Urachal remnant disease is uncommon in adults and can present with symptoms ranging from drainage near the umbilicus to a severe abdominal infection. Most cases are referred for treatment once diagnosed either clinically or radiographically with ultrasound or computerized tomography. We present a unique case of an infected urachal cyst visualized on a series of CT scans in an adult patient with abdominal pain over a period of years. A 33-year-old-male patient presented initially with lower abdominal pain in March 2006. He was diagnosed with appendicitis based upon his exam and history. A CT scan of the abdomen was performed at that time and demonstrated extensive inflammatory changes in the right lower quadrant with possible interloop abscess . Two nonThe patient presented again in August 2011 with right flank pain and urinary symptoms. A noncontrast CT scan of the abdomen was performed and demonstrated a right ureterovesicular junction calculus with mild right ureteral dilation . Again, He subsequently presented again 3 weeks later with severe abdominal pain and induration of his lower abdominal wall. CT imaging now demonstrated interval enlargement of a complex cystic mass inseparable from the anterior superior wall of the urinary balder and abutting the posterior margin of the abdominal wall . The radAt the time of surgery, cystoscopy did not demonstrate any abnormalities of the bladder. Exploratory laparotomy was performed and an indurated mass and abscess were encountered near the area of the anterior abdominal wall and bladder. The abscess was drained and a few small calcifications were encountered and removed for pathologic examination. A complete exploration was performed and the phlegmon appeared to be limited to the dome of the bladder. This area was excised and the dome of the bladder was closed primarily in layers with absorbable suture. Drains were placed and the abdominal wall was debrided and closed. He had an uncomplicated postoperative course. Final pathology demonstrated urachal remnants with no evidence of malignancy. The urachus is an embryologic tract that extends from the bladder to the umbilicus during development . RemnantOur case highlights the potential complications related to an urachal cyst disease in one patient who was treated for multiple causes of lower abdominal pain. The initial presentation of appendicitis was the first glimpse of the cystic remnant disease. The lower abdominal inflammatory changes were nonspecific and therefore it was impossible to differentiate perforated appendicitis with a fecalith from a less common cause of infection. Since the operative and pathologic findings were consistent with appendicitis at that time, consideration of a simultaneous condition was not considered.Later when the patient presented with a ureterovesicular stone and mild hydronephrosis, the CT scan demonstrated the proximity of the lower abdominal calcifications to the bladder. However, at that time there was no obvious inflammatory changes or infection associated with the bladder or abdominal wall and his symptoms were more consistent with nephroureter obstruction. Despite the radiographic presence of the urachal cyst each time the patient was evaluated for his symptoms, he was diagnosed with a potentially more common cause of pain each time. It was not until the patient developed a severe infection of his lower abdominal wall that the urachal cyst was finally treated surgically and excised. Urachal remnant disease should be considered in any patient with calcifications or cysts adjacent to the anterior abdominal wall or bladder dome. Urachal disease can be an incidental finding radiographically or it may present once infection or some other complication develops. Our case presentation highlights the potential for early diagnosis or potential misdiagnosis due to the frequent use of computerized tomography for patients with lower abdominal pain.


Background. Ameloblastoma is the most common odontogenic tumor. It represents about 1% of all tumors of the jaw. Extragnathic location of the ameloblastoma is typical and extremely rare. Case Report. We report a case of ameloblastoma of the nasal septum origin, causing nasal obstruction. According to our information, this is the first reported case of ameloblastoma coming from the nasal septum as a primary tumor without maxillary sinus involvement. Conclusions. Ameloblastoma can not only locate in the maxilla and mandible, but also in other regions of the craniofacial. Ameloblastoma should be considered in the differential diagnosis of tumors causing nasal obstruction. Nonspecific clinical features of sinonasal ameloblastoma make it extremely important to perform accurate diagnostic imaging and histopathological examination. Ameloblastoma is the most common odontogenic tumor. It represents about 1% of all tumors of the jaws , 3.Macroscopic appearance of ameloblastoma is usually a cavernous tumor, cushioned with a thin lining, sometimes without tumor capsule, filled with gray-white or gray-pink gelatinous mass. The microscopic examination reveals focuses of epithelial tissue and mimicking enamel organ, surrounded by mature connective tissue rich in collagen.The tumor shows a considerable variety in radiological imaging—presenting a view of one or more multi-cave cysts—sometimes containing a primordial tooth or images of soap bubbles, honeycomb, or solid tumor appearance .Usually ameloblastoma is diagnosed late, in advanced stages, due to its long and scantily-symptomatic development. Wide surgical excision of the tumor in the normal tissue margin is considered to be the treatment method of choice.Ameloblastomas developed in the maxilla can secondarily grow in the nasal cavity and paranasal sinuses, but the primary focus of ameloblastoma in this area, with no relation to the jaw area, is extremely rare. There are only a few reports of such cases in the literature , 5–7.We report a case of ameloblastoma of the nasal septum origin, causing nasal obstruction. According to our information, this is the first reported case of ameloblastoma coming from the nasal septum as a primary tumor without maxillary sinus involvement. The previous reports of ameloblastomas causing nasal blockade were associated with cases of tumors expanding into the nasal cavity from a starting point in the maxilla.A thirty-five-year-old man, had undergone septoplasty in the Department of Plastic Surgery because of impaired nasal patency lasting for three months. Due to the lack of improvement reported 3 years after surgery, he was admitted to the district ENT ward. The rhinoscopy showed complete obstruction of the right nasal passage due to creation of a cyst, extending from the nasal septum. The cyst was removed surgically. The histopathological examination revealed the cyst wall fragment with chronic inflammation.Nasal obstruction appeared again after two months. The another biopsy of lesions was collected for histopathological examination. The specimen has been described as ameloblastoma. The patient was transferred to the Department of Otolaryngology Head and Neck Surgery, Medical University of Wroclaw. On admission, the patient's main complaint was nasal obstruction on the right side. In the posterior rhinoscopy, a tumor of the posterior upper part of the nasal septum was revealed . The comThe tumor was removed surgically. For the radical surgery and in the interest of cosmetics, the access through eversion of the face coverings  was chosen. The removal of the front and medial wall of the right maxillary sinus with lower concha allowed an easy access to the tumor occupying the upper part of the posterior nasal septum and passing to the sphenoid sinus. The advantage of this method is obtaining no external cuts and, consequently, a good aesthetic result. The tumor was completely removed. There was no tumor in the right maxillary sinus and nasopharynx. The tissue samples from the tumor margin were collected.The histopathological examination confirmed the diagnosis of ameloblastoma. The margins of the removed tumor were free of tumor cells. The postoperative course was uncomplicated. The patient after surgery feels good. Nasal patency is still correct and he remains under constant care of ENT outpatient clinic.Extragnathic location of the ameloblastoma is unusual and extremely rare , 7. The The diagnosis of the sinonasal tumors at an early stage is difficult. It is important to collect the interview carefully, pay attention to the symptoms of a unilateral nature, and perform physical examination necessarily accompanied by nasal and nasopharynx endoscopy. Ameloblastoma diagnosis is based on the clinical and radiological image with the decisive histopathological confirmation. However, the differential diagnosis can cause problems, especially during the examination of a biopsy of the small fragments of tissues or obtained superficially, due to the lack of assessment of the whole structure of the tumor , 6. RadiThe ameloblastomas differential diagnosis of the sinonasal region takes into account the most common acute and chronic sinusitis, inverted papilloma, squamous cell carcinoma, adenocarcinoma, angiofibroma, basal cell adenoma, and proliferation of craniopharyngioma . In the The treatment of choice of ameloblastomas and other sinonasal tumors is surgery. In some cases, the endoscopic procedures are applied , 6. The This case shows that ameloblastoma can be located not only in the maxilla and mandible but also in other regions of craniofacial. Ameloblastoma should be considered in the differential diagnosis of tumors causing nasal obstruction. Nonspecific clinical features of sinonasal ameloblastoma make it extremely important to perform accurate diagnostic imaging and histopathological examination.en bloc with a wide margin. The implementation of a fast, radical surgical treatment in the highly-specialised center is a key for the successful prognosis and for avoiding complications. A long-term followup is required after the completion of the treatment [Despite the seemingly benign tumor histology, the lesions should be removed reatment .


A 32-year-old man was referred to the Clinic of Plastic Surgery for evaluation of an enlarging, nonhealing lesion on the scalp that had been present for 12 months. The patient was somnolent and dehydrated. Serum calcium level was 4.5 mmol/L .Incisional biopsies from 4 quadrants demonstrated an epithelioid sarcoma comprising epithelioid cells and discrete areas of central necrosis. Plain skull radiographs showed bony erosion. Full-body computed tomographic scans confirmed a soft-tissue mass fixed to the skull and multiple metastatic nodules in the cervical lymph nodes and both lungs.What are the characteristics of epithelioid sarcoma?What is the treatment plan for an epithelioid sarcoma of the scalp?What are the pathogenesis and treatment of humoral hypercalcemia of malignancy?Epithelioid sarcoma is a mesenchymal malignancy that has been mostly reported as affecting the distal extremities. It is exceptionally rare as a primary neoplasm in the head and neck. Early diagnosis poses a challenge. The first appearance of the tumor can be very ambiguous, most frequently as slow-growing subcutaneous nodules and ulcers that are easily misdiagnosed as benign lesions for long times, as was the reported case. Moreover, even the histological diagnosis may pose some difficulties. Specific enzymatic, histochemical, and ultrastructural tests are required for accurate diagnosis.A treatment plan for this neoplasm has not been well defined in the literature because of the rarity of sarcomas in general and of epithelioid sarcomas located in the head and neck in particular. The tumor may be localized in the dermis and subcutaneous tissue, but it has a propensity for extension through deeper structures, even the cortical bone. Thus, it is imperative that antitumor therapy be implemented promptly. Early intervention is important. For epithelioid sarcoma, aggressive surgical management and radical lymphadenectomy seem to be the treatments of choice. Reconstruction of the tumor excision defect can be challenging. Chemotherapy may play a limited role in advanced stages. Radiation therapy, although usually performed, needs further research on efficacy.The osteolytic process of bone invasion and several factors generated by disseminated tumor (such as parathyroid hormone–related peptide) can result in an increase in serum calcium concentration, a condition known as humoral hypercalcemia of malignancy. Hypercalcemia can potentially threaten the life of the patient, and this previously unreported association with epithelioid sarcoma stresses the importance of a high index of suspicion for early diagnosis. Treatment of tumor-related hypercalcemia consists of normal saline administered intravenously at a rate of 200 to 500 mL per hour, followed by intravenous bisphosphonate. If normocalcemia is not achieved, the prognosis is very poor, and approximately 50% of such patients die within 30 days.


