (Fletcher et al. Parietal recurrence of GIST after laparoscopy is rarely reported. They are considered as metastasis. This complication could be avoided with preventive measures such as the use of extraction bags or wound protectors.The patient gave signed statement, which authorises the use of her personal and/or medical information in the publication of this study.