An undifferentiated carcinoma with osteoclast-like giant cell tumors (UC-OGC) is a rare type of tumor, which predominantly occurs in the pancreas. Due to the rarity of UC-OGC, sufficient clinical data are not available and its prognosis following surgical resection remains unclear. In the current report the case of a 37-year-old female is presented, in whom an UC-OGC of the pancreas was removed and following this, a second carcinoma of the remnant pancreas was removed during a second surgical procedure. At the patient’s initial admission, the preoperative images demonstrated a well-demarcated mass with a marked cystic component at the pancreatic head. The patient underwent a pylorus-preserving pancreaticoduodenectomy. The final pathological diagnosis was UC-OGC of the pancreas and the tumor was considered to have been curatively resected based on the histopathological findings. Four years after the initial surgery, a small mass was detected in the remnant pancreas and a partial resection of the remnant pancreas was subsequently performed. Histopathologically, the tumor consisted of a poorly differentiated tubular adenocarcinoma. A retrospective pathological analysis showed a segment of a poorly differentiated tubular adenocarcinoma in the initial resected specimen. Therefore, the final diagnosis was considered to be an intra-pancreatic recurrence of UC-OGC. The patient survived 66 months following the initial surgery and 18 months since the second resection. A meta-analysis was performed in the current study by comparing UC-OGC patients who survived more than two years following surgical resection (long-term survivors) with those who succumbed less than one year following surgical resection (short-term survivors). The characteristics of the short-term survivors were patients of an older age, males, and those exhibiting smaller tumors, positive lymph node metastasis, and concomitant components of ductal adenocarcinoma, as well as pleomorphic giant cell carcinoma. The concomitant component of mucinous cystic neoplasm was not considered to be a prognostic factor. To the best of our knowledge, the patient in the current report is the first five-year survivor following a curative second resection. Anaplastic carcinoma of the pancreas is rarely observed and accounts for <10% of all types of pancreatic carcinoma . UndiffeA 37-year-old female was referred to the was referred to the Tsukuba Gastrointestinal Hospital  due to epigastralgia. The patient had no specific medical or family history. The laboratory data demonstrated elevated levels of serum amylase , however, the leukocyte count  and C-reactive protein level  did not indicate inflammation. Among the tumor markers examined, the carbohydrate antigen (CA) 19-9 and elastase-1 values were increased to 135 U/ml  and 8,600 ng/ml , respectively. Abdominal ultrasonography demonstrated a tumor containing a cystic component  in the pancreatic head. Abdominal computed tomography (CT) demonstrated a tumor containing a cyst-like low-density area and an enhanced septum . Lymph n2) was administered once every four weeks, with one rest week, for the six months following surgery, and no recurrence was observed until three years postoperatively.Based on these findings, the preoperative diagnosis of the cystic pancreatic tumor was a mucinous cystadenocarcinoma due to the interruption of the main pancreatic duct. A pylorus-preserving pancreaticoduodenectomy was performed to resect the suspected malignancy. Macroscopically, the tumor measured 4 cm in diameter and was covered with a relatively thick capsule; internal bleeding and necrosis on the cut surface was also observed . HistopaFour years following surgery, the patient’s CA19-9 level increased again to 380 U/ml. CT revealed a small lesion  in the remnant pancreas  and therA meta-analysis of patients with UC-OGC who underwent surgical resection was conducted in the current study. The inclusion criteria for the meta-analysis were as follows: i) Reports of UC-OGC published in English; ii) cases of patients surviving more than two years following surgical resection (long-term survivors); and iii) cases of patients who succumbed less than one year following surgical resection (short-term survivors). A statistical comparison between the long- and short-term survivors was performed.2 test). The localization of the tumor did not differ between the two groups. The maximum diameter of the tumor was found to be smaller in the short-term survivor group compared with those of the long-term survivors . The number of patients with a solid mass was greater in the short-term survivor group than in the long-term survivor group . The value of CA19-9 was not mentioned for all of the cases; however, the level of CA19-9 was increased in two of the three patients in the short-term survivor group, and one of two patients in the long-term survivor group for which the values were mentioned. The incidence of lymph node metastasis was identified to be significantly higher in the short-term survivor group compared with that of the long-term survivor group . A second surgery was performed on only one patient in the short-term survivor group and on three patients in the long-term survivor group; one patient from the long-term survivor group succumbed shortly after the surgery. One patient in the short-term survivor group did not undergo any surgical resection. Dworak et al . –24. 4–24The present study reported the case of a patient who exhibited UC-OGC of the pancreas and underwent two surgical resections, which resulted in a favorable long-term outcome. A meta-analysis using previous reports showed that the characteristics of the short-term survivors following surgical resection were an older age, males, and those exhibiting smaller tumors, positive lymph node metastasis and a concomitant component of DAC. The concomitant component of an MCN was not considered to be a prognostic factor. The current patient, to the best of our knowledge, is the first five-year survivor after undergoing a second curative resection.Giant cell tumors of the pancreas are rare neoplasms, which present as two variations. One variation is UC with a pleomorphic/sarcomatoid growth pattern and multinucleated tumor giant cells ,2. UC-OGet al  The tumor was a metachronous metastasis in the remnant pancreas; or ii) the tumor was a multifocal secondary carcinoma. As a poorly differentiated tubular adenocarcinoma was retrospectively identified in a section of the initially resected specimens, it was speculated that the tumor of the remnant pancreas was an intra-pancreatic metastasis.In conclusion, the meta-analysis demonstrated that the characteristics of the patients in the short-term survivor group following surgical resection were those of an older age, males, and those exhibiting smaller tumors, positive lymph node metastasis and a concomitant component of DCA. The current patient, to the best of our knowledge, was the first five-year survivor following a curative second resection, which has been reported thus far in the English literature.


The current study presents a rare case of osteoblastic osteosarcoma arising from an extremely rare site, namely, the parapharyngeal space. To the best of our knowledge, this is the first study of computed tomography (CT) and magnetic resonance imaging (MRI) of an osteosarcoma in the parapharyngeal space with pathological correlation. A 56-year-old male presented with a mass of the right facial region. CT and MRI showed a heterogeneous mass, with ossification or calcification, occupying the parapharyngeal space. Open biopsy revealed an osteoblastic osteosarcoma containing calcified malignant osteoid. Lung CT also showed multiple lung metastases at the time of the first visit to the Department of Orthopedic Surgery, Osaka City University Graduate School of Medicine . Systemic chemotherapy and radiotherapy were administered to the patient for palliation. The patient was alive at the 24-month follow-up subsequent to this treatment. Although a definitive diagnosis requires the use of a biopsy, the CT and MRI findings described in the present study suggest inclusion of this rare tumor in the differential diagnosis that is formed when such findings occur in the parapharyngeal space. The present study also briefly discusses osteosarcoma of the parapharyngeal space. Osteosarcoma is rare in the head and neck, accounting for <0.5%  of all mA 56-year-old male presented with a mass in the right facial bone that had been apparent for three months, and dysesthesia, which had occurred gradually at this site. The patient incidentally identified the tumor and visited Wakakusa-Daiichi Hospital, Higashi-Osaka , where the mass was confirmed with computed tomography (CT) examination. Nothing of note was found in the patient’s past medical and family histories, and the findings from the routine laboratory studies were within the normal limits. Plain X-rays showed no particular findings, but regional CT showed a soft-tissue mass with prominent ossification in the central region, and involving the parapharyngeal space , in whic2 Adriamycin and 120 mg/m2 cisplatin. However, subsequent to two courses of the regimen, a perforation of the sigmoid colon was found, and surgical repair was immediately performed with a colostomy. Moreover, irreversible renal dysfunction occurred, and this was worsened by sepsis following surgery. No discernable effect was observed from the chemotherapy on the mass in the parapharyngeal space and the lung target lesion. High-dose chemotherapy was deemed to be unsuitable for treating conventional osteosarcoma owing to the adverse effects. Thus, local radiation therapy, with a total dose of 39 Gy, and a total of seven courses of low-dose systemic chemotherapy (50–100mg/m2 cyclophospamide per day) for palliation, were administered. At the last follow-up, 24 months after the first visit, the size of the mass occupying the parapharyngeal space was unchanged, but the size and number of lung metastases had increased. At the time of writing this study, the patient was alive with the disease. Written informed consent was obtained from the patient for publication of this case study and the accompanying images.An open biopsy was performed, and histologically, the lesion showed atypical cell proliferation with production of calcified malignant osteoid. The pathological diagnosis was of an osteoblastic osteosarcoma . The disOsteosarcoma is a primary high-grade malignant tumor in which the neoplastic cells produce osteoid . OsteosaRadiologically, tumor matrix mineralization and aggressive bone destruction are strongly suggestive of osteosarcoma. The present case demonstrated a high-density mass in the central area of the tumor on CT examination. On MRI, an intermediate intensity region was observed T1-weighted images and a heterogeneously high intensity region was observed on T2-weighted images. An ossified region in the central lesion, which was produced by tumor cells, showed a low intensity on each of the T1- and T2-weighted images. There was no discernible bone-forming reaction to indicate an osteosarcoma .Histologically, osteosarcoma is an osteoid-producing tumor, and the identification of anaplastic stromal cells and the osteoid they produce aid in the histological diagnosis. The incidence of the classic type of osteosarcoma presents as osteoblastic 50%), chondroblastic (25%), or fibroblastic (25%) in the extremities . Among h0%, chondOsteosarcoma usually presents with an aggressive course, with a high rate of distant metastasis and recurrence. In the absence of distant metastasis, the major therapeutic approach is surgery to achieve local control for high-grade osteosarcoma; surgery with a negative margin is necessary , and mulet al (The five-year survival rate of patients with head and neck osteosarcoma is estimated to be 57–63% ,11, whicIn conclusion, the present study presents a rare case of osteoblastic osteosarcoma arising from a rare lesion of the parapharyngeal space. Although a definitive diagnosis requires a biopsy, the CT and MRI findings described in this study suggest the inclusion of this rare tumor in the differential diagnosis when such findings occur in the parapharyngeal space.