A 50-year-old male patient presented with microcytic anemia , leukocytosis of 19.7 x 109/L , and normal platelet count. During the etiological work-up of his anemia an abdominal computed tomography (CT) scan was done, which showed a mass of 6.5x5.5 cm located in the ascending colon and hepatic flexura causing a wall thickening of 20 mm. The thoracic CT was normal. A complete colonoscopy showed an ulcerovegetative lesion in the transverse colon narrowing the lumen and multiple biopsies were performed, which revealed a well-differentiated colon adenocarcinoma. During the surgical removal of the tumor, 4 peripancreatic and 48 mesenteric lymph nodes and the perilymphatic fat tissue were resected, which all had diffuse infiltration of atypical lymphocytes. In one mesenteric lymph node, both invasion of the colon adenocarcinoma and atypical lymphocytes were demonstrated  and 1B. Patients with CLL have more than twice the risk of developing a second cancer, and this increased incidence is attributed to disease- or therapy-related immunosuppression . The mosThe synchronous diagnosis of B-CLL/SLL and colon adenocarcinoma in our case is most probably coincidental. However in the synchronous presentation of these two malignancies, an epidemiological association has been noted , and thiConflict of Interest StatementAll authors have no conflict of interest to declare.


Acute appendicitis is a common disease in older children but rare in neonates.We report the case of a 2-day-old Asian baby who suffered from neonatal appendicitis mimicking intestinal duplication. Laparoscopic appendectomy was successfully performed after the trans-umbilical division of adhesions, and the postoperative course was uneventful.There are few reports describing abdominal masses caused by appendicitis mimicking intestinal duplication. The laparoscopic approach for neonatal appendicitis is considered to be a safe and useful therapeutic modality with good cosmetic results. Neonatal appendicitis is rare and has a high mortality rate -3. DiagnA 2-day-old Asian baby, born at 36 weeks’ gestation, weighing 2944g, was admitted to a local hospital for abdominal distension and poor general status. His white blood cell count was 19,800 (Neutrophil 75%). The administration of antibiotics and non-per oral feeding were performed based on a preliminary diagnosis of sepsis and paralytic ileus. His condition improved slightly; however, he was admitted to our hospital on the fourth day after his birth under a suspicion of NEC. His abdomen was slightly distended but a normal color. The results of ultrasonography examinations and an X-ray showed no signs of NEC . A lower intestinal tract contrast study showed a normal colon structure. The results of a spinal fluid test revealed an increased cell count, and conservative management was continued under suspicion of sepsis and meningitis.An abdominal ultrasonography examination 6 days after birth showed an abdominal cyst with a double layer surrounding sign at right lower quadrant Figure A. ComputThe open Hasson technique was used. A 5mm umbilical port was inserted via an inferior-umbilical incision. The intestine, colon and peritoneum all strongly adhered to the right abdominal region and no other ports could be inserted. A wound retractor XS™  was used to perform the trans-umbilical division of any adhesions. Laparoscopic examination revealed adhesions and inflammation around the ileum, ascending colon and liver bed and an inflamed appendix was strongly attached to the retro-peritoneum Figures A–D. No iAlthough acute appendicitis is a very common disease in adults and children, the incidence of neonatal appendicitis is rare -3. A malThe causes of neonatal appendicitis are thought to differ from those in adults and older children. Amyand’s hernia and other coexisting diseases (e.g. Hirschsprung’s disease and cystic fibrosis) may cause appendicitis ,7. PremaNeonatal appendicitis has no specific signs or symptoms, therefore a preoperative diagnosis is very difficult and the majority of neonates have so far been diagnosed intra-operatively . A diffeet al. [Laparoscopic appendectomy was performed on the neonate. This is the third case (second report) of laparoscopic appendectomy for neonatal appendicitis . Tajiri et al.  reportedThere are few reports describing abdominal masses caused by appendicitis mimicking intestinal duplication. The laparoscopic approach for neonatal appendicitis is considered to be a safe and useful therapeutic modality with good cosmetic results.Written informed consent was obtained from the parents of 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.CT, computed tomography; FIP, focal intestinal perforation; NEC, necrotizing enterocolitis.The authors declare that they have no competing interests.IS, TY and RM were operators of this case study. ST and YZ were major contributors to the editing and revising of the manuscript. TK was a contributor in evaluating the radiological aspects of the manuscript. All authors read and reviewed the final manuscript.


We report the case of a yolk sac tumor of the ovary in a 26 years old woman. The laparoscopic approach and the BEP chemotherapy were fundamental to have a minimally invasive treatment and to preserve fertility. The ovarian germ cell tumors (OGCTs)  represeWe report here the case of a 26 years old women with a YST treated with laparoscopic technique with success.A 26 years old female with body height of 1,68 m was admitted to our department with abdominal pain. She referred us a history of intermittent abdominal pain which had slowly been worsening over three month. In the last month there was loss of appetite. She reported amenorrhea for the past two cycles. She had no important past medical or surgical history. On palpation a vague mass was felt in the lower abdomen. The vaginal examination revealed a mass in the left fornix that was mobile and sore.After the visit we executed an ultrasound examination with 3.5 MHz curvilinear probe that revealed a large mix echogenic mass measuring approximately 4cm in mayor diameter and 2,5 cm in smaller arising from pelvis that appears multilocular, cystic and solid in nature. At color doppler examination there was increased vascularity noted in the solid component of mass. No evidence of calcification. No fluid in the Douglas. The origin of the mass could not be ascertained, but was felt to be likely from the left ovary because a separate, normal left ovary could not be visualized. The right ovary appeared normally with the presence of a follicle of 1,5 cm.Biochemical and laboratory investigations were made. Labs included a hemoglobin of 12,1 g/dl, a hematocrit of 37,9% and platelets of 275.000; creatinine was normal  and also BUN (32 mg/dl); liver function tests that included bilirubin , aspartate aminotrasferase (19 u/L), alanine aminotrasferase (17 u/L) were normal; Serum human gonadotropin hormone levels  and CA-125 levels (293 UI/L) were within normal limits. A markedly elevated serum alpha-fetoprotein (AFP) level to 18.178 ng/ml oriented toward the diagnosis of an ovarian yolk sac tumor.Subsequently, CT of the abdomen was performed. A 4,5 cm size complex mass lesion in the left ovary with solid component and cystic areas adjacent to it was present. Pelvic and paraahortic lymph nodes were in the range limits for the dimension and morphology. No calcified peritoneal implants were apparent, the others abdomen bowels appeared of normal structure.After our evaluations we decided to plan a laparoscopic surgery. We provide the steps of surgery; after the creation of pneumoperitoneum with Verres needle, a central trocar was used for the optics, others three trocars were inserted as lateral operative accesses. The uterus and right adnexa appeared normal with no lesion; the left adnexa showed a large mass with cystic and necrotic aspects. There was no peritoneal carcinomatosis and no lesions on the liver or subdiaphragmatic areas. A peritoneal washing was made; After a total omentectomy, obturator and paraahortic lymph nodes dissection were also added to the left salpingo-oophorectomy; a biopsy of right ovary, of left parieto-colic sulcus and appendicectomy were made. Specimens were sent for histopathological examination . The leYolk sac tumor is also called endodermal sinus tumor (EST) because there is a link to its discovery. Histologically the malignant tissue resemble structure found in early embryonic development, and it is Shiller’s stressing of the unique glomeruloid structure that led Telium to compare it with the yolk sac endodermal sinuses of Duval in the rat placenta .High values of AFP orientate strongly to diagnosis of YST. Furthermore it is a sensible marker for tumor’s evolution; infact a rapid decrease of AFP levels in the serum after surgery is a sign of absence of residual tumor. Also the efficiency of chemotherapy its related with normalization of AFP . It isAs regard clinical, abdominal pain is the principal symptom leading the discovery of the disease which may require the emergency surgery especially in cases of ovarian torsion . OtherPreoperative radiological diagnosis is difficult. Yolk sac tumors can be cystic, with signs of hypervascularization and areas of haemorrhage. However YST haven’t a specific image both ultrasound that MRI and CT. If YST appear with a solid portion, multiple small arterioles with lower RI are detectable by color Doppler ultrasound, haemorrhagic spots can be demonstrated by T1 weighted MRI, hypervascularity can be shown on contrast-enhanced T1 weighted scans . The mThe standard management of malignant ovarian germ cell tumors is complete surgical excision.Because of most of them are unilateral, it’s possible executing a fertility sparing surgical treatment. The type of surgical procedure was not an important prognostic factor for patients with malignant germ cell tumors of the ovary at all clinical stages, and so the conservative surgery is possible if it’s followed by chemotherapy .In the past after the laparoscopic diagnosis of an ovarian cancer, laparo-conversion was recommended to have an optimal staging and to avoid an uncertain tumor cell spread. Today, advancement in technologies offers a minimally invasive surgery that recognizes in the laparoscopic surgery a new approach for ovarian cancer. It gave us not only a same or even better surgical prognosis as compared the conventional laparotomy but benThe aim of surgery is removing the primary ovarian tumor without excessive morbidity. Thus cycles of chemotherapy should be administer while monitoring the rate of decline of serum AFP. Specifically, before the introduction of effective combination chemotherapy, the prognosis for patients with malignant nondysgerminomatous cancers was poor; as regards patients with YST, they had a 3-year survival rate of 13% . CurreCompared with other regimens, BEP appears to be the best active first-line option for primary, metastatic, or recurrent disease. As regards the chemotherapy-related toxicity, it was acceptable and severe collateral effects was infrequent, although myelosuppression can be observed in the majority of patients. The administration of G-CSF would help manage this toxicity. Infrequent severe pulmonary toxicity, ototoxicity, or nephrotoxicity are observed .Administration of bleomycin as a slow infusion (over 24 h)may reduce the incidence of toxicity, but this has yet to be confirmed . AdminProgressive or recurrent ovarian YST after treatment with BEP chemotherapy is associated with a poor prognosis. There are not approved schemes of chemotherapy in this cases, so it is possible adapt chemotherapy used for recidivist testicular cancer. Possible options of chemotherapy include combination of vinblastine, ifosfamide, cisplatinum, or paclitaxel, ifosfamide, cisplatinum, . It shAs regards the follow-up of chemotherapy, the determination of initially elevated markers (AFP) should be repeated before each cycle of therapy, soon after the end of the treatment and during the 2 years after the end of chemotherapy . An anThe BEP regimen, no ascites at presentation, stage I disease, less of 42 days to AFP normalization, fertilitysparing surgery and a serum AFP half-life less of 10 days are factors associated with a good prognosis.Lumbaraortic lymphadenectomy has been proposed to identify patients with a higher risk of relapse after surgery, in order to guide the choice of adjuvant chemotherapy .In the study of de La Motte, Rouge and collaborators lymphnoAnother important consideration is the impact of chemotherapy on gonadal and reproductive function. In the past the majority of patients with MOGCT receiving multidrug combination chemotherapy, showed a decreased number of primordial follicles and an increase in stromal fibrosis and in atrophied cortices . TodayTherefore the risk of of infertility following treatment of MOGCT is always a concern, even if the majority of patients, especially in the early stage, will maintain their ovarian function and fertility. Following this strategy, most patients will be cured and be able to give birth.


Leukemias rarely debut by pleural involvement as the first manifestation of the hematologic malignancy. This complication is most commonly seen in solid tumors such as carcinomas of the breast, lung, gastrointestinal tract and lymphomas. We present a case of a 66 year old male who presented with a pleural leukemic infiltration of his undiagnosed Acute Myeloid Leukemia that was not a complication of the disease extension, but the acute presentation of the illness. Progressive shortness of breath for two weeks, cough, clear sputum and weight loss were the initial complaints. Serum dyscrasia suggested a hematologic abnormality. A chest x-ray performed demonstrated a buildup of fluid with layering in the left pleural cavity. Diagnostic thoracentesis suggested an exudative etiology with cytology remarkable for 62% leukemic myeloblast. The diagnosis was confirmed by bone marrow biopsy with expression of the antigens CD 34+ and CD13+, with unfavorable cytogenetic prognosis and a trisomy 21 chromosomal defect. Chemotherapy was initiated, though no remission achieved with induction chemotherapy. Complications and disease progression precludes in the patient’s death. Although rare, due to the unusual presentation of the disease, this case clearly demonstrates the importance of biochemical analysis and cytopathology specimens obtained in pleural fluid. As a result, there is an accumulation of leukemic blasts or immature forms in the bone marrow, peripheral blood, and occasionally in other tissues, with a variable reduction in the production of normal red blood cells, platelets, and mature granulocytes. The increased production of malignant cells, along with a reduction in these mature elements, result in a variety of systemic consequences including anemia, bleeding, and an increased risk of infection1. Less than 1 percent of patients present with prominent extramedullary disease2. These extramedullary manifestations can manifest simultaneously with, or precede, bone marrow involvement. Sites of isolated expression include bone, periosteum, soft tissues, and lymph nodes, and less commonly the orbit, intestine, mediastinum, epidural region, uterus, and ovary2. To our knowledge this is one of the few reported cases of pleural effusion as the initial manifestation of AML.Acute Myelogenous Leukemia (AML) is a group of hematogenous neoplasms characterized by clonal proliferation of myeloid precursors with a reduced capacity to differentiate into more mature cellular elementsA 66 year old man with a long-standing history of mild to moderate asthma and arterial hypertension was evaluated for a worsening productive cough of clear sputum, dyspnea, wheezing, and unintentional weight loss of approximately thirty pounds. The patient denied fever, chills, hemoptysis, night sweats, chest pain, or exposure to sick contacts. His medications were frequent use of short acting β-agonist with minimal resolution of symptoms.2 38.3 mmHg, and PO2 67 mmHg; oxygen saturation was 89% without supplemental oxygen.On physical examination, the patient was alert but in mild respiratory distress, afebrile without hemodynamic compromise. The cardiac examination was normal; pulmonary examination revealed diffusely decreased breathing sounds, inspiratory crackles, and dullness to percussion, decreased fremitus and egophony in up to two thirds of the left lung field. There was no use of accessory muscles and oxygen saturation was 90% with the patient breathing ambient air. Neither lymphadenopathy nor organomegaly was palpated. CBC was abnormal for hemoglobin 8.1 g/dL, platelet 60,000/µL, leukocyte count 87000/µL with 64% blast . ArteriaA hematologic malignancy was suggestive due to the serum dyscrasia. Chest radiograph showed a large free flowing left pleural effusion . A diagnA karyotypic abnormality of Trisomy 21 was revealed through cytogenetic studies , which i1. AML generally presents with symptoms related to complications of pancytopenia. Most patients have more subtle evidence of bone marrow involvement for weeks, or perhaps months, before the diagnosis can be made. Despite the pancytopenia, and/or coagulopathy, it is unusual for leukemias, either acute or chronic, to manifest with malignant pleural effusions as the initial presentation5. Usually this abnormal amount of fluid collection is a complication more commonly seen in solid tumors and lymphomas5.Physicians dealing with the diagnostic workup of pleural effusions rarely discover an underlying hematologic malignancy7. AML in this particular case, and prompt treatment could undoubtedly contribute in avoiding complications associated with the condition; an essential factor for improving quality of life. For this reason chest physicians should be aware of all possible pulmonary manifestations of hematologic malignancies.Our patient presented with AML and pulmonary involvement with signs and symptoms secondary to the pleural effusion itself rather than with classical appearance of the acute myeloid leukemia. An unusual case where neither the complications of the hematologic dyscrasia such as bleeding and recurrent infections, nor the physical findings of a swollen spleen, liver, or lymph nodes were the primary target organs that would lead to a presumptive diagnosis. This demonstrates the importance of the biochemical analysis and the cytopathology specimens obtained in pleural fluid since an early detection of any determined disease could guide effective therapyWritten informed consent for publication of clinical details and clinical images was obtained from the relative of the patient. The authors report a case of pleural effusion as an initial presentation of Acute Myeloid Leukemia (AML) in an adult patient with no physical exam findings suggestive of leukemia and or malignancy.The authors corroborated the exudative nature of this symptomatic s pleural effusion by flow cytometric analysis for CD34+ and CD13+ antigens and supported their diagnosis by bone marrow studies and cytogenetic analysis which showed a trisomy 21 pattern. While a chemotherapeutic regimen of Cytarabine and Idarubicine inhibited further progression and re accumulation of the pleural effusion, the patient’s condition deteriorated leading to his death. Given the dismal nature of the patient’s AML prognosis, hematologists need to be aware of this atypical and rare AML presentation. More importantly, pulmonary physicians should acquaint themselves with this clinical scenario because such patients present first to them and not to hematologists.Pleural effusion has been reported in association with solid tumors and sometimes as their presenting sign. A multivariate analysis by Faiz et al., Leukemia Lymphoma (2012) between 1997 and 2007 showed that 111 patients with acute leukemias had pleural effusions and the median overall survival in these patients was shorter than those with absence of pleural effusions. Other studies revealed rare occurrences of pleural effusion in AML patients.The authors need to highlight salient features of previously reported patients and compare them with their patient hoping to find predictive factors for development of such rare clinical scenarios and define better treatments.I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. This article points out an unusual clinical presentation of acute myeloid leukemia (AML) that illustrates the heterogeneity of presentations of extramedullary disease.While the diagnosis was clear at the onset, the patient had 64% circulating blasts and the pleural effusion was unusual and required evaluation. As monocyte subtypes of AML have a propensity for tissue invasion and the core binding factor (CBF) leukemia can be associated with granule cystic sarcomas, a bit more information on the immunophenotype of the AML would have been helpful in assessing this case. Also, given the many molecular markers that define the prognosis of AML patients, more details on any molecular studies that were done, or could have been done, would be helpful.I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. This case report describes a case of acute myeloid leukemia (AML) that presented with a large pleural effusion. This unusual presentation is important to recognize when evaluating patients with pleural effusions. This particular case report illustrates the importance of a cytopathology evaluation.The report itself would benefit from an updated reference on chromosomal abnormalities in AML, and it would also be helpful to know the doses of the chemotherapeutic agents and if the patient was treated on a clinical trial for newly diagnosed AML.I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard.