Few reports have explored laparoscopic adnexal tumorectomy as a treatment for large and symptomatic ovarian leiomyomas during pregnancy. The current study presents the case of a patient with a large and symptomatic ovarian leiomyoma at 14 weeks of pregnancy. A laparoscopic adnexal tumorectomy was performed without complications. The laparoscopic management of large primary ovarian leiomyoma during pregnancy has not been reported in literature. Therefore, laparoscopy may be considered as a minimally invasive and feasible alternative to laparotomy for the treatment of large ovarian solid tumors during pregnancy, resulting in reduced postoperative pain, a smaller scar and shorter recovery time. By contrast, with respect to the ovarian solid tumor, surgery prior to gestation is advised, even for tumors of <3 cm in diameter, due to the probability of rapid growth of the tumor during pregnancy. Leiomyoma is a rare ovarian tumors, which accounts for 0.5–1% of all benign ovarian tumors . It is eIn October 2012, a 28-year-old female was referred to the local hospital due to two missed menstrual periods one month previously. Abdominal ultrasonography revealed a single normal fetus in the uterus and a right adnexal hypoechoic solid mass of 4.1×3.2 cm in size. The mass had been identified three years previously with a diameter of 2.5 cm during a routine gynecological check up and was followed-up by an ultrasound scan every six months for three years, which showed that the size of mass had remained stable. No further measurements were recorded due to the stability of the mass for a long period of time, without related symptoms. However, 16 days following the initial visit, the patient presented to the local hospital once again with a complaint of acute right lower abdominal pain following activity. An emergency ultrasound examination revealed that the adnexal mass had increased to 7×6.5 cm in size, without characteristics of torsion or rupture. The pain was significantly alleviated 1 h later without any therapy. Due to the increasing size of mass and the presence of pain, the patient was referred to Zhejiang Provincial People’s Hospital  for surgical intervention.During the following days, mild intermittent abdominal pain was experienced, without fever, vomiting, vaginal bleeding or uterine contractions. Approximately one week later the patient was admitted to Zhejiang Provincial People’s Hospital  with slight abdominal pain on the right side. Physical examination revealed a tender mass of ~11×10 cm in size, located in the right upper abdominal quadrant region. The mass was palpable and movable, with clear edges. During the pelvic examination, the cervix was normal and no amniotic fluid or bloody discharge was observed. The uterus was movable with no tenderness, and was of the size corresponding to the gestational age.Ultrasound revealed a single live fetus and a partial cystic adnexal mass of 10.4×10×6.6 cm in size .Surgery was scheduled to remove the mass one week later. The patient underwent laparoscopic ovarian tumorectomy under endotracheal intubation and general anesthesia. During the surgery, basic vital signs, including oxygen saturation and the end-tidal carbon dioxide level of the patient, were monitored continuously by the anesthetist. The laparoscope revealed an enlarged uterus of the size corresponding to that expected for a three-month pregnant uterus, normal bilateral fallopian tubes and a normal left ovary. A mass arising from the right ovary was ~18×16×10 cm in size and grossly appeared solid, with smooth/shiny surfaces and marked superficial vascularity. No adhesion or infiltration was observed in the pelvis. The ovary was preserved and the mass was excised. Following the completion of tumor enucleation, the ovarian defect was sutured with 2–0 absorbable VICRYL suture  in a continuous manner, while bipolar electrocoagulation was used to secure hemostasis. The samples were removed using the bag-retrieval technique, where a 35×25 cm bag was used to remove the mass through the extended left trocar incision, and were subsequently sent to the Department of Pathology  for histological testing. The examination of frozen sections reported a benign spindle cell tumor with focal ischemic infarction. Pathological examination of the paraffin section following surgery demonstrated that the mass was comprised of typical smooth muscle cells, which formed strands and bundles arranged in a whorled interlacing pattern. Microscopically, ischemic infarction focus of the mass was observed, while significant nuclear atypia or pleomorphism was absent. Immunohistochemical staining with antibodies against vimentin, inhibin, α-smooth muscle actin and the α-helical rod domain of desmin was performed to confirm the diagnosis of this rare tumor. The tumor cells stained positively for vimentin, inhibin and α-smooth muscle actin, but not for the α-helical rod domain of desmin, which aided in confirming the diagnosis of ovarian leiomyoma .Fetal surveillance monitoring via ultrasound prior to and following surgery indicated that the surgery had been successful. Prophylactic antibiotics and progesterone at a dose of 20 mg twice daily for four days were prescribed following surgery to prevent miscarriage. The surgery time, defined as the period between the skin incision and the closure of the skin, was 118 minutes and the estimated blood loss was 800 ml. The patient experienced an uneventful postoperative recovery. Routine follow-up after discharge was conducted every six months for three years and no complications were observed. A 3.4-kg healthy baby was delivered spontaneously at full term.Due to the widespread use of routine ultrasound in maternity examinations, it has been reported that the incidence of adnexal masses in pregnant females is ~3%, of which 55% may spontaneously resolve completely or significantly decrease in size , but conet al (et al (et al (et al (et al (The first case of a primary ovarian fibroid was described in 1862 ,8, <100 et al  reportedet al . Wei et l (et al  presentel (et al , the patl (et al  reportedWe hypothesize that surgery is not to be considered for adnexal tumors of <3 cm in diameter during pregnancy. In the case presented in the current study, an adnexal tumor was observed in the patient, which had remained at ~2.5 cm in diameter for three years prior to gestation. However, the patient presented with symptoms of abdominal pain and rapid tumor growth during pregnancy, which suggested that surgical intervention prior to the birth should be considered for solid tumors identified before or during gestation, even for those <3 cm in diameter, in order to avoid complications during pregnancy.2 gas pneumoperitoneum (2 abdominal insufflation and increased intraoperative complications during pregnancy has not been investigated to date. This case supports the previously observed safety and feasibility of laparoscopic approach in pregnancy.Numerous studies have reported that a laparoscopic approach to the removal of ovarian masses provides benefits, including an improved view of the surgical site, reduced blood loss, fewer adhesions, decreased postoperative pain, shorter hospitalization and smaller scars, compared with transabdominal surgery. These advantages are particularly significant for gravid females. A laparoscopic approach for the management of benign ovarian tumors in pregnant females has become the preferred approach by surgeons, due to its efficacy and safety . Potentiritoneum . The patThe predominant challenges of laparoscopic tumorectomy of solid large ovary tumors during pregnancy are enucleation of the tumor and removal of the mass from the abdominal cavity, particularly when the mass exists in marked vascularity. During the surgical enucleation of large tumors with marked vascularity, bipolar and monopolar cautery, as well as ultrasonic scalpel devices were used to control bleeding, all of which were deemed entirely profitless exercises for the patient in the present case. Therefore, in this case, the tumor was enucleated and the residual ovary tissue was sutured rapidly for hemostasis, based on a skillful laparoscopic technique. However, ~800 ml of operative blood loss was observed. Temporary ligation of the ovarian pedicle, the proper ligament and the suspensory ligament may be promising in reducing operative blood loss; however, this approach was considered in this surgery. An additional challenge is the integral extraction of the solid tumor. Although the morcellation technique is prevalent in laparoscopic surgery for solid tumors, an endobag remains a necessity when removing the tumor, in order to minimize the probability of the diffusion and implantation of the tumor cells. During the surgery in the current case, the entire sample was enveloped in a 30×35-cm medical endobag, and removed via the trocar incision at the left anterior axillary line, which was extended to 3 cm following the initial scalpel incision.In conclusion, to the best of our knowledge, no studies have investigated the laparoscopic management of primary ovarian leiomyomas during pregnancy. The case presented in the current study demonstrates that symptomatic ovarian leiomyoma may be successfully managed without complications by laparoscopic surgery, even when large in size and during pregnancy. By contrast, when considering solid progestational tumors, operative therapy prior to gestation is advised, even for tumors <3 cm in diameter, due to the probability of rapid growth of the tumor during pregnancy.