Alimentary tract duplications are rare congenital anomalies. The presentation depends on their anatomical location, size and other characteristics. The most common variety is small bowel cystic duplication. We report a case of an eight years old girl who presented with recurrent abdominal pain and melena. Radioisotope technetium scan showed increased uptake of tracer in right lower abdomen and a diagnosis of Meckel’s diverticulum made. At surgery a cystic, communicating, ileal duplication found which was resected along with adjacent gut. It is thus reiterated that while investigating children with recurrent abdominal pain and melena, gut duplications must be included in the differential diagnosis. Alimentary tract duplications (ATD) are rare congenital anomalies . These lDuplications of small bowel can cause life threatening complications like volvulus, intussusception, gut perforation and massive bleeding. Herein we report one such case that remained elusive till laparotomy was performed.An eight years old girl was admitted in medical ward through emergency department because of recurrent abdominal pain and melena for the last one month. She had similar complaints since the age of 3 years. The symptoms usually settled within few days. At the time of admission she was otherwise healthy but pale looking with tenderness around umbilicus. Her weight was 19 kg, hemoglobin 6.8 gram per cent and hematocrit 21 percent. Her coagulation profile was within normal limits. Abdominal radiograph and ultrasound examination were reported as insignificant.Radioisotope technetium scan done for suspected Meckel’s diverticulum showed increased tracer uptake in right lower abdomen close to urinary bladder suggestive of ectopic gastric mucosa . She was prepared for surgery with transfusion of packed cells and initially underwent laparoscopy. The lesion could not be localized so laparotomy was performed. The omentum was found adhered to a lesion in the mid ileum and gut was twisted around it. On separation about 4×5 centimeter cystic structure found in the mesentery of ileum two feet proximal to the cecum . The cystic structure with adjacent part of ileum was resected and continuity of gut restored with a primary anastomosis. The duplicated part was found communicating with both proximal and distal parts of gut resected. The post-operative recovery was uneventful. Histology of resected gut confirmed the presence of gastric mucosa in duplicated part. Alimentary tract duplication cysts are hollow, epithelium-lined, cystic, spherical or tubular structures that are attached to the wall of gastrointestinal tract . They maThe clinical presentation of these lesions can vary according to the age of the patient as well as anatomical location. Some may remain asymptomatic and identified on routine physical examination or during investigations for other problems . The smaThe diagnosis is usually not established before surgery as occurred in our patient where Meckel’s diverticulum was suspected based upon radioisotope findings thus duplications must be considered in differential diagnosis of recurrent abdominal pain and occult gastro intestinal bleeding. Cystic duplications can be resected easily along with adjacent intestine and same was possible in case reported here. Presence of adhered omentum at surgery points towards episodes of inflammation of the cyst. Nowadays laparoscopy is gaining importance in managing different pediatric surgical conditions. This minimally invasive procedure was used in this patient but had to be converted to open approach, as the lesion could not be identified. With increasing learn curve we hope to use this technique more liberally.Source of Support: NilConflict of Interest: None declared


A 10-year boy presented with a painless swelling in the left upper abdomen which gradually increased in size. Physical examination revealed a non tender, smooth, firm and an irregular mass in left hypochondrium extending to left lumbar region. On ultrasound, a hypoechoic lesion (14cm x10cm) in left suprarenal region was noted. Computed tomography (CT) revealed a large (18cm x 12cm) lobulated hypodense lesion with linear hyperdense enhancing areas in the left suprarenal region . The lesion displaced the stomach and pancreas anterio-superiorly, the left kidney posterio-inferiorly and the spleen laterally. The fat planes surrounding these structures were intact. There was stretching of the splenic and left renal veins. Medially, the mass was seen extending anterior to the abdominal aorta. There was anterio-lateral displacement of the portal vein and inferior vena cava while the bowel loops were displaced inferiorly. Serum electrolyte and hormonal studies confirmed the non functional nature. On exploration, the mass was found arising from left suprarenal region. There was no evidence of invasion of the adjacent organs. The resected specimen was pale yellow in color with firm to hard consistency . The histological examination of the mass revealed a mixture of immature and mature ganglion fibers with the neuronal cells, suggestive of ganglioneuroma. There was no evidence of malignancy. Patient was discharged on post operative day-15 and was kept on 3 monthly follow up with serum electrolyte, serum cortisol and ultrasound. Ganglioneuroma (GN) is a rare benign tumor. They may occur spontaneously or during the therapy for neuroblastoma with either chemotherapy or radiation therapy [1]. GNs are mostly sporadic with familial predisposition. Association with Turner’s syndrome and multiple endocrine neoplasia II has been reported [2]. It is most commonly found is posterior mediastinum and the retroperitoneum [2]. The reported incidence of GN is one per million population [3]. As a primary retroperitoneal tumor, it constitutes only a small percentage of 0.72 to 1.6% [3].Retroperitoneal GNs are usually nonfunctioning and asymptomatic until they reach large sizes, when they cause symptoms due to local expansion and compression of adjacent structures [4]. Ganglioneuroma may release catecholaminergic peptides, which can produce hypertensive crisis during the surgery [4]. Preoperative diagnosis of retroperitoneal ganglioneuroma is often difficult and is usually based on histopathological findings after surgical excision of the tumor . CT is the most commonly used imaging modality for assessment because it reveals the extent of the tumor, organ of origin, regional invasion, vascular encasement, adenopathy, and calcification [5]. Interestingly, GNs tend to partially or completely surround major blood vessels, with little or no compromise of the lumen [6]. Pathologically, GNs should be differentiated from ganglioneuroblastoma, which have a ganglioneuromatous component of more than 5%; the ganglioneuromatous component of GN is minimal [7]. Treatment of GN consists of complete surgical removal of the tumor, when possible [8]. Preoperative or postoperative chemotherapy or radiotherapy has no value, unless the GN is associated with ganglioneuroblastoma changes [9]. The possibility of slow progression and late recurrence, rare malignant transformation has been reported; therefore, long-term periodic radiologic surveillance is necessary .Source of Support: NilConflict of Interest: None declared


Transitional cell carcinoma (TCC) of the female urethra is a rare urological malignancy, and intracranial metastasis of this cancer has not yet been reported in the literature. This review is intended to present a case of multiple intracranial metastasis in a female patient with a remote history of primary urethral TCC.A 49-year-old woman, presented with a prolapsed mass in urethral orifice that was diagnosed as primary urethral TCC with distant lung and multiple bone metastases. The patient subsequently underwent chemotherapy under various regimens. A year later, the patient developed headache and vomiting which as was found to be due to multiple intracranial metastasis. The patient underwent surgical resection of the largest lesion located on the cerebellum, and consecutively gamma knife radiosurgery was performed for other small-sized lesions. Pathological examination of the resected mass revealed a metastatic carcinoma from a known urethral TCC. Serial work-up of systemic metastasis revealed concomitant aggravation of lung, spleen, and liver metastasis. The patient died of lung complication 2 months after the diagnosis of brain metastasis.To the best of our knowledge, this is the first reported case of cerebral metastasis from primary urethral TCC, with pathological confirmation. As shown in intracranial metastasis of other urinary tract carcinoma, this case occurred in the setting of uncontrolled systemic disease and led to dismal prognosis in spite of aggressive interventional modalities. Primary urethral carcinoma is a rare entity, accounting for only 0.02% of all female cancers . The oveIntracranial metastasis from TCC of urinary system is reported as rare events . MajoritA 50-year-old woman was admitted with headache and vomiting for 2 days. One year prior to the present admission, she had visited the department of urology for the treatment of a prolapsed mass in urethral orifice. Cystoscopy revealed a proximal urethral mass with an unremarkable bladder. Based on the pelvic computed tomography (CT) Figure , cystosc1-weighted MR due to hemorrhage and peritumoral edema as well , especially with a mass effect (> 1cm midline shift), surgical resection should be considered with/without adjuvant whole brain radiotherapy (WBRT). WBRT has been generally accepted as the standard treatment for multiple cerebral metastases. However, considering cognitive deficits after WBRT and the better results of radiosurgery, radiosurgery would play a more important role for the treatment of metastatic brain tumors. Although the role of surgical resection for multiple brain metastases has not been established, surgery has been performed in cases with large lesions or significant mass effects and in cases where two or more lesions are accessible through a single craniotomy approach . CerebelThe current case had showed unfavorable factors in determining surgical resection, such as multiplicity in brain metastasis and uncontrolled systemic disease. Considering the questionable survival benefit and high incidence of postoperative complications, surgical resection for a large cerebellar lesion could seem to be excessive as a treatment option. The current patient was young and regularly received systemic treatments, and, most importantly, demonstrated high performance status without definitive neurological signs. Of aforementioned prognostic factors, high performance status was the most significant parameter in choosing surgical resection for treatment option ,21. We aIn selected cases with single brain lesion and well controlled systemic cancer, aggressive treatment including complete surgical resection can prolong the survival and improve the life quality. Nonetheless, once the cerebral metastasis occurred in TCC of urinary tract system, the prognosis is poor, especially in the case with multiple cerebral lesions and/or uncontrolled systemic disease ,7.In conclusion, urethral TCC in female, a very rare cancer, can metastasize to the brain in uncontrolled systemic state and result in dismal prognosis despite of aggressive treatment, as observed in other urinary tract TCC.Written informed consent was obtained from the husband of 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.CT: computed tomography; TCC: transitional cell carcinoma; MR: magnetic resonanceThe authors declare that they have no competing interests.KSM & ECH analyzed the data and drafted manuscript. SJ participated in study design and coordination. KHL participated in reviewing literatures and revising the manuscript critically for pathologically content. Kim IY performed the surgical procedure and helped to draft the manuscript. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2407/11/23/prepub


The incidence of adrenal involvement in MEN1 syndrome has been reported between 9 and 45%, while the incidence of adrenocortical carcinoma (ACC) in MEN1 patients has been reported between 2.6 and 6%. In the literature data only unilateral development of ACCs in MEN1 patients has been reported. We report a 31 years-old female MEN1-patient, in whom hyperplasia of the parathyroid glands, prolactinoma, non functioning pancreatic endocrine carcinoma and functioning bilateral adrenal carcinomas were diagnosed. Interestingly, a not previously described in the literature data, novel germline mutation (p.E45V) in exon 2 of MEN1 gene, was detected. The association of exon 2 mutation of the MEN1 gene with bilateral adrenal carcinomas in MEN1 syndrome, should be further investigated. Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder with penetrance reaching 100% with age [It is characterized by parathyroid glands hyperplasia, anterior pituitary gland tumours and pancreatic islets tumours . HoweverA simple definition of MEN 1 can not cover all index cases and all families. As a practical definition, sporadic MEN1 is characterised by the occurrence of primary tumours involving two of the three main MEN1 related endocrine tissues within a single patient, while familial MEN1 is defined as at least one MEN1 case plus at least one first degree relative with one of those three tumors .The incidence of adrenal lesions in MEN1 patients varies between 9 and 45% -20 and tHerewith, we report a 31 years-old female patient with sporadic form of MEN1 in whom functioning bilateral adrenocortical carcinomas were diagnosed. Interestingly enough, a novel germline mutation in exon 2 of MEN1 gene, was detected.st Department of Surgery, for further evaluation and treatment.A 31 years-old female patient with the preoperative diagnosis of MEN1, was referred by the Endocrinologists to the 1On clinical examination, centripetal obesity, moon face, fat deposition over the thoracocervical spine , excessive terminal hair in various parts of her body and four angiofibromas in her face, were observed. Her arterial blood pressure was moderately abnormal (155/80 mmHg), while she reported menstrual irregularity (infrequent uterine bleeding) and easy bruising. She was suffering from diabetes mellitus since 2002 and she was currently on glargine insulin, whereas she reported having been prescribed Dopamine agonists for her pituitary prolactinoma lesion. Her family history was insignificant®) disclosing radionuclide material uptake in the left upper quadrant of the abdominal cavity at some point between the left lobe of the liver and the spleen, while in the abdominal MRI scan bilateral adrenal tumors   of the head disclosing a 1.4 × 1.3 cm pituitary adenoma, cervical ultrasound and Tc99 m Sestamibi-scans both disclosing hyperplasia of the parathyroid glands, abdominal MRI scan and endoscopic ultrasound (EUS) both disclosing a 4.5 × 3.0 cm tumor in the body and tail of the pancreas, fine needle aspiration biopsy (FNAB) of the pancreatic tumor under EUS guidance concluding in histological findings compatible to neuroendocrine tumor, somatostatin receptor imaging with Indium 111  a 1.4 × 1.3 cm prolactine producing pituitary adenoma, (ii) primary hyperparathyroidism due to hyperplasia of the parathyroid glands, (iii) a 4.5 × 3.0 cm non functioning endocrine tumor in the body and tail of the pancreas and (iv) functioning bilateral adrenal tumors, was established.th postoperative day.The patient was submitted to an exploratory laparotomy through a bilateral subcostal incision. She underwent distal pancreatectomy, splenectomy, bilateral adrenalectomy and nephrectomy of the ectopic left kidney. Parathyroidectomy was postponed until the development of clinical or laboratory findings compatible to hyperparathyroidism. The patient had an uneventful postoperative recovery and she was discharge on the 7The histological findings were as follows: Distal pancreas (size 9.5 × 5.1 × 2.8 cm). Grossly, the pancreatic tissue exhibited multiple cystic and hemorrhagic lesions ranging from 0.3 to 2 cm in maximum diameter. In the peripancreatic fat five lymphnodes measuring 0.4 to 0.6 cm, were detected. Microscopically, the pancreatic lesions corresponded to neoplastic tissue consisting of uniform neoplastic cells with scant eosinophilic cytoplasm and stippled nuclei, arranged in nests and trabecula. There was mild to moderate nuclear atypia and the mitotic figures were less than 2 per 10 HPF (magnification X40). Neoplastic emboli in pancreatic vessels, as well as tumor nests in the peripancreatic fat were also evident. The tumor appeared to extend within less than 0.3 cm of the surgical margins. The resected lymphnodes were free of metastatic deposits. The neoplastic cells exhibited immunopositivity for neuroendocrine markers (chromogranin and synaptophysin), while the proliferation index assessed by Ki-67 immunohistochemistry was about 5%. Taking into account the above histological observations  as well as the data from the literature, the diagnosis of well-differentiated endocrine carcinoma of the pancreas was established.Both adrenals glands. The left one weighed 250 gr, measured 10.1 × 7.3 × 4.5 cm and upon sectioning, a well circumscribed brownish tumor with central calcification sizing 7.5 × 5.5 × 4.1 cm, was found. The right adrenal gland weighed 120gr and measured 10.5 × 5.5 × 3.7 cm. Gross examination revealed the presence of a tumor measuring 8 × 4 × 3 cm, with features similar to those of the left gland, but without central calcification. Histologically, both adrenal tumors showed features of adrenocortical neoplasms. The neoplastic population consisted of round to oval cells, with scant eosinophilic cytoplasm and moderate to marked nuclear pleomorphism, arranged in a loose growth pattern  in exon 2 of MEN1 gene was detected in the postoperative genetic test for MEN1.MEN1 is a tumor suppressor gene located at chromosome 11q13 loci, encoding a 67 kD nuclear protein of 610 amino acids, called menin . VariousKnudson's two-hit model for tumorigenesis  addresseThe first clinical manifestation of MEN1 is hyperparathyroidism, mainly due to multiglandular hyperplasia, which usually affects more than 95% of all MEN1 patients . AlthougPancreatic tumours occur in about 30-75% of MEN1 patients and are the second commonest clinical manifestation of MEN1 . One thiThe incidence of pituitary adenomas in MEN1 patients varies between 10 and 60% , while tRegarding the adrenocortical lesions, DNA analysis for allelic loss at the MEN1 locus shown no loss of heterozygosity ,31, probSince the malignant potential of MEN1-related adrenal neoplasia is of important clinical significance, close biochemical and radiologic follow-up is recommended. Newly diagnosed adrenal lesions should undergone a control investigation after 6 months and in case of a stable lesion, control intervals of 2 years seem to be sufficient . AdrenalIn the patient we describe, a non functional, fully calcified ectopic pelvic kidney was also detected and surgically removed. Since ectopic kidneys are more susceptible to diseases such as nephrolithiasis, hydronephrosis, injury of aberrant vessels or overlying abdominal viscera and nerves or malignancy than the normally positioned ones, a non functional pelvic kidney requires removal .Preoperatively, the patient we describe, had elevated (7.5-fold) serum CA-125 levels. It is well known the high false positive rate and the poor sensitivity and specificity of the CA-125. Although cardiovascular, lung and chronic liver diseases are the most frequent diagnoses in patients with increased CA-125, other intra-abdominal non-malignant non-hepatic diseases can also cause elevation of the CA-125 , as in tMany proteins that localize to the nucleus contain a polybasic motif, the Nuclear Localization Signal (NLS), which is necessary for proper nuclear targeting. Menin contains two NLSs. Both NLS-1 (amino acid 479-497) and NLS-2 (amino acids 588-608) are present in the C-fourth of menin . Menin bThe patient we describe harbors the germline p.E45V mutation of the MEN1 gene which, to the best of our knowledge, has not been previously described in the literature data. It is a point mutation in exon 2 of MEN1 gene, in nucleotide 134 substituting Adenosine to Thymine, changing the Glutamic acid (GAG) to Valine (GTC) in the menin molecule. Although, pathogenetic mutations of glutamic acid either to lysine (p.E45K) or glykine (p.E45G) or alanine (p.E45A), have been previously described , the cloIn conclusion, we present a MEN1 patient with the novel p.E45V mutation in the exon 2 of the MEN1 gene, in whom bilateral ACC was detected. Whether this novel mutation predisposed to bilateral ACC development or whether bilateral ACC can also occur in MEN1 patients, remains to be proved.The authors declare that they have no competing interests.JG performed the operation and contributed to the preparation of the manuscript; ND assisted to the operation and wrote the manuscript; AZ prepared preoperatively and followed-up postoperatively the patient, wrote the manuscript and reviewed the literature; SS and KE performed the histopathology; SK performed the genetic test; PK performed the histopathology and immunohistochemistry; GK diagnosed the case, checked the final version of the manuscript and made significant comments. All authors read and approved the final manuscript.


Gastric schwannomas are rare mesenchymal tumours that arise from the nerve plexus of the gut wall. They present with non-specific symptoms and are often detected incidentally. Pre-operative investigation is not pathognomonic and many are therefore diagnosed as gastrointestinal stromal tumours (GISTs). Operative resection is usually curative as they are almost always benign, underpinning the importance of differentiating them from GISTs.Three cases of gastric schwannomas were identified over a seven-year period. The clinical details and management were reviewed retrospectively.There were two women and one man with a mean age of 62 years (range: 51–69 years). Two patients presented with bleeding and one with abdominal pain. The mean tumour size was 5.2cm (range: 2–10cm) and the tumours were resected completely following total or wedge gastrectomies. Histology in all cases showed spindle cells with a cuff of lymphoid tissue. Immunohistochemistry confirmed positive S100 staining and negative CD117 and DOG-1 staining in all cases.We report our experience with these unusual primary stromal tumours of the gut and their presentations, preoperative investigations, operative findings and pathological findings are discussed. Operative resection in all cases has been considered curative, which is supported by previous series confirming the excellent prognosis of gastric schwannomas. This is in distinct contrast to the major differential diagnosis clinically, endoscopically, radiologically and histopathologically, the GIST. We describe three cases of gastric schwannomas that underwent operative resection. The aim of this paper is to report our experience of these unusual tumours, highlighting diagnostic adjuncts, operative strategies and histological findings.Three patients with a histological diagnosis of gastric schwannomas were identified in one cancer network over a seven-year period (2004–20H). The clinical details and management of each case were analysed retrospectively. These details are summarised in A 51-year-old woman was investigated for atypical right upper quadrant pain and mildly elevated serum aspartate transaminase levels. Abdominal ultrasonography detected an incidental epigastric mass. CT revealed a 9cm x 6cm irregular homogenous lesion arising from the lesser curve of the stomach  and two At laparotomy, a 10cm lesion arising from the upper half of the lesser curve that was encasing her left gastric vessels was found and a total gastrectomy was performed. Histological examination revealed a benign gastric schwannoma with haphazardly arranged spindle cells and a cuff of lymphoid tissue peripherally  and 3. IA 69-year-old woman underwent gastroscopy for melaena and anaemia. A 3cm ulcerated tumour was identified on the greater gastric curve that was clinically suspicious of a GIST. Biopsy of the lesion was not diagnostic, revealing only inflammatory cells, and EUS demonstrated a well demarcat ed, submucosal lesion that did not breach the serosal layer. CT confirmed the location and size of the tumour , with noA 66-year-old man was investigated for anaemia with melaena and haematemesis with an upper gastrointestinal endoscopy. At gastroscopy, a 5cm submucosal lesion was identified with an area of central ulceration. It was located on the anterior gastric wall, approximately 5cm from the oesophagogastric junction. EUS measured the lesion at 5.8cm and illustrated that it had a 50% intraluminal component but there were no features of local invasion, lymphadenopathy or distal spread. Abdominal CT confirmed the absence of any metastatic spread and showed the lesion to be amenable to laparoscopic resection .At laparoscopy, a 4cm anterior tumour was seen and ontable endoscopy confirmed a 50% intragastric component. The tumour was excised in a wedge via an endoscopic stapler. Pathological examination confirmed a 55mm ulcerated tumour that was composed of spindle cells and mast cells. The tumour was surrounded by a florid lymphoid infíltrate. It was negative for CDH7 and DOG-1 staining and positive for S100 staining on immunohistochemistry.––Gastric schwannomas represent 2–7% of all gastrointestinal mesenchymal tumours (the most common types being GISTs and smooth muscle tumours).––et al, there has only been one reported malignant case of a gastric schwannomaGastric schwannomas can occur at any age but are most frequently noted in the fifth decade with a female predominance.Definitive pre-operative diagnosis can be difficult to establish due to the non-specific findings seen on cross-sectional imaging and endoscopy.CT shows that schwannomas have a homogenous appearance while the majority of GISTs (87–92%) are heterogeneous (often due to haemorrhage and necrosis within them).Tumours are usually described as having a firm or rubbery consistency with a homogenous tan-yellow or whitish cut surface.Another highly characteristic feature is that they lack the typical Antoni A and Antoni B areas of schwannomas seen elsewhere and may have pronounced nuclear pleomorphism (but very low mitotic activity). This is because many of these tumours are discovered incidentally and have been present for some time. They tend to therefore show features more characteristically associated with so-called ancient schwannomas as described above. Immunohistochemistry reveals positive staining for S100 but not for CD45, CDH7 or DOG-1, which helps distinguish them from GISTs.–Operative resection should follow the same principle as for GIST, including the lesion and any involved adjacent structures, with the goal of achieving an R0 resection. The extent of gastric resection is dependent on the size and location of the tumour as well as the presence of any invasion of local structures. Resection can be achieved via an open or laparoscopic approach. Reported outcomes are excellent given the predominance to benign disease.Our cases illustrate the spectrum of presentation of gastric schwannomas. Moreover, they highlight how these unusual tumours may be misdiagnosed pre-operatively  despite both radiological and endoscopic assessment. Pathological investigations were consistent with the published literature and operative resection should be considered curative as these tumours are benign.