Non-Hodgkin's lymphoma of the cervix is an extremely uncommon entity, with no standard established treatment protocol. A 43-year-old asymptomatic female with a history of dual hit blastic B-cell lymphoma/leukemia in complete remission presented with an incidental cervical mass, which was initially felt to represent a cervical fibroid on computed tomography (CT). It was further evaluated with ultrasound, biopsy, and positron emission tomography-computed tomography (PET-CT), which demonstrated a growing biopsy-proven lymphomatous mass and new humeral head lesion. The patient was started on chemotherapy to control the newly diagnosed humeral head lesion, which then regressed. She then underwent radiation to the cervix with significant improvement in the cervical lymphoma. A review of cross-sectional imaging findings of lymphoma of the cervix is provided, including how to differentiate it from other more common diseases of the cervix. Clinical awareness of rare cervical masses such as lymphoma is very important in order to achieve timely diagnosis and appropriate treatment. Lymphoma of the female genital tract is very rare, accounting for less than 0.5% of gynecologic malignancies and 1.5% of extranodal non-Hodgkin's lymphoma . The invClinical symptoms are nonspecific and may be similar to squamous cervical carcinoma or endometrial adenocarcinoma with pain, vaginal discharge, and bleeding or in this case be asymptomatic. Most cases are incidentally detected by Papanicolaou smear tests, but some are incidentally detected with imaging, as in this case report in which initial detection was with CT .A 43-year-old female presented to the gynecology clinic with an incidental 2.3 cm × 2.2 cm mass in the cervix detected on CT, which was initially felt to represent a fibroid. She had no gynecologic symptoms. Her recent PET-CT scan from 2 months prior was normal in the cervical region Figures . She hadA transvaginal ultrasound performed 2 weeks later demonstrated a large heterogeneously echogenic vascular mass measuring 4.6 × 4.1 × 3.6 cm, involving most of the cervix Figures . It incrAn ultrasound-guided fine needle aspiration (FNA) performed a few days later revealed malignant B-cell lymphoma, consistent with her known history of blastic B-cell lymphoma. The aspirate showed large atypical lymphoid cells in a background of extensive necrosis. Immunophenotyping by flow cytometric analysis demonstrated a population of monotypic B-cells expressing kappa light chain which were positive for CD19, CD10, and CD38 and negative for CD5, CD200, CD22, CD43, CD11c, CD23, CD79b, and CD30. Immunoperoxidase stains (CD3 and Ki-67) performed on cytospin preparations showed a Ki-67 proliferation index of 80%. These findings were consistent with involvement related to the known blastic B-cell lymphoma Figures .A PET-CT obtained 10 days later again demonstrated the new metastatic lesion in the left humeral head . It alsoThe patient began systemic first salvage chemoimmunotherapy with dose-adjusted EPOCH , ofatumumab, dose reductions of bortezomib and continuous infusion of vincristine owing to peripheral neuropathy. The humeral head metastasis significantly improved .The patient then subsequently underwent intensity-modulated radiation therapy (IMRT) to the cervix. The cervical lymphomatous mass subsequently regressed in size, with no measurable abnormal SUV uptake on subsequent PET-CT Figures .Lymphomas of the female genital tract are very rare. The median age of presentation is 44 years, and the range is from 27 to 80 years. Clinical symptoms are nonspecific and may be similar to squamous cervical carcinoma or endometrial adenocarcinoma, with vaginal bleeding, perineal discomfort, or vaginal discharge . InvolveIt is important to be aware of the imaging findings of lymphoma of the cervix as it occurs rarely, and how to differentiate it from more common diseases of the cervix. Cross-sectional imaging modalities including ultrasound, MRI, and PET-CT are useful in the detection of lymphoma in the cervix.On ultrasound, lymphoma of the cervix presents as a solid mass. The larger lesions tend to be lobulated. The mass typically has abundant vascularity on the Doppler imaging. Other entities to consider in the workup of a cervical mass are fibroids and carcinoma . FibroidMRI is used for tumor delineation with excellent spatial resolution and tissue contrast. An endorectal coil can be useful due to close proximity to the cervix and provides better tissue delineation due to its superior spatial resolution in a relatively small field of view (FOV) . Use of CT imaging is not the modality of choice for evaluating lymphoma of the cervix because the findings are nonspecific with uterine enlargement and lobulated contour, which is also seen commonly with fibroids . It is mPET-CT is used for localizing and identifying sites of lymphoma which may not be readily apparent on CT. It is also used for identifying lymph node or bone marrow involvement, which is fluorodeoxyglucose- (FDG-) avid. One must be careful to exclude other uterine causes of FDG uptake which include myomas, postpartum state, normal physiologic uptake in the uterus, and other malignancies such as cervical cancer and endometrial cancer . PET-CT The main differential diagnosis for a cervical mass includes a fibroid and cervical carcinoma. Diagnosis of non-Hodgkin's lymphoma in this case was obtained by direct tissue sampling. Cervical biopsy and immunophenotyping are necessary to differentiate primary cervical non-Hodgkin's lymphoma from benign and malignant disease of the cervix –15.Since B-cell lymphoma of the cervix is such a rare entity, a standard therapeutic regimen has not been established . AccordiAwareness of non-Hodgkin's lymphoma of the cervix is important in order to provide timely diagnosis and treatment. Prognosis depends on the lymphoma type and tumor size , 23. Cas


Masses or mass-like lesions located in proximity to mitral valve encompass a wide range of differential diagnoses including neoplasias, abscesses, thrombi, and rarely caseous calcification of mitral annulus. Due to asymptomatic presentation, its diagnosis is usually incidental. Echocardiography is the first choice of imaging in evaluation. Cardiac computed tomography (CT) is helpful in establishing diagnosis by showing dense calcifications while cardiac magnetic resonance imaging (MRI) is used primarily as a problem solving tool. Imaging in evaluation of mitral annulus caseous calcification is essential in order to prevent unnecessary operations. Caseous calcification is a rare cause of mitral calcification, encountered more commonly in elder women. Since it is a benign process, differentiating it from malignant intracardiac masses precludes unnecessary surgical interventions. Various imaging modalities can be used for definitive diagnosis. Herein we present four cases of caseous calcification of mitral annulus (CCMA).A 76-year-old male patient with symptoms of tachycardia, palpitation, sweating, and headache was admitted to our hospital. His history for coronary or cerebral atheroslerotic disease was not remarkable. Physical examination revealed diastolic murmur. The transesophageal echocardiography demonstrated hyperechoic mass in posterior leaflet of mitral valve and mild mitral regurgitation. There was not accompanying leaflet prolapse or mitral stenosis. Cardiac magnetic resonance imaging (MRI) was performed for further evaluation. It showed a hypointense mass-like lesion of 2.5 × 2 × 3 cm. Lesion was hypointense in both T1- and T2-weighted sequences and showed peripheral enhancement in postcontrast delayed series. The aforementioned MRI findings were suggestive of caseous necrosis Figures . MultislA 62-year-old female patient presented with chest pain and history of essential hypertension. Physical examination was unremarkable. Laboratory tests revealed no abnormality except minimal high level of LDL cholesterol. Multislice coronary artery CT angiography demonstrated caseous calcification of mitral annulus. CT examination also revealed atherosclerotic wall thickening and millimetric plaque formation in arcus aorta and descending aorta as well as approximately 50% stenosis in proximal left anterior descending artery.A 57-year-old female patient having a history of lupus nephritis was admitted to our hospital with complaints of shivering and fever. She has been undergoing dialysis for several years due to chronic renal failure. She did not have history of atherosclerotic vascular disease. On echocardiography, calcific mass-like lesion was noticed and, with respect to her clinical findings, infective endocarditis was considered as presumptive diagnosis and the patient was referred to radiology for further evaluation. The lesion's echocardiographic features were not suggestive of Libman-Sacks endocarditis. Thorax CT confirmed calcific nature of lesion and showed minute atherosclerotic plaques in coronary arteries and aorta. In order to document the exact nature of lesion located in mitral annulus, cardiac MRI was performed. MRI showed diffuse hypointensity in polypoid lesion in all sequences compatible with caseous calcification. Blood samples did not reveal any bacterial overgrowth suspicious for infective endocarditis.A 69-year-old female patient was referred to our institution with suspicion of cardiac mass in transesophageal echocardiography. Cardiac MRI performed in our hospital confirmed the mitral annulus location of mass and peripheral contrast enhancement in delayed postcontrast series Figures . CT sectCCMA is rarely encountered in routine clinical practice, accounting for approximately 0.6% of all mitral calcifications. In different series, general prevalence is estimated to be 0.05% . PatientDiagnosis is usually based on various imaging modalities. Echocardiography is the mainstay diagnostic technique. Typical echocardiographic findings include an echogenic, round, or occasionally semilunar mass with well-defined borders, located usually on the posterior mitral annulus . A relatCardiac MRI may be extremely helpful in equivocal cases as a problem solving tool. CCMA is seen as a hypointense lesion both in T1- and T2-weighted sequences . In postMain differential diagnosis of CCMA includes cardiac neoplasms, endocarditis vegetations, abscesses, and thrombi located in left chambers. Cardiac heterogeneous mass lesions with accompanying local invasion and metastases favor the diagnosis of cardiac malignant neoplasm. In a septic patient or with a given clinical setting compatible with sepsis or widespread infection, presence of a mitral mobile mass should raise the suspicion of a vegetation or abscess . Myxoma CCMA is a rarely encountered clinical entity; however it should be kept in mind in the differential diagnosis of intracardiac masses especially when located near mitral annulus. Although echocardiography is the mainstay imaging modality for diagnosis of CCMA, definitive diagnosis can be established via multidetector CT and cardiac MRI.


With modern prenatal imaging, liver cysts are being diagnosed more often. Although large cysts are usually asymptomatic, they may present as an abdominal emergency requiring surgery in the first weeks of life. We report a series of 3 patients with prenatal diagnosis of isolated cystic liver lesions diagnosed at 22, 31 and 33 weeks of gestational age. The hepatic origin of the cysts was confirmed prenatally by a MRI in 2 cases, with visualization of a normal gallbladder. The prenatal course was uneventful. Postnatal ultrasound confirmed the diagnosis of liver cyst, showed normality of the biliary tract and in one case, rupture of the cyst during delivery. Because of an uncommon rapid increase in size, the 3 children underwent surgical excision of the cysts within the first weeks of life. These were non-bile-containing intrahepatic cysts arising from segment IV. Long-term follow up was uneventful. Improvements in fetal ultrasound (US) have increased the accuracy of prenatal diagnosis of congenital liver and biliary malformations. There are relatively few reports in the literature about prenatal diagnosis of isolated liver cysts [1-3]. Clinicians dealing with such fetuses are presented with the difficult task of prenatal counseling concerning neonatal management. Usually, if a liver cyst is suspected, it is of paramount importance to differentiate this lesion from other cysts, which require prompt neonatal treatment . Liver cysts are considered to be of good prognosis, especially if there is prenatal visualization of a normal gallbladder thus excluding the diagnosis of biliary atresia [4]. We report a series of 3 fetuses with prenatal diagnosis of isolated non-bile-containing intrahepatic cyst (NBIC), which presented unusual postnatal growth requiring surgery within the first weeks of life.Patient 1 :A 25-year-old woman, with no past medical history was referred to our tertiary care hospital following diagnosis of an abdominal cystic lesion in her male fetus. Her previous ultrasound (US) performed at 22 weeks gestation was normal. The US realized at 31st week of pregnancy showed an isolated anechoic cyst of 43x21mm, on the inferior part of liver. MRI confirmed the liver cyst. Spontaneous delivery occurred at 37 weeks. Postnatal US and CT (computed tomography) showed ascites due to rupture of the cyst. Spontaneous resolution during the first month of life was good with normal clinical findings, but at the age of six weeks, the baby presented with signs of acute abdomen  requiring emergency percutaneous aspiration followed by surgery because of cyst recurrence. Laparotomy confirmed the diagnosis of a NBIC in segment IV and marsupialization was performed.Patient 2 :A 34-year-old patient was referred to our institution following prenatal diagnosis at 33 weeks of pregnancy of a liver lesion in her male fetus. Our US confirmed a cystic lesion of 75x65 mm, arising from the right aspect of the liver with no vascularization abnormalities and normal biliary tract. CT-scan performed on the second day of life confirmed the liver cyst and surgery was performed on day 7 because of the size of the lesion. Marsupialization of a NBIC arising from right lobe was performed.Patient 3 :A 38- year-old patient was referred to us following prenatal diagnosis at 22 weeks gestation of a cystic lesion developed under and behind the right part of the liver. The lesion seemed to communicate with the gallbladder on ultrasound and initially a diagnosis of choledochal cyst was first envisaged. MRI confirmed the presence of a cystic lesion of right liver lobe as well as the lack of communication between the cyst and the biliary tract. The prenatal diagnosis was then revised to that of a liver cyst. Postnatal US confirmed the diagnosis of liver cyst and showed rapid growth and complete excision of the lesion was hence performed at 6 weeks of life because of digestive symptoms due to compression by the cyst. The NBIC was located in segment IV.In these three cases, histology confirmed the diagnosis of NBIC with a unilocular cyst with largely cuboidal epithelial lining and an underlying loose connective tissue. Long-term outcome was uneventful at 2 years follow-up with no recurrences, even in the first two cases that underwent only marsupialization.Prenatal diagnosis of congenital liver cyst is rare and their incidence is unknown  [5]. The prevalence of simple liver cysts in adults is about 3-18% [6]. Their etiology is unknown, and it is believed that they arise from aberrant bile ducts either by an inflammatory or obstructive process or by vascular disruption [7].As in our experience, NBICs are usually unilocular, and are characterized by cuboidal epithelium with no connection to the normal biliary tree [3]. The diagnosis of NBIC can be envisaged in presence of an anechogenic cyst surrounded by liver parenchyma on prenatal US. Doppler sonography can be of use by showing the relation between the cyst and the portal vessels and hepatic artery. Presence on US of septa within the cyst is usually regarded as a marker for other pathologies such as mesenchymal hamartomas.The differential diagnosis of these anechoic intrahepatic cysts includes choledochal cysts, biliary atresia  or duodenal duplication [4]. If there are multiples hepatic cysts, the possible diagnoses include congenital polycystic disease, congenital hepatic fibrosis or Caroli’s disease. Most hepatic cysts are diagnosed during the third trimester of pregnancy [8]. Liver cysts seem to be more frequent in females and in the right lobe, especially segment IV (which constitutes the bulk of the liver from the fourth to the eighth week of development) [9]. They are not associated with cysts in other organs, and can be completely intrahepatic, partially extra hepatic or pedunculated.Simple NBICs are rarely symptomatic unless they are large as in our series. In such cases, abdominal distension, feeding difficulties (as in patient 3), respiratory distress can be present and require surgical management within the first weeks. Acute complications have also been described, such as rupture (as in patient 1) or hemorrhage.Even if large (more than 10 mm in diameter) and/or complicated NBICs are rare, the possibility that these cyst might be symptomatic at birth and require urgent surgical management should be explained during prenatal counseling and guide where the birth will take place .Source of Support: NilConflict of Interest: None declared


Diverticulitis and carcinoma represent the most common causes of colon perforation, but other causes, like ingestion of foreign bodies, should be taken into account.We report the case of a 64-year old man presenting in our Emergency Department with a 2 days history of right lower abdominal pain, nausea, vomiting and low grade fever. Physical examination evocated mild pain with positive rebound tenderness in the right lower abdominal quadrant, and positive right costovertebral angle tenderness. Routine blood tests, abdominal X-rays and CT scan were inconclusive for perforation. At explorative laparoscopy a cecal perforation with localized peritonitis was found, and a right colectomy was performed due to the suspicion of cancer. Histological examination confirmed the presence of a perforation caused by a piece of wood (toothpick) of 6 cm in length.Foreign body ingestion should be taken into account in the evaluation of acute abdominal pain. A detailed patient’s history may be crucial for a correct diagnosis and treatment. Diverticulitis and carcinoma represent the most common causes of colon perforation. Other causes are iatrogenic perforation, penetrating abdominal trauma, and ingestion of foreign bodies. When animal bones, needles, toothpicks or other sharp pointed objects are ingested, the risk of perforation is even higher.Toothpick ingestion is a relatively rare event that may results in serious gut injuries with peritonitis, sepsis or even death. Interestingly, a 4-year survey performed in the United States found 8176 reported toothpick-related injuries yearly, a rate of 3.6 per 100000 person-years. We repoA 64-year-old man was referred to our emergency department with a two days history of right lower abdominal pain, nausea, vomiting and low grade fever. His vital signs were normal, except for a central body temperature of 38°C. There was no history of previous abdominal surgery. Physical examination evocated mild pain with positive rebound tenderness in the right lower abdominal quadrant, and positive right costovertebral angle tenderness. There was no evidence of intra-abdominal masses.3 with 84,6% neutrophils. On plain abdominal X-rays the small bowel was distended and dislocated on the left upper abdominal quadrant with some air-fluid levels  of 6 cm in length (Figure Routine blood tests were normal except for a WBC count of 11,860/mm Figure . The patA recent Medline literature research identified 136 case reports of ingested toothpicks.Table 1Toothpick ingestion is a medical emergency since it leads to acute abdomen and gut perforation. In fact, most patients swallow toothpicks while eating sandwiches and are not able to recall or even unaware of this event. Since spontaneous elimination of these sharp foreign bodies through the gastrointestinal tract is unlikely, foreign body ingestion should be taken into account during the evaluation of acute abdominal pain.The correct diagnosis is very difficult because of the low sensitivity of diagnostic investigations Table . EndoscoUnfortunately, our patient was unaware of swallowing a wood toothpick that rapidly progressed and reached the colon. CT scan was unable to identify the cause of symptoms. Considering the presence of an acute abdomen, with evidence of peritonitis, the patient was scheduled for surgical exploration and required hemicolectomy.This case suggests that a potential cause of lethal hazard may be unknown to the patient, but most of all it emphasizes the difficulty of a correct diagnosis with the most widely used instrumental exams, as endoscopy, ultrasound or CT scan.Consent to publish the case was obtained from our patient prior to discharge. The patient allowed us to share his story and clinical images because he understood the importance of raising awareness among physicians on this unusual surgical entity.


We report a unique first case of benign heterotopic pancreas arising within the proximal hepatic bile duct, containing a focus of intraductal papillary mucinous neoplasm (IPMN). The condition was diagnosed on pathological explant after left hepatic lobectomy with total extrahepatic bile duct excision. The condition of heterotopic pancreas is a relatively common finding, where small foci of pancreatic rest tissue arise in intra-abdominal sites other than the pancreas. First described in 1727, aberrant pancreatic tissue is defined as ectopic location of pancreatic tissue without vascular, neural, or anatomic connections to the anatomically normal pancreas . The majAn obese otherwise healthy 47-year-old female (body mass index 36) presented with intermittent nausea and right upper quadrant discomfort after intentional weight loss of 27 kg. The serum lipase was found to be mildly elevated at 132 units. Abdominal ultrasound demonstrated focal dilatation of the intrahepatic left hepatic ducts, and a subsequent contrast-enhanced magnetic resonance cholangiopancreatogram (MRCP) and endoscopic retrograde cholangiopancreatogram (ERCP) demonstrated an occlusive filling defect within the proximal main biliary tree . An intrThe imaging findings were concerning proximal hepatic duct cholangiocarcinoma (Klatskin tumor), although the CA19-9 was not elevated. The patient then underwent surgical exploration where a palpable filling defect was identified within the proximal hepatic duct. Intraoperative ultrasound demonstrated no additional lesions. For oncological clearance, a left hepatectomy with caudate lobectomy was completed, which required a triple right-sided bile duct Roux-en-Y reconstruction (two posterior and one anterior right bile ducts) over temporary externalized 5 French stents. No blood products were required, and no inflow occlusion was used. She had a smooth and relatively uncomplicated recovery other than a superficial wound infection.Surgical pathology demonstrated histological features consistent with heterotopic pancreatic tissue with IPMN contained within the proximal biliary tree .Sections showed superficial atypia with areas suggestive of papillary formation. There was also prominent bland duct proliferation with lobular architecture and microscopic foci of pancreatic acini surrounding the bile duct. The histological diagnosis was consistent with heterotopic pancreatic tissue. The superficial atypia with papillae suggests a low grade IPMN of the bile duct.At follow-up at 2 months, the patient had recovered fully, and the biliary stents were removed uneventfully.Continued advancement in cross-sectional and other imaging modalities facilitates early diagnosis and more precise treatment for hepatobiliary malignancies. We describe an extremely rare condition of IPMN arising within heterotopic pancreatic tissue obstructing the proximal hepatic duct. We believe this to be the first report of this condition arising within and obstructing the biliary tree. Interestingly SpyGlass cholangiography was able to demonstrate the lesion but was not able to precisely define the pathology. Furthermore, obstruction of the proximal biliary tree necessitated definitive resectional treatment. Had we been confident preoperatively of the benign nature of the filling defect, a less aggressive localized bile duct resection with hepatic parenchymal preservation could potentially have been entertained. However, the presence of IPMN change with potential for malignant transformation would still have provided concern. Preoperative diagnosis of heterotopic pancreas may be challenging and typically this is an incidental finding.Heterotopic pancreas is most commonly found in the proximal jejunal wall of the gastrointestinal tract. Pathological changes that occur within the native pancreas may also occur rarely within the heterotopic gland, including pancreatitis, abscess, and occasionally malignant transformation . IPMNs aIn conclusion, although the condition of heterotopic pancreas is usually asymptomatic and is often discovered incidentally, IPMN with potential for malignant transformation may rarely occur remotely within the biliary tree.