Cavernous hemangiomas of the uterus are rare benign vascular lesions. Nine cases of diffuse cavernous hemangioma of the gravid uterus have been reported, most of which diffusely involved the myometrium. These vascular malformations are clinically significant, and may cause pronounced bleeding resulting in maternal or fetal demise. Thrombosis of cavernous hemangiomas of the uterus has been previously reported. We here report the first case in which a thrombosed cavernous hemangioma of the myometrium resulted in a fatal pulmonary embolism in a post-partum woman.Staphylococcus aureus in the sputum, leukocytosis and asystole. A transabdominal ultrasound examination showed heterogeneous thickened and irregular products in the endometrial canal. The laboratory values were relevant for an increased prothrombin time, activated partial thromboplastin time, ferritin and a decrease in hemoglobin. The clinical cause of death was cited as acute respiratory distress syndrome. At autopsy, a 400g spongy, hemorrhagic uterus with multiple cystic spaces measuring approximately 0.5 × 0.4cm filled with thrombi within the myometrium was identified. Immunohistological examination with a CD31 stain for vascular endothelium associated antigen confirmed several endothelium-lined vessels, some of which contained thrombi. These histological features were consistent with cavernous hemangioma of the myometrium. A histological examination of the lungs revealed multiple fresh thromboemboli in small- and medium-sized pulmonary arteries in the right upper and lower lobes without organization, but with adjacent areas of fresh hemorrhagic infarction.A 25-year-old obese African-American woman who had one pregnancy and was delivered of twins by cesarean section was admitted 1 week after the successful delivery. The 12-day clinical course included ventilator-dependent respiratory failure, systemic hypertension, methicillin-resistant This case underscores the importance of a high index of suspicion in a pregnant or post-partum woman presenting with respiratory symptoms. Thrombosis of the cavernous hemangiomas of the gravid or post-partum uterus is a rare entity. This case is of interest because it indicates that this condition can be fatally complicated by embolization of the thrombi in the cavernous myometrial hemangiomas. Although delivery by conservative methods, as well as cesarean section, is possible without resorting to hysterectomy, occasionally, the consequences could be fatal as in this case. Both capillary and cavernous hemangiomas rarely occur in the uterus. A search of the current literature reveals reports of fewer than 50 cases . CavernoSuch cavernous hemangiomas are associated with numerous obstetric and gynecological complications, ranging from intermenstrual spotting, menometrorrhagia and infertility, to maternal and/or fetal demise, resulting from pronounced bleeding of the gravid uterus -8. OccasStaphylococcus aureus in the sputum, leukocytosis and asystole. She was treated with gentamicin, ampicillin, clindamycin, Dilantin® (phenytoin sodium), Lovenox® (enoxaparin), Ativan® (lorazepam), hydralazine, and morphine. Acute respiratory distress syndrome (ARDS) was attributed to an infectious source, and multiple antibiotics were administered. The critical condition of the patient did not allow for a magnetic resonance imaging (MRI) study to locate sites of infection.A 25-year-old obese African-American woman who had one pregnancy and was delivered of twins by cesarean section was admitted 1 week after the successful delivery. She had collapsed, and was found unresponsive with bloody frothy fluid issuing from her mouth. Her social history included smoking tobacco and marijuana. The clinical differential diagnoses were eclampsia and post-partum cardiomyopathy. The 12-day clinical course included ventilator-dependent respiratory failure, systemic hypertension, methicillin-resistant A transabdominal ultrasonography (USG) examination showed heterogeneous thickened and irregular products in the endometrial canal. The laboratory values were relevant for an increased prothrombin time, activated partial thromboplastin time, ferritin and a decrease in hemoglobin. The clinical cause of death was cited as ARDS.At autopsy, a 400g spongy, hemorrhagic uterus with multiple cystic spaces measuring approximately 0.5 × 0.4cm filled with thrombi Figures , 2 withiHistological examination of the lungs revealed features of acute and early diffuse alveolar damage (DAD) and early organization with fibrosis. Multiple fresh thromboemboli in small- and medium-sized pulmonary arteries in the right upper and lower lobes without organization, but with adjacent areas of fresh hemorrhagic infarction, were identified Figure . ImmunohHemangiomas are benign tumors  originatHemangiomas of the uterus may occur at any age; the youngest patient reported in the literature was 14 years old . Such hePhysiological changes related to pregnancy involve hypertrophy of the myometrium, but not hemangiomatosis . During USG can reveal a thickened uterine wall and a mixed-echo texture suggestive of cavernous changes with turbulent flow . DopplerConservative treatments including carbon dioxide laser excision, knife excision, cryotherapy, radiotherapy, electrocauterization and uterine embolization have been attempted with varying results. In cases of uncontrolled bleeding especially during operative delivery, or not responding to conservative treatment, a hysterectomy is imperative. Successive pregnancies have been reported in women harboring cavernous hemangiomas of the uterus .In cavernous hemangiomas of the uterus, the uterine wall is partly or completely transformed into cavern-like arteriovenous fistulas as a result of a proliferation of arterial and venous vessels of various sizes which later replace the normal myometrium . A histoCavernous hemangiomas may be complicated by thrombosis, and such patients may develop DIC  due to pThis is the first case of a thrombosed cavernous hemangioma of the uterus resulting in fatal pulmonary thromboemboli. Grossly, a 400g spongy and hemorrhagic uterus was identified. Histopathological examination revealed multiple vascular spaces within the myometrium lined by endothelial cells. Immunohistological examination with a CD31 stain for vascular endothelium associated antigen confirmed the endothelium-lined vessels, a few of which were filled with thrombi. The histologic features were in accordance with the previously reported cases of cavernous hemangioma of the uterus . HistoloThe proximate cause of death was attributed to terminal thromboemboli from cavernous hemangiomatosis of the myometrium and pulmonary infarcts with severe DAD. Although thrombosis of the cavernous hemangiomas has been previously reported ,10, subsThis case underscores the importance of a high index of suspicion in a pregnant or post-partum woman presenting with respiratory symptoms. Cavernous hemangioma of the gravid or post-partum uterus is a rare entity which conventionally can be complicated by refractory uterine bleeding and corresponding symptoms of acute blood loss. Thrombosis of the cavernous hemangiomas is not uncommon. This case is of interest because it indicates that this condition can be fatally complicated by embolization of the thrombi in the cavernous myometrial hemangiomas. These can be undiagnosed clinically, as happened here. Cavernous hemangiomatosis of the uterus is a very rare condition in a pregnant or post-partum woman. Although delivery by conservative methods, as well as cesarean section, is possible without resorting to hysterectomy, occasionally, the consequences could be fatal as in this case.Written informed consent was obtained from the deceased patient’s next of kin for publication of this case report and any accompanying images. Copies of the written consents are available for review by the journal’s Editor-in-Chief.ARDS: Acute respiratory distress syndrome; DAD: Diffuse alveolar damage; DIC: Disseminated intravascular coagulation; USG: Ultrasonography.The authors declare that they have no competing interests.TB conceived the case report, performed the gross examination of the specimen, acquired the patient’s data, searched the literature, and drafted the manuscript. JW helped in the histopathological evaluation of the slides, and made critical revisions to the manuscript. ND performed the histopathological evaluation of the slides, and made a critical analysis of the manuscript. All authors read and approved the final manuscript.


In this report, we describe the case of a 46-year-old male who presented with a three-month history of progressive intermittent pain over the right posterior knee. Magnetic resonance imaging showed soft tissue masses over the right popliteal fossa. Surgery was performed, and histological examination revealed the mass to be a sarcomatoid carcinoma of poor differentiation. Fluorodeoxyglucose (FDG)-positron emission tomography showed FDG uptake in the lungs and in the right para-aortic and popliteal regions. On the basis of the morphological and immunohistochemical features of the specimens, the patient’s condition was diagnosed as a pulmonary pleomorphic carcinoma with multiple metastases. Systemic chemotherapy was initiated with paclitaxel and cisplatin. The patient then developed paraneoplastic hypercalcemia and ultimately succumbed to healthcare-acquired pneumonia. The results of this rare case indicate that pulmonary pleomorphic carcinomas respond poorly to combination chemotherapy with paclitaxel and cisplatin. The firm mass in the popliteal fossa that was situated behind the knee was considered to be a Baker cyst; however, the possibility of malignant metastatic sarcomas, such as pulmonary sarcomatoid carcinoma, should be considered in the differential diagnosis. In conclusion, we emphasize that pretherapeutic examinations should be the basis for the diagnosis of a mass lesion at either an unusual or usual site. Pulmonary sarcomatoid carcinomas (SCs) are a heterogeneous group of non-small cell lung carcinomas with a rare histological subtype, and they have been reported to have a poor prognosis –3,5. The9/l; hemoglobin count, 96 g/l; platelet count, 240×109/l; and serum calcium, 13.9 mg/dl. The patient immediately underwent surgery for resolution of the neurological symptoms. Pathological evaluation of the popliteal mass showed a poorly differentiated carcinoma, with sarcomatoid changes characterized by a solid and focal individual tumor composed of marked pleomorphic tumor cells of the soft tissue . In March 2012, magnetic resonance imaging (MRI) showed a large lobulated cystic mass filled with debris or tissue thickening, measuring approximately 5.9×5.4×8.6 cm over the popliteal fossa . The comt tissue . Immunoht tissue . The morThe present report describes a case of pulmonary PC complicated by a soft tissue mass in the right posterior knee with progressive post-popliteal soreness and stiffness. Pulmonary PCs are rare, accounting for 0.3% of all invasive lung malignancies, and they frequently present as large tumors with a mean size of 5–8 cm range, 1–28 cm) (–28 cm (1Pathologically, the majority of these tumors can be classified using light microscopy alone. The diagnosis of these tumors requires a resected specimen, largely owing to the histological heterogeneity and pleomorphism of the tumor. Small biopsy specimens with adequate cytological material presented with loose clusters of poorly differentiated epithelial cells, giant cells, malignant spindle cells and a necrotic background with neutrophils and lymphocytes, which are highly indicative of PC . The spiSystemic chemotherapy with a single course of paclitaxel and cisplatin was unsuccessful. The patient developed a paraneoplastic syndrome consisting of hypercalcemia, and eventually acquired pneumonia. A previous case report indicated that pulmonary PC responds well to a combination of gemcitabine and docetaxel ; howeverIn conclusion, pulmonary sarcomatoid neoplasms are rare, and we report here the first case of pulmonary PC with multiple metastases to the right posterior knee. The present case indicates that pulmonary PC with paraneoplastic hypercalcemia responds poorly to combination chemotherapy with paclitaxel and cisplatin. The addition of combination therapies, such as i.v. calcitonin and bisphosphonates, to anticancer drugs may be beneficial in cases of paraneoplastic hypercalcemia, which is associated with aggressive disease progression and a poor prognosis. Notably, we emphasize that pretherapeutic examinations should be the basis for the diagnosis of a mass lesion at either an unusual or usual site, such as the popliteal mass lesion presented in this case report.


This report describes a case of rectal cancer with endoscopically observable white nodules caused by distal intramural lymphatic spread. A 57-year-old female presented to our hospital with frequent diarrhea and hemorrhoids. Computed tomography showed bilateral ovarian masses and three hepatic tumors diagnosed as rectal cancer metastases, and also showed multiple lymph node involvement. The patient was preoperatively diagnosed with stage IV rectal cancer. Colonoscopy demonstrated that primary rectal cancer existed 15 cm from the anal verge and that there were multiple white small nodules on the anal side of the primary tumor extending to the dentate line. Biopsies of the white spots were performed, and they were identified as adenocarcinoma. The patient underwent Hartmann’s procedure because of the locally advanced primary tumor. The white nodules were ultimately diagnosed as being caused by intramural lymphatic spreading because lymphatic permeation was strongly positive at the surrounding area. Small white nodules near a primary rectal cancer should be suspected of being intramural spreading. Endoscopic detection of white nodules may be useful for the diagnosis of distal intramural spread. To avoid local recurrence of rectal cancer, it is important to determine an appropriate distance of the distal resection margin of the tumor. Distal intramural spread is one of the crucial factors involved in making the decision about the extent of the anal resection margin -5. TransA 57-year-old female presented with frequent diarrhea and hemorrhoids. Computed tomography (CT) showed bilateral ovarian masses  and three hepatic tumors diagnosed as rectal cancer metastases; multiple upper lymph node involvement including the superior rectal artery region and the inferior mesenteric artery region; and lateral lymph node involvement including the internal iliac, common iliac, and external iliac artery regions Figure . FluorodHistological examination of the biopsy from a white spot near the dentate line specimen showed moderately differentiated adenocarcinoma. Therefore, abdominoperineal resection was planned for the patient. However, because of peritoneal metastasis, the patient underwent Hartmann’s procedure. The primary tumor was 36 × 29 mm in diameter with a positive distal margin and circumferential resection margin  after surgery, and then the metastatic hepatic tumors shrank. The patient is doing well at 12 months after surgery.The pathological findings revealed moderately differentiated adenocarcinoma invading the serosa, which was exposed with metastatic lymph nodes. Lymphatic permeation was strongly positive, and the cancer cells formed a small mass Figure A. The smThe local recurrence rate of rectal cancer patients after surgery with total mesorectal excision is reported to be 6–9.7% -5,10,11.The pattern of metastases to the anus is implanted or lymphovascular metastases . ImplantLocal recurrence by lymphovascular metastasis to the anus has been shown to be uncontrollable despite wide resection . The preThis report presented a case of rectal cancer with white nodules on the anal side of the tumor. The white nodules were caused by distal intraluminal lymphatic spread. Endoscopic diagnosis of white nodules around the tumor may reduce local recurrence of rectal cancer.Written consent was obtained from the patient for publication of this study and the related photos.s.The authors declare that they have no competing interestAT performed the surgery, literature search, and write up of the manuscript. SY initiated the concept, performed the surgery, helped in the literature search, and gave approval for the final write up. KT performed the surgery. TH helped in the revision of the article. KM helped in the revision of the article. TW helped in the revision of the article. YM helped in the revision of the article. HY contributed to the clinical management of the patient and helped in the revision of the article. All authors read and approved the final manuscript.Writing assistance: Dr. Brian Quinn, Editor-in-Chief, Japan Medical Communication.


Liver angiosarcoma is a rare disease, however it still ranks as the third of most common primary liver maligancies. The prognosis of liver angiosarcoma is very poor with almost all patients with this kind of disease die within 2 years after diagnosis. No specific symptoms and signs are closely associated with this disease. Here, we report a case presenting shock status at first due to rupture of liver angiosarcoma- induced internal bleeding. After emergent transarterial embolization (TAE), she received partial hepatectomy two weeks later. 4 months after operation, she is still with a good performance status without obvious recurrence or metastasis identified. Hepatic angiosarcoma is a very rare disease, accounting for only 2% of primary liver malignancy -3; howevth, 2011, she was brought to our emergency department for aid by her families. In our emergency department (ER), unstable systolic blood pressure of 90 mmHg with tachycardia of 110 beats/min was found. Resuscitation was then given. Subsequent blood examination showed leukocytosis (161000/μL), anemia (9 g/dL), and thrombocytopenia (85000/μL). Abdomen distension with mild tenderness while pressed was also noted. Under the impression of internal bleeding, bedside echo was then conducted, which revealed intra-abdomen fluid accumulation and a liver mass. Abdomen computer tomography(CT) was thereafter arranged for this patient with contrast injection, which indicated a 3 cm hypervascular mass over segment 5 of liver with active bleeding (Figure An 83 year-old female complained about lower abdomen pain for two months. After one episode of fierce right upper abdomen pain occurred on July 12The resected liver contained a tumor composed of anastomosing vascular channels in an infiltrating growth pattern. The vascular channels were lined by atypical endothelial cells with enlarged hyperchromatic nuclei Figure . The othAfter operation, this patient recovered very well and was discharged two weeks later. Adjuvant chemotherapy and radiation were not given after taking the effectiveness of them and her old age into consideration. No local recurrence or distant metastasis has been found at the time of writing, 4 months after operation. This patient is now still with a good performance status.Angiosarcoma, a subtype of soft tissue sarcoma, is an aggressive malignant disease deriving from endothelium, lymphatics, or blood vessels. Angiosarcoma occurs most commonly in head and neck, followed by breast. Liver angiosarcoma is ranked as fifth in the list of most common seen sites of angiosarcoma  and is vLiver angiosarcoma occurs in the elder mainly . AlthougDue to the hypervascular characteristic of liver angiosarcoma, it is difficult to differentiate liver angiosarcoma from other vascular tumors in liver, such as hepatoma or adenoma, radiologically. As shown in the present case, CT and angiogram all indicated a hypervascular mass, which was in line with the image findings of hepatoma. After resection, pathologically, the resected liver showed a tumor composed of anastomosing vascular channels infiltrating surrounding hepatic tissue in a destructive fashion. The channels were lined by atypical endothelial cells with enlarged hyperchromatic nuclei. Immunohistochemically, the tumor was positive for CD31, CD34, and Vimentin, a mesenchymal marker, and negative for HAS and GPC-3, which excluded the possibility of hepatocyte origin. Taken together, the liver angiosarcoma was confirmed as the final diagnosis -12.The survival of patients with liver angiosarcoma is very poor with media survival of 6 months without treatment. Even after treatment, only 3% of patients were reported to live longer than 2 years -7, althoLiver angiosarcoma also easily induces severe intraabdominal hemorrhage due to spontaneous rupture, leading to subsequent tumor cell spillage and peritoneal tumor seeding, which further complicates the prognosis ,19. OnceSeeking a new strategy in addition to surgical resection to treat liver angiosarcoma should be prioritized due to its high recurrence rate and very dismal prognosis. Since traditional chemotherapy and radiotherapy could not benefit this kind of patients, combined with the rapid progress of current molecular biology, target therapy may be the further direction against liver angiosarcomaThis study has been approved by Chang Gung memorial hospital IRB board. The approved IRB number is 100-3732B. A copy of the approval of IRB is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.CY wrote the manuscript, Cheng-cheng carried out the pathological examination, Chun-nan Yeh helped wrote the manuscript, Huang-yang helped wrote the manuscript, Jui- Teng participated in data collection, Chan-Siu carried out the image reading, Chih-Lang helped data collection, Cho-li participated in pathological examiantion, Wen-yen participated in data collection, Kun-Chun finalized the manuscript. All authors read and approved the final manuscript.