Primary pancreatic paraganglioma is an extremely rare extra-adrenal paraganglioma.We report a case of primary pancreatic paraganglioma undergoing middle segment pancreatectomy in a 42-year-old woman. Histological examination showed that the tumor was composed of well-defined nests of cuboidal cells separated by vascular fibrous septa, forming the classic Zellballen pattern. The chief cells showed positive staining to neuron-specific enolase, chromogranin A, synaptophysin, and the chief cells were surrounded by S-100 protein-positive sustentacular cells. The patient has remained tumor free for 12 months after surgery. A brief discussion about the histopathological features, clinical behavior, and treatment of primary pancreatic paraganglioma, and review of the relevant literature is presented.Primary pancreatic paraganglioma is a rare clinical entity, its diagnosis mainly depends on histopathological and immunohistochemical examinations. Complete surgical resection is the first choice of treatment and close postoperative follow-up is necessnary. Paragangliomas are rare neuroendocrine tumors (NETs) that arise from the extra-adrenal chromaffin cells of the autonomic nervous system, with an average annual incidence rate of only 2 to 8 per 1 million adults. Paragangliomas could derive from the extra-adrenal chromaffin cells of the sympathetic paravertebral ganglia of the thorax, abdomen, and pelvis, also arise from the parasympathetic ganglia located along the glossopharyngeal and vagal nerves in the neck and at the base of the skull. However, primary pancreatic paraganglioma is extremely rare. Herein, we present a case of primary pancreatic paraganglioma and review of the literature.A 42-year-old woman presented in September 2014 with recurrent upper abdominal pain for 3 months. She had no history of hypertension, headache, and palpitation. A physical examination revealed slight upper abdominal tenderness. Laboratory test results including liver function, renal function, and blood glucose were within normal ranges. Serum levels of CEA, CA19-9, and CA125 were normal. The level of 24-h urinary norepinephrine excretion was also normal. Unenhanced computed tomography (CT) revealed a 5.2 cm × 6.3 cm, solid, low density tumor on the body of the pancreas. On contrast-enhanced CT, the tumor demonstrated marked enhancement in the arterial phase Fig. . The chiPrimary paraganglioma that arises in the pancreas is rare. Whether this tumor type is an extension of a retroperitoneal tumor of true visceral origin, derived from ectopic paraganglia, remains unknown. To the best of our knowledge, only 21 cases of pancreatic paraganglioma having been reported from 1943 to the present in the literature  the classic Zellballen pattern composed of chief cells and sustentacular cells within the tumor; (ii) the chief cells showed positive staining to NSE, CgA, Syn, and negative for CK; and (iii) the sustentacular cells showed positive staining to S-100 or GFAP. Pancreatic paragangliomas are potential to be malignant –15, but 131-metaiodobenzylaguanidine (I131-MIBG) radiotherapy has been advocated in cases proven to be malignant [The first choice of treatment for primary pancreatic paraganglioma is complete surgical resection. Postoperative Ialignant , chemothWe report a case of primary nonfunctional paraganglioma on the body of the pancreas. Middle segment pancreatectomy could be a reasonable procedure for such tumor. Pancreatic paraganglioma has malignant potential, and requiring close postoperative follow-up.Written informed consent was obtained from the patient for publication of this Case Report and any accompanying images. A copy of the written consent form is available for review by the Editor-in-Chief of this journal.


Elastofibroma is a rare neoplasm that characteristically occurs in subscapular area in response to microtrauma. There are some reports of this tumor in other sites of the body but, up till now, there has been no report of elastofibroma in the face. A 20-year-old man presented with a slow growing painless mass in the face without any history of trauma. Histopathologic examination revealed a soft tissue mass composed of eosinophilic fibers admixed with aggregation of fat cells, capillary blood vessels, and fibroblasts. Elastic stain and Masson’s trichrome stain confirmed the nature of elastic and collagen fibers. It was a case of elastofibroma in the face.  Elastofibroma is a rare benign connective tissue neoplasm that mostly occurs in subscapular area of elderly patients, deep in the serratus anterior muscle. There ar2 which was easily movable .A Verhoeff-Van Gieson stain and Masson’s-trichrome stain identified elastic fibers and abundant collagen fibers, respectively . Based on microscopic findings and special staining, the diagnosis of elastofibroma was made. Up to now, there has been no clinical sign of recurrence during the 3-year follow-up of the patient.Elastofibroma is a benign process involving the subscapular region in most cases. However, isolated lesions have been reported in thigh, lip, deltoid muscle, and stomach. It was fMicroscopically, collagen bundles alternate with numerous degenerative fibers in irregular shapes are seen which stain strongly with elastic stain that is allied to mucoid materials, fibroblasts, and small collections of mature lipocytes.-16 In thTo the best of our knowledge, this is the first case of elastofibroma that has been presented as a facial mass. Thus, this benign soft tissue tumor should be considered in the differential diagnosis of painless soft tissue masses in the face.


Spinal subdural hematoma is rare and can cause serious neurological symptoms. Sometimes, idiopathic spinal subdural hematoma can spontaneously occur without any identifiable underlying etiologies. In this report, we present such an uncommon case of paraplegia caused by idiopathic spinal subdural hematoma that was successfully managed by laminectomy.A 45-year-old Chinese woman presented with sudden onset of progressive asthenia and numbness in both lower extremities, accompanied by difficulty in micturition. An initial non-contrast spinal magnetic resonance imaging at a local hospital suggested a spinal subdural tumoral hematoma at the T9 level. She was referred to our hospital and an emergency laminectomy from T8 to T10 was performed 22 hours after onset of her initial symptoms. However, nothing but a hematoma was identified during the operation, and a final diagnosis of spontaneous acute spinal subdural hematoma was concluded. She had partial return of sensations and voluntary movement after the operation.On imaging findings, spinal subdural hematoma could manifest as focal and independent from the dura matter, and, therefore, it should be included in the differential diagnosis of medullary compressive lesions. Spinal hemorrhages include epidural, subdural and intramedullary hemorrhage, of which spinal subdural hematoma (SSDH) is the least common form. SSDH usually manifests as a sudden onset of back pain and lower limbs paralysis. On a magnetic resonance imaging (MRI) scan of the spine, SSDH is usually hyperintensive on T2-weighted images and isointensive on T1-weighted images in acute phase -5. The oA 45-year-old Chinese woman presented with sudden onset of progressive asthenia and numbness in both lower extremities, accompanied by difficulty in micturition; she was first sent to a local hospital. Five hours later, she was unable to walk. She reported no fever, back pain, or trauma history. She had no past medical history of note and was on no medication. A general physical examination performed on admission revealed increased heart rate (94 beats per minute) and blood pressure (193/104mmHg). A neurological examination revealed a normal mental state, but grade 0 myodynamia and paresthesia in both lower limbs. An initial non-contrast MRI scan of her spine performed at the local hospital showed a well-demarcated, oval-shaped extramedullary lesion located at the T9 level, ventral to her spinal cord. The lesion appeared isointensive on T1-weighted images and hyperintensive on T2-weighted images, but gadolinium was not administrated in this scan  are important to a patients’ prognosis, special attention should be paid to those patients with SSDH who present untypical symptoms in future clinical practice. Although there are still some debates on the surgical indications for SSDH, we think emergency surgical hematoma evacuation at the local hospital or more prompt referral might be more preferable than simply conservative treatments. In fact, there is no consensus on the optimal management strategy for those patients with SSDH; strategies range from conservative treatments like intravenous methylprednisolone (pulse) therapy , to percAlthough preoperative images of the presented case suggest it to be an extramedullary intradural lesion, for example arachnoid cyst or tumoral lesion with proteinaceous or bloody fluid in it, the intraoperative surgical findings confirmed the diagnosis of idiopathic SSDH. Therefore, SSDH should be included in the differential diagnosis of those lesions that compress the spinal cord.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Primary cardiac tumors are rare and often asymptomatic or present with unspecific symptoms. Benign cardiac tumors of vascular origin are especially rare, with only few existing data in the literature.A 35-year-old Caucasian female patient presented to our department with an asymptomatic giant intracardiac angioma infiltrating both ventricles. Evaluation of this tumor involved electrocardiography, echocardiography, cardiac magnetic resonance imaging, coronary angiography, an open myocardial biopsy, and histological examination of the resected specimen. Because our patient was asymptomatic, she was managed conservatively with regular follow-up. We discuss the treatment options available in comparison with similar cases.Diagnosis and therapy of benign cardiac tumors, especially of asymptomatic lesions, can be a challenge. There is no evidence available to help in the management of such patients. An extensive evaluation is needed with different imaging modalities, and case-specific decisions should be made that involve experts in cardiology, cardio-oncology, and heart surgery.The online version of this article (doi:10.1186/s13256-016-0860-4) contains supplementary material, which is available to authorized users. Primary cardiac tumors are rare and their incidence ranges from 0.0017 to 0.28 % as reported in autopsy studies [2). Her medical history included bronchial asthma, previous gestational diabetes, and minor depression. She was taking beclometasondipropionat, fluticason-17-propionat and formoterol-fumarate-dihydrate for the bronchial asthma, and fluoxetine for the depression. A cardiac murmur was not detected during a routine physical examination. Laboratory parameters were unremarkable, with no elevation in her levels of high-sensitive cardiac troponin T , n-terminal pro-brain natriuretic peptide , or C-reactive protein . A 12-lead electrocardiogram showed T-wave inversion in the inferior and precordial leads (Fig. A 35-year-old Caucasian female patient was referred to our hospital because of an incidental finding of a large right ventricular mass during sonography of her upper abdominal organs performed for the evaluation of transient and moderate abdominal pain. Our patient did not have any specific cardiac symptoms like chest pain, dizziness, nausea, palpitations, syncope, or signs of congestive heart failure. Cardiovascular risk factors involved current smoking and obesity (body mass index 32.8 kg/mads Fig. . Late gaads Fig. . Online A transvenous right ventricular biopsy was performed to gain tissue material for histological analysis; however, the obtained material was insufficient for a definitive diagnosis. Our patient was then referred for an open myocardial biopsy via a partial inferior sternotomy Fig. . PreviouPrimary cardiac neoplasms are rare and have an incidence range of 0.0017–0.28 % as reported in autopsy studies .We report the case of a 35-year-old Caucasian female patient with an incidental finding of a large intracardiac angioma that represented a challenge for diagnosis and therapy. Echocardiography and CMR imaging are the most important diagnostic modalities for the evaluation of intracardiac masses . In addiet al. recently reported the total surgical resection of an incidentally discovered large hemangioma attached to the left ventricle with good results after 20 months of follow-up [et al. reported a case of a left-sided cardiac hemangioma in an adult woman that was managed conservatively for 11 years without major complications [There are very limited data with respect to the treatment of patients with these extremely rare benign intracardiac vascular tumors . Total sollow-up . Alternaollow-up , 11. ForThe choice between surgical and conservative management should be made on an individual basis for each patient. Malign arrhythmias, congestive heart failure, ventricular outflow tract obstruction, coronary insufficiency, and higher grade atrioventricular blocks are especially alarming symptoms that may promote a surgical approach. Interdisciplinary discussion involving the cardiologist, cardiac surgeon, and oncologist should be undertaken in such cases.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Granulocytic sarcomas are rare tumors composed of neoplastic blood cells, typically occurring during the course of acute nonlymphoblastic leukemia or before its onset. We present a case of a 23-year-old young adult man with metachronous granulocytic sarcoma of the testis without hematologic manifestations who was diagnosed with granulocytic sarcoma (GS). The patient was treated with right orchiectomy but relapsed with a left testicular mass 16 months later when a left orchiectomy was performed. The patient has been free of disease for 13 months following the left orchiectomy. This case highlights a rare hematologic cancer that urologists and pathologists should be aware of since it can present as a testicular mass. Only 3 cases of testicular GS without an associated hematologic disorder have been described. To the best of our knowledge, our patient is the first reported case in the English literature of metachronous GS of the testis with no evidence of hematologic disorder. Granulocytic sarcomas are rare tumors composed of neoplastic blood cells, typically occurring during the course of acute nonlymphoblastic leukemia or before its onset. It infiltrates an extramedullary site and appears as a localized tumor mass. It has been reported in association with acute myeloid leukemia (AML), chronic leukemia, and other myeloproliferative disorders. Rarely, granulocytic sarcoma (GS) may be found in patients without hematologic evidence of leukemia or hematologic disorder, which is a diagnostic challenge, as in the case we report here.In this paper, we present the first case in the English literature of metachronous GS of the testis.α-fetoprotein and β-human chorionic gonadotropin, and a systemic survey, which included chest radiography and abdominal computed tomography, could not identify any primary neoplasm or lymphadenopathy.A 23-year-old young adult man had a painless right hemiscrotal mass for 5 to 6 months. He visited the urology clinic. On physical examination, a 2.5 × 1.5 cm nontender mass was found in the right testicle. The patient had no history of any major systemic disease. Laboratory data revealed normal serum levels of An intratesticular hypoechoic nodule was detected with the use of scrotal ultrasound. The lesion was approximately 2.5 × 1.5 × 1 cm in size and was located at the cranial aspect of the right testicle.It had a well-demarcated, smooth border and had a homogeneously hypoechoic echo pattern relative to the ipsilateral testis . The patThe patient has been free of disease for 13 months following the left orchiectomy.On gross examination, the lesion appeared as a white, firm mass. Microscopic examination revealed diffuse infiltration of myeloblastic, monoblastic cells . On immuA bone marrow biopsy was performed, which revealed normocellularity and no tumor . CurrentGranulocytic sarcoma (GS), also known as chloroma, is a localized mass of primitive myeloid cells of the granulocytic series. The original name chloroma was first used by King  in 1853 There have been many case reports of GS in patients without leukemia in the literature. Most of the reports have suggested that GS almost invariably progresses to AML in patients without leukemia within a mean time span of 10 months , but thiThe prevalence of testicular involvement in patients with lymphoproliferative disease is high, up to 64% in patients with acute leukemia and 25% in patients with chronic leukemia [For a confirmative pathologic diagnosis of GS in the absence of leukemia, it is necessary to have light microscopic examination and immunohistochemical staining. The use of immunohistochemical stains such as those for myeloperoxidase, CD34, CD43, CD117, and lysozyme may identify up to 96% of extramedullary leukemia cases [GS has been described as occurring at many body sites, such as the skin, intracranial and pineal regions, orbit, mandible, soft tissue, epidural region, breast, liver, pancreas, stomach, small intestine, ovary, uterus, prostate, urinary bladder, and testis. The most common sites are the cranium, facial bones, subcutaneous tissue, and lymph nodes. Involvement of multiple sites has been reported, and tumors may recur at their original locations , 8.Economopoulos et al.  reportedThe patient in our case was treated with a right orchiectomy but relapsed 16 months later with a left testicular mass measuring about 5 × 4 cm in size, when a left orchiectomy was performed. The patient has been free of disease for 13 months following the left orchiectomy.This case highlights a rare hematologic cancer that urologists and pathologists should be aware of since it can present as a testicular mass. Only 3 cases of testicular GS without an associated hematologic disorder have been described in the literature . To the