Benign metastasizing leiomyoma (BML) is a rare disease that usually occurs in women of reproductive age. They typically have history of uterine leiomyoma treated with hysterectomy. BML can metastasize to distant organs, with the lung being the most common organ. We report two patients who presented with benign metastasizing leiomyoma to the lung. Our first case was a fifty-two-year-old female who presented with multiple lung masses, with a past medical history of uterine leiomyoma who underwent hysterectomy 17 years ago. A CT-guided biopsy showed benign appearing spindle cells and pathology confirmed her diagnosis with additional positive estrogen/progesterone receptor stains. Our second case was a fifty-six-year-old female who presented with multiple cavitary pulmonary nodules. She subsequently underwent a video-assisted thoracoscopic surgery (VATS) with wedge resection of one of the nodules. Pathology confirmed the diagnosis based on morphology and immunohistochemical staining strongly positive for estrogen/progesterone receptors. Benign metastasizing leiomyoma is a rare condition which may affect women of reproductive age. This should be considered in the differential in patients who present with multiple pulmonary nodules, especially with a history of uterine leiomyoma. Additional stains, such as estrogen/progesterone receptors, may need to be done to confirm the diagnosis. Benign metastasizing leiomyoma (BML) represents a rare disease entity. This may present as lesions in lymph nodes, deep soft tissues, mesentery, bones, central nervous system, and heart. However, the most commonly affected organ is the lung; thus, it might be confused with leiomyosarcoma . BMLs ocHere we report two patients who presented with the unusual presentation of benign metastasizing leiomyomas to the lungs.A fifty-two-year-old female presented with abdominal pain. An abdominal computed tomography (CT) scan demonstrated a right lower lobe mass. She then underwent a chest CT which showed a right lower lobe (RLL) mass (3.9 × 3.5 cm) with numerous smaller nodules throughout both lungs CT, . A positShe underwent a bronchoscopy and endobronchial ultrasound (EBUS) with biopsy of the right lower lobe (RLL) lung nodule and a 12R lymph node. These biopsies were nondiagnostic, so she underwent a CT-guided fine needle aspiration which showed benign appearing spindle cells. Given the persistent concern for malignancy, she was referred to our institution for further evaluation. Consequently, she underwent a video-assisted thoracoscopic surgery (VATS) with wedge resection of the left lower lobe lesion. Pathology microscopic examination demonstrated well-demarcated nodules composed of bland spindle cells forming broad fascicles. There were entrapped scattered mature adipose tissue and tubules lined by benign bronchoepithelial cells Figures . No mitoA fifty-six-year-old female presented to us with recently diagnosed multiple pulmonary cavitary nodules. Her past medical history included coronary artery disease, previous myocardial infarction, hyperlipidemia, diabetes type 2, Grave's disease, and a right renal stent for renal artery stenosis. She has no prior history of malignancy. On review of systems, she reported a dry cough, chest pain, dyspnea on exertion, palpitations, fatigue, weight gain, urinary frequency, and nocturia. She smoked 1 pack per day for 15 years and quit smoking 2 years prior. Her physical exam, including lung exam, was unremarkable.On chest CT angiography imaging, multiple bilateral, relatively thick-walled cavitary pulmonary nodules were found, measuring approximately 1 cm in size with prevalence in the upper lobes . She undBenign metastasizing leiomyoma (BML) is a rare disease that usually occurs in women of reproductive age with a history of uterine leiomyoma treated with hysterectomy. BML can metastasize to distant organs, such as the lung, skin, bone, mediastinum, lymph nodes, muscular tissue, heart, and retroperitoneum . Most paAlthough the specific etiology of BML has not been fully established, the presence of hormone receptors (estrogen/progesterone receptors) and amenability to antihormonal treatments strongly suggest a uterine origin . HoweverSince these tumors are rare, no definite treatment strategy has been established. Treatments used include surgical removal of the lung lesions. For multiple lesions not amenable to removeal, conservative antiestrogen management has been tried using oopherectomy or gonadotropin-releasing hormone analogue. Other antiestrogen therapies such as selective estrogen receptor modulator, progestrone, and aromotase inhibitor have been suggested , 12.With regard to the prognosis, patients with BML tend to have a favorable clinical course and outcome. Out of ten patients reported in a case series , only onBenign metastasizing leiomyoma should be in the differential diagnosis in females of reproductive age who present with pulmonary nodules and have a history of uterine leiomyoma. Other types of spindle cell neoplasm, such as sarcoma, nerve sheath tumor, and malignant melanoma need to be excluded. Immunostains to demonstrate smooth muscle origin and positivity of estrogen and progesterone receptor may be helpful in establishing the diagnosis.


Case 1. A 73-year-old female presents with a slight swelling on the right mandible, associated with an unilocular well-defined radiolucent lesion. Case 2. A 27-year-old female presents with a painful mandibular swelling associated with an unilocular radiolucent lesion posterior to the 4.8. Case 3. A 61-year-old male was casually detected presents with an unilocular radiolucent lesion distal to the 4.8. Conclusion. The OOC is a specific odontogenic clinicopathological entity that should be differentiated from the KCOT as it presents a completely different biological behaviour.The orthokeratinized odontogenic cyst (OOC) is a rare developmental odontogenic cyst that has been considered as a variant of the keratocystic odontogenic tumour (KCOT) until Wright (1981) defined it as a different entity. Surgery is the usual treatment, and recurrence or association with Gorlin-Goltz syndrome has rarely been described. In this report, we presented three cases of this pathology, and we review the principal clinical, histological, radiological, and therapeutic aspects.  The orthokeratinized odontogenic cyst (OOC) is a developmental odontogenic cyst relatively rare, arising from the cell rests of the dental lamina , 2. It wSince then it has received various designations, such as “orthokeratinized variant of odontogenic keratocyst” or “orthokeratinized cyst of the mandible”. It is not until 1998 that Li et al. suggest the term “orthokeratinized odontogenic cyst,” which is the most accepted at the present time , 5.The OOC occurs predominantly in males between the third and fourth decades, with a mean age of 33.5 years , 5. The Radiographically the cyst appears as a well-circumscribed, unilocular, or multilocular radiolucency that occasionally is associated with an unerupted tooth or with the root without causing resorption , 6, 7. DThe histopathological analysis confirms the diagnosis which shows a cystic cavity lining composed of a thin and uniform stratified squamous epithelium with a thick granular layer and orthokeratin , 8, 9. A differential diagnostic feature with the keratocystic odontogenic tumour is that no cases of OOC have been associated with nevoid basal cell carcinoma syndrome , 10.Surgical enucleation is the treatment of choice for the OOC, and low recurrence rate has been described between 0 and 2% of cases which is in marked contrast with the 40% recurrence of KCOT , 12.On this paper, we report three clinical cases of the orthokeratinized odontogenic cyst and a review on the main clinicopathological aspects.A 73-year-old female was referred presenting a slight asymptomatic swelling on the right mandibular angle and ascending ramus of years of evolution . The patRadiographically, the lesion appeared as a well-defined multilocular radiolucency on the right posterior mandible and ascending ramus . The comThe complete enucleation of the lesion was performed under general anesthesia, and the material obtained was analyzed for its histopathological figures. Histopathological examination of excised tissue revealed the presence of cystic cavity integrated by dense connective tissue with an inner lining of thin and uniform epithelium with marked granular layer and hyperorthokeratosis. Based on the clinical, radiological, and histological data, a diagnosis of orthokeratinized odontogenic cyst was made. After eleven years of follow-up, no sign of recurrence has been noted. A 27-year-old female presents with a slightly painful swelling in the region of the right inferior third molar of 15 days of evolution, with no other clinical data of interest, nor does she report toxic habits. The orthopantomography shows an unilocular radiolucent lesion, posterior to the 4.8, extending to the ascending ramus . The lesThe extraction of the 4.8 and nucleation of the lesion were made under general anaesthesia. In the histopathological analysis, a cystic lesion was recognized with a fibrous connective tissue capsule without inflammation, covered by a stratified epithelium with orthokeratosis and marked granular layer . With thAfter the excision of the lesion and the third molar extraction, the pain disappeared.The outcome was favourable, and after 15 months, no signs of recurrence were recognized.A 61-year-old male who was on a routine dental follow-up presents with a well-defined radiolucent lesion, posterior to the right inferior third molar and extended to the ramus (4.8) on the radiographic exploration . The pat The cavity was filled with keratin flakes . With thThe outcome was favourable, and after 9 months, no signs of recurrence have been recognized.The orthokeratinized odontogenic cyst is an infrequent developmental cyst that occurs most often between the fourth and fifth decades and with a male gender predilection 3, 2 : 1) . As in most cases described previously, our lesions were located in the posterior mandible and extend to the ascending ramus , 5, 11. Radiographically, the OOC may appear as a well-defined, unilocular or multilocular, radiolucent solitary lesion associated frequently with impacted teeth, and it on occasions can displace the inferior alveolar nerve. Histologically, the OOC shows a cystic cavity lined by a regular stratified squamous epithelium, usually thin and uniform about 4- to 9-cell layers thick. This epithelium presents a defined basal layer that exhibits palisade cuboidal or flat cells, with nuclear hyperchromatism, an intermediate layer of polyhedral cells with eosinophilic cytoplasm, and a thick superficial layer of orthokeratin , 2, 8. TIn relation to the immunohistochemical pattern, the OOC does not show activity of the epithelial membrane antigen (EMA) and of the carcinoembrionary antigen (CEA). Moreover, the level of expression of Ki-67 and p53 is lower than on KCOT that suggests a reduced proliferative activity , 2, 10. The differential diagnosis of the OOC includes other radiolucent lesions of the jaws, mainly odontogenic lesions such as dentigerous cyst or paradental cyst , 5. OdonConservative surgical removal with complete enucleation of the cystic lesion seems to be the treatment of choice , 5, 7. All our cases were removed with conservative enucleation and have had a periodical follow-up with no recurrence reported to date. We conclude that the orthokeratinized odontogenic cyst is an independent clinical and pathological entity of the keratocystic odontogenic tumour (KCOT) with a different prognosis. This lesion should be included in differential diagnosis of the radiolucent lesions of the jaws, preferably of the mandibular lesions associated with an impacted tooth.