Pachydermodactyly (PDD) is a rare and benign acquired form of digital fibromatosis, characterized by asymptomatic fusiform soft tissue hypertrophy of the lateral aspect of the proximal interphalangeal (PIP) joints of the fingers. The etiology of PDD remains unknown, but it usually affects healthy males around the age of puberty. It can be misdiagnosed as inflammatory rheumatic diseases, especially as juvenile chronic arthritis. Here, we report a case of PDD in an 18-year-old man who had progressive fusiform swelling of the PIP joint on his left middle finger. Although he had no pain or functional limitation of movement, he chose to undergo surgical resection of the lesion to obtain a conclusive diagnosis and to rectify the deformity's appearance. Histologically, the lesion was characterized by coarse fibrosis in the adipose tissue, peripheral nerve fibers, and eccrine glands; this is compatible with a diagnosis of PDD. Pachydermodactyly (PDD), from the Greek words pachy (thick), dermo (skin), and dactylos (finger), was first reported by Bazex et al. in 1973 . The diaAn 18-year-old man presented at our hospital with asymptomatic, gradually increasing fusiform swelling of the PIP joint in his left middle finger. He was right-handed. Over the past 5 years, he had undergone examinations by various specialists without a convincing diagnosis being made. On physical examination, there was diffuse thickening, predominantly, of the radial and ulnar aspects of the PIP joint, without any skin pigmentation or neurological deficits . He had PDD has been recognized as a distinct clinical entity because of its typical presentation of asymptomatic thickening of the soft tissues that overlie the radial and ulnar aspects of the PIP joints without any fundamental damage to the joint structures . The dis


Cystadenocarcinoma is primarily characterized by cystic structures of varying sizes, that are lined by epithelial cells. As a rare neoplasm, only four cases of cystadenocarcinoma of submandibular glands have been previously reported. Herein, we reported a unique case of submandibular gland cystadenocarcinoma in a 44-year-old man. By in large, this case had typical morphologic cystadenocarcinoma features. However, mucinous adenocarcinoma-like areas were additionally observed in the tumor tissues. This case was initially misdiagnosed as mucinous adenocarcinoma due to the low incidence of submandibular gland cystadenocarcinoma and the presentation of mucinous adenocarcinoma-like areas in the tumor tissues. Histopathology of additional tumor tissues revealed that mucinous adenocarcinoma-like areas accounted for only a small percentage of the tumor tissues, confirming the submandibular gland cystadenocarcinoma diagnosis.http://www.diagnosticpathology.diagnomx.eu/vs/1387916949121142The virtual slide(s) for this article can be found here:  A 44-year-old man presenting with a painless mass in the right submandibular region that he reported to have for 30 years visited our clinic in August, 2013. The patient complained that the mass had enlarged rapidly in the gland area during the past three months. Physical examination revealed a hard and nodular mass (7 × 6 cm) with an unclear boundary. The temperature and color of the overlying skin was normal, with no ulcerations or hemorrhaging. The patient’s regional lymph nodes were not enlarged. A magnetic resonance imaging (MRI) scan revealed an irregular mass in the right submandibular gland with multiple cystic regions  as a malignant tumor that rarely occurs in submandibular glands . SalivarBased on WHO descriptions, cystadenocarcinoma is primarily characterized by a cystic structure of varying sizes, lined by epithelial cells with or without papillary projections. In the first round of biopsy and histopathology examination, while these typical features were observed, we also identified a number of cysts lacking epithelial lining that were filled with abundant mucus-like materials. Scattered single tumor cells and cell clusters floated freely in the mucous lakes. These areas were shown to have conventional morphologic features of mucinous adenocarcinoma. Due to the low incidence of submandibular gland cystadenocarcinoma and presentation of mucinous adenocarcinoma-like areas, this tumor was initially misdiagnosed as a mucinous adenocarcinoma. Following consultation with senior physicians, additional tumor tissues were examined. Generally speaking, the majority of tumor tissues presented morphologic features of cystadenocarcinoma, but limited areas had cysts lacking epithelial cell lining caused by hemorrhaging and shedding of epithelial cells. The immunohistochemical staining of the markers mentioned above revealed no significant difference between these two types of tumors because both have glandular epithelium origins. Therefore, differential diagnosis primarily depends on examination of more tissues.Written informed consent was obtained from the patient for publication of this case report.The authors declared that they have no competing interests.JW and CZ conducted histological examinations. CZ drafted the manuscript. CC performed H&E staining, Immunohistochemical staining, and Alcian Blue and Periodic Acid Schiff staining. All authors read and approved the final manuscript.


Intussusception is invagination of a proximal segment of bowel into the distal segment in telescopic manner. Although intussusception is common among children, intussusception secondary to terminal ileal endometriosis in an adult is a very rare encounter. We present such a case of intussusception in a Sri Lankan female.A 43 year old Sri Lankan female presented to the surgical casualty unit with features of a subacute intestinal obstruction. Her past surgical and medical histories were unremarkable. On examination she was haemodynamically stable with distended abdomen and there was generalized tenderness. There was no guarding or rigidity. No masses were palpable. Bowel sounds were increased. Her urine was negative for Human Chorionic Gonadotrophin hormone. Full blood count revealed an increased white blood cell count with predominant number of neutrophils. Plain abdominal X-ray film showed dilated small bowel loops with empty rectum and distal colon. Patient underwent emergency exploratory laparotomy. An annular growth at terminal ileum was noted. Proximal bowel loops were distended. There was no free fluid in the abdomen. Ileo caecal tuberculosis was suspected and right hemicolectomy was performed. Uterus and bilateral ovaries appeared normal. Post surgical recovery was uneventful. The pathologist has noted endometriosis of terminal ileum contributing to the stricture formation and intussusception at the site. Following recovery patient was referred to a Gynaecologist for management of endometriosis.Though terminal ileal endometriosis is a very rare cause of intussusception it is important to consider the possibility of it, especially when a female patient of reproductive age presents with symptoms and signs of intestinal obstruction. Intussusception is invagination of a proximal segment of bowel into the distal segment in telescopic manner causing intestinal obstruction . This coA 43 year old, single and nulliparous female from southern province of Sri Lanka, presented to the surgical casualty ward with generalized colicky abdominal pain and constipation for 7 days duration. However she has passed flatus. She has had nausea and vomiting for 4 days duration which had been exacerbated on the day of presentation. Her usual menstrual periods had a cycle duration of 35–42 days and moderate bleeding lasting for 6–7 days with tolerable lower abdominal and back pain lasting for 3 days from the onset of bleeding. Her last menstrual bleeding had occurred 3 weeks prior. Her past medical and surgical histories were unremarkable. There was no family history of bowel carcinoma or inflammatory bowel pathologies.On examination patient was afebrile and not pale. She was moderately dehydrated and was in pain. Pulse rate was 130 beats per minute and blood pressure was 100/70 mmHg. Abdomen was distended and there was generalized tenderness. There was no guarding or rigidity. No masses were palpable. Bowel sounds were increased.9/L (per litre) with 80 % neutrophils. Plain abdominal X-ray film showed dilated small bowel loops with empty rectum and distal colon.Her urine HCG (Human Chorionic Gonadotrophin hormone) was negative. Full blood count revealed increased white blood cell count of 14 × 10A nasogastric tube was inserted and intravenous fluid resuscitation was begun. Exploratory laparotomy was planned. Midline abdominal incision was made and peritoneal cavity was opened into. An annular growth at terminal ileum was noted. Proximal bowel loops were distended. There was no free fluid in the abdomen. Ileo caecal tuberculosis was suspected and right hemicolectomy was performed. Uterus and bilateral ovaries appeared normal.Post surgical recovery was uneventful. Patient was started on prophylactic antibiotics. Bowel sounds appeared on post operative day 1 and she passed flatus and faeces on post op day 2 and 3 respectively. Patient was discharged from hospital 4 days following surgery and she had no complains on discharge.The pathologists report explained that on macroscopic examination the terminal ileum had been oedematous and enlarged with a diameter of 4 cm. A stricture had been identified in terminal ileum 2 cm away from caecum. An intussusception had been visualized at the site of stricture. Microscopic examination revealed endometriosis of terminal ileum contributing to the stricture formation and intussusception at the site.The patient was referred to a Gynaecologist for management of endometriosis.Small bowel obstruction is a common cause for surgical casualty admissions. It accounts for over 20 % of all hospital admissions due to acute abdominal pain. Malignant growths, strangulated herniae and adhesions are the common causes for small bowel obstruction in adults . EndometIntussusception is a common cause of bowel obstruction in children. Over 95 % of all reported intussusception cases have occured in children, whereas only 1–5 % were reported in adults . HoweverComplications of intestinal endometriosis include intestinal obstruction, hemorrhagic ascites, perforation and intussusception. If not treated immediately intussusception due to endometriosis may lead to intestinal obstruction and gangrene due to impediment of venous followed by arterial blood flow . The typThough terminal ileal endometriosis is a very rare cause of intussusception it is important to consider the possibility of it, especially when a female patient of reproductive age presents with symptoms and signs of intestinal obstruction.


Intradural herniation of a cervical disc is rare; less than 35 cases have been reported to date. A 52-year-old man with preexisting ossification of posterior longitudinal ligament developed severe neck pain with Lt hemiparesis while asleep. Neurological exam was consistent with Brown-Séquard syndrome. Magnetic resonance images showed a C5-6 herniated disc that was adjacent to the ossified ligament and indenting the cord. The mass was surrounded by cerebrospinal fluid signal intensity margin, and caudally the ventral dura line appears divided into two, consistent with the “Y-sign” described by Sasaji et al. Cord edema were noted. Because of preexisting canal stenosis and spinal cord at risk, a laminoplasty was performed, followed by an anterior C6 corpectomy. Spot-weld type adhesions of the posterior longitudinal ligament to the dura was noted, along with a longitudinal tear in the dura. An intradural extra-arachnoid fragment of herniated disc was removed. Clinical exam at 6 months after surgery revealed normal muscle strength but persistent mild paresthesias. It is difficult to make a definite diagnosis of intradural herniation preoperatively; however, the clinical findings and radiographic signs mentioned above are suggestive and should alert the surgeon to look for an intradural fragment. Intradural disc herniation is a known but rare condition, comprising less than 0.3% of all herniated discs. Over 90% of intradural disc herniations occur in the lumbar spine, less than 5% in the cervical spine . ExtensiHsieh et al. in 2010 reported a case in which a patient who suffered from ossification of posterior longitudinal ligament (OPLL) sustained a cervical intradural disc herniation after spinal manipulation therapy [The patient was a 52-year-old male with a history of posterior neck pain and subjective feeling of lower extremity weakness for 6 months. He woke up in the middle of one night with severe neck pain and Lt hemiparesis. The patient denied any recent trauma, chiropractic manipulation therapy, or even increased physical activity.At the emergency room he was noted to have grade 3-4 over 5 motor hemiparaparesis on the left side and partial loss of pain and temperature sensation on the right side, consistent with a diagnosis of incomplete Brown-Séquard syndrome. A CT scan showed segmental OPLL with a large ossified mass at the C5-6 level on the left . T2-weigBecause of the preexisting OPLL with spinal stenosis and the presence of cord edema consistent with a spinal cord at risk, the authors decided on a two-stage procedure. A laminoplasty was performed from C3 to C7 and secured using the Centerpiece plate fixation system . The posterior dura was intact and there was no CSF leakage. This was followed by an anterior C6 corpectomy. A tear was noted in the posterior longitudinal ligament (PLL) on the left side at the C5-6 disc and cranial C6 body level directly posterior to the OPLL mass. The PLL was carefully excised along with the OPLL fragment. There were spot-weld type focal adhesions between the PLL and the dura at the edges of the tear, but separation of the layers was possible. There was a 1 cm long dural defect which was plugged by an arachnoid mater bleb, with small amounts of CSF leakage. A fragment of herniated disc could be seen indenting the arachnoid . The disThe patient had marked relief of his fulgurant pain, along with improvement of motor strength. CT scan at 2 weeks postoperatively showed good position of the implants with relief of the stenosis . MRI at Permission to present this case report was obtained from the Medical Ethics Committee of the Shandong Provincial Hospital Affiliated to Shandong University.To the best of our knowledge, Dandy reported the first case of lumbar intradural disc herniation in 1942 . The truIt is difficult to make a definitive diagnosis of the disease before surgery. Clinically, the most commonly reported presentation is Brown-Séquard syndrome (15 out of 32 cases), as it was in our case. Horner's syndrome has also been reported. These syndromes are by no means specific for intradural cervical disc herniation, but in our opinion they are uncommon enough that, in combination with the imaging findings discussed below, they should alert the surgeon to be on the lookout for an intradural fragment.The pathogenesis of intradural cervical disc herniation is uncertain. Trauma is consistently listed by previous authors as a probable cause. Warade and Misra in 2014 claimed to have reported the first patient with a spontaneous cervical intradural disc herniation , but theYildizhan et al. in a cadaveric study published in 1991 showed that in some cases the dura mater was attached firmly to the posterior longitudinal ligament, most frequently at L4-L5, L3-L4, L5-S1, C5-C6, and C6-C7 interspaces [Iwamura et al. were able to procure a gross pathological specimen from their case . HypertrPan et al. noted that adhesion between ligaments and dura was often observed in case reports of intradural disc herniations . In the In our patient, OPLL probably caused chronic inflammation and mechanical irritation to the adjacent dura, leading to chronic scarring and adhesion of the thecal sac to the PLL. These adhesions also serve as a barrier to lateral migration of the fragment, forcing it directly dorsally through the annulus-PLL-dura layer. In We are not aware of any reliable method to definitely identify perforation of the dural sac on MR images. In some cases one may be lucky enough to see a clear nondisplaced PLL combined with a widened subarachnoid space. Several indirect signs have been reported as being suggestive of intradural extension in the lumbar spine. Choi et al. described abrupt discontinuity of PLL and hawk-beak sign on MRI . HidalgoMore recently, Sasaji et al. described what they called the “Y-sign” in the lumbar spine . In the The above mentioned signs are suggestive but not definitely predictive. We have experience with patients that had such signs on imaging, but during surgery the fragments were the usual retroligamentous epidural type. At present we feel that the diagnosis of intradural HIVD can only be confirmed by surgical exploration.Pan et al. stated that preoperative CT scan of cervical spine is undoubtedly important and necessary in the treatment of the cervical disease, especially in exclusion of presence of OPLL, and that it was a pity that CT scan was not taken at that time in the two cases described in his article . The autWe agree with most previous authors on this subject that an anterior discectomy is the treatment of choice, as it directly addresses the problem. Whether a corpectomy is needed depends on the size and location of the extruded disc fragment. In our case the presence of OPLL and myelomalacia further prompted us to start with a laminoplasty in order to gain extra space for the cord during the anterior discectomy and fusion.


The ameloblastoma is a benign but aggressive neoplasm of odontogenic origin. However, no enamel or hard tissue is formed by the tumor cells. Ameloblastomas are infamous for their invasive growth and their tendency to recur. Robinson (1937) as a benign tumor that is ‘usually unicentric, nonfunctional, intermittent in growth, anatomically benign and clinically persistent.’ They may occur at any age, even though nearly half of the tumors do occur between the ages of 20 and 40 years. This is the most common neoplasm affecting the jaws, yet only accounts for 1% of all tumors of the maxilla and mandible and 11% of all odontogenic tumors.This report presents a case of ameloblastoma involving entire ramus and part of body of mandible with resorption of the mesial and distal root apices of second molar and distal root of mandibular first molar. The lesion extending till the base of mandible surrounding the crown of the unerupted third molar resembling the dentigerous cyst. This was surgically resected followed by harvesting the contralateral sixth costochondral rib graft.How to cite this article: Celur S, Babu KS. Plexiform Ameloblastoma. Int J Clin Pediatr Dent 2012;5(1):78-83. This is the most common neoplasm affecting the jaws, yet only accounts for 1% of all tumors of the maxilla and mandible and 11% of all odontogenic tumors.2 It is an aggressive benign tumor of epithelial origin which may arise from the enamel organ,3 follicle, periodontal ligament,4 and lining of an odontogenic (dentigerous) cyst34 or the marrow of the jaws. Ameloblastomas are infamous for their invasive growth and their tendency to recur. Small incipient lesions may be mistaken for a common periapical granuloma or cyst and the tooth may be treated endodontically or extracted with the lesion going undiagnosed or inadequately treated. Differential diagnosis prior to definitive treatment is mandatory and necessitates a tissue biopsy as this lesion requires to be treated more aggressively than other benign periapical lesions. This case report presents a study of a plexiform ameloblastoma with surgical management.The ameloblastoma is a true neoplasm of enamel organ- type tissue which does not undergo differentiation to the point of enamel formation. It is described by Robinson (1937)A 13-year-old male presented with a small swelling in the vestibular area of the left second mandibular molar region. Extraoral clinical examination of the patient was notable for facial asymmetry and a firm swelling in the area of the left mandibular ramus region extending to the base of the mandible  and 2. IRadiographically, solitary radiolucent lesion involving entire ramus and part of body of mandible with resorption of the mesial and distal root apices of second molar and distal root of mandibular first molar. The lesion extending till the base of mandible surrounding the crown of the unerupted third molar resembling the dentigerous cyst . The medIncisional biopsy was done under local anesthesia and sent for histopathological examination. The pathology report confirmed the diagnosis of a plexiform ameloblastoma  and the Surgical resection of the tumor was carried out through an extraoral submandibular approach. Premolars were extracted and mandible was sectioned maintaining a safe margin of 1.5 mm of uninvolved bone  to 7. TeAfter a week-patient was recalled for the check-up, a radiograph was taken to ensure the stability of the graft  and 12.5 It comprises 1% of all radiolucent jaw lesions.6 Ameloblastomas arise from either neoplastic transformation of odontogenic cyst epithelium or from residual epithelial rests left over from the formation of teeth, such as remnants of the enamel organ (reduced enamel epithelium) found over the crown of an unerupted tooth, remnants of Hertwig’s epithelial root sheath  in the periodontal ligament or remnants of the dental lamina (rests of Serres). Ameloblastomas may be confused clinically with other jaw lesions, and occasionally with infiltrating neoplasms of the maxillary sinus, particularly those of salivary gland origin.5The ameloblastoma is a benign but aggressive neoplasm of odontogenic origin. However, no enamel or hard tissue is formed by the tumor cells.9with the average age being 27.18. There is no sex or racial predilection. Eighty percent of ameloblastomas occur in the mandible—almost exclusively in the molar ramus region of the mandible12 and are often associated with an unerupted teeth.13 The remaining 20% occur in the maxilla with the maxillary tuberosity being the most common site.3Ameloblastomas are nonencapsulated tumors.The age range for ameloblastomas is from 20 to 50 years3 The common denominator to all ameloblastomas is well-differentiated palisaded cells found around the periphery of nests, strands and networks of epithelium. Nuclei of the palisaded cells are typically polarized away from the basement membrane. Budding of epithelium from these proliferative nests and strands is also characteristic of this lesion. Palisading cells and budding epithelium are found in all microscopic subtypes .5Histologically, there are two basic patterns, follicular and plexiform with the former being more common. In both patterns the stroma is composed of mature fibrous connective tissue; however, the follicular form contains islands of epithelial elements within the stroma and the plexiform contains cords of epithelial elements within the stroma.14 Radiographically, it is a well- circumscribed slow-growing radiolucency. Microscopically, it is a ‘cystic’ lesion with three significant features as described by Leider et al:Ameloblastomas are classified into four groups; unicystic, solid or multicystic, peripheral and malignant. The unicystic ameloblastoma is essentially a ‘cystic’ lesion with either an intraluminal or an intramural proliferation of the cystic lining.Columnar cells resembling ameloblasts occupy the basal cell layerHyperchromatic nuclei having a vacuolated atypical cytoplasm are polarized from the basal lamina and15 Unicystic ameloblastomas are less aggressive than solid ameloblastomas.14A loose stellate reticulum-like epithelium lining the basilar ameloblasts.16 Microscopically, this lesion can exhibit a variety of patterns. Multicystic ameloblastoma has a poorer prognosis than the cystic lesion.16The multicystic or solid ameloblastoma can grow to an enormous size. It can infiltrate into adjacent structures including intracranial sites, and has the ability to recur and even metastasize. It occurs in a slightly older age group than its unicystic counterpart. The majority of the cases involve the mandible, but the maxilla can also be involved. Radiographically, the appearance is variable with the exception of the desmoplastic variant, but it is generally unilocular or multilocular.17 This type of tumor is rare.The peripheral ameloblastoma is the soft tissue version of the central intraosseous ameloblastoma. It occurs in the alveolar mucosa; however, the underlying bone can be involved due to secondary erosion.1819 Clinically, the majority of patients (75%) present with a chief complaint of a slow growing painless swelling. Signs and symptoms may include; facial deformity, swelling (75%), pain (33%), malocclusion, loosening of teeth, ill-fitting dentures and bridges, ulcerations and periodontal disease.20The malignant ameloblastoma is also a rare entity. Elzay and Corio et al defined this lesion as an ameloblastoma that has metastasized but still maintains its classical microscopic features.21Radiology and location of ameloblastomas are key factors to a correct diagnosis. Plain radiography, panoramic radiographs, conventional tomography (CTs), and magnetic resonance imaging (MRIs) are all used as diagnostic aids. Findings may include expansion of cortical plate with scalloped margins, multiloculations or ‘soap bubble’ appearance and/or root resorption. CTs are used to delineate soft tissue masses, destruction of cortical bone and extension of the tumor into adjacent structures. MRIs, even though not useful for hard tissue examination, are used to provide information regarding edge definition and tumor consistency.22 but local recurrence may occur even in patients who have undergone satisfactory primary surgical treatment.23 As these tumors recur they become more aggressive and can develop into a lesion that is more aggressive than a sarcoma.20 Recurrence seems to depend on several factors, such as (1) method of treatment of the primary lesion, (2) the extent of the lesion and (3) the site of origin.24Ameloblastoma is a locally benign invasive tumor that has a high tendency to recur, metastasize and even undergo malignant transformation. It has a high recurrence rate if not adequately removed2526 Gardner and Pecak stress that the type of treatment required is highly dependent on the type of ameloblastoma present.22Among the various types of ameloblastomas there are differences in recurrent patterns. Multicystic ameloblastoma has a much higher rate of recurrence than unicystic ameloblastoma. The reason for this higher rate is believed to be because of the numerous microextensions the tumor has projecting into the bone.Proper diagnosis and management of ameloblastomas cannot be overemphasized because of the high recurrence rate demonstrated by this lesion. Ominous signs, such as root resorption and tooth mobility should not go unnoticed. Paresthesia which may be prevalent in larger lesion should also raise suspicion as to an aggressive or malignant lesion. It is imperative therefore, that, after clinical and radiographic evaluation of a patient with a periapical lesion, diagnosis be confirmed by obtaining a specimen and sending it for microscopic examination  so that prompt appropriate treatment may be rendered.16An incisional biopsy is advantageous if a representative specimen can be obtained. This will provide the clinician with a definitive diagnosis and allow for an appropriate workup before developing a therapeutic protocol.An incisional or excisional biopsy may be done depending on the size of the lesion and its clinical features.16An exception is a patient with a small, unilocular lesion in which the clinical impression is an odontogenic cyst or fibro-osseous lesion where an excisional biopsy is usually performed.If subsequent microscopic examination confirms an ameloblastoma, the clinician must then decide on additional surgery and take the necessary measures.Surgery is the mainstay of therapy for ameloblastomas today. Treatment ranges from conservative surgery to more radical procedures. Conservative therapy includes radiotherapy, curettage and enucleation.11 However, the incidence of recurrence following radical resection is 5 to 15%.24,25Recurrence rates also vary for the different procedures used to treat the primary lesion. Several authors have found a recurrence rate of 55 to 90% for all ameloblastomas treated conservatively (enucleation and curettage).10 Most investigators believes in resecting at least 1 cm of normal bone beyond the tumor margin.2227 When the tumor has perforated bone, removal of adjacent soft tissue extending to the next adjacent anatomic boundary must be performed to ensure complete tumor-free soft tissue margins.16Radical surgery, as defined by Muller and Slootweg, is a procedure in which the ameloblastoma is removed with a margin of ‘normal bone’.5Preservation of the inferior alveolar nerve is not prudent when it is involved by the tumor, and it should be resected en bloc with the specimen in such cases. This may have been the cause of the recurrence in our case. Immediate nerve reconstruction after extirpation of the tumor restores lower lip sensation for the patient. It is most important to emphasize, to both the clinician and the patient, the need for a definitive treatment protocol and lifetime periodic follow-up for detection of recurrence as even a 5 year tumor- free period does not necessarily mean a cure.28 However, radiation therapy is rarely used as a primary treatment. Gardner believes that radiotherapy should only be used for inoperable cases.20 Other investigators advocate that radiotherapy in conjunction with surgery may have a place in the management of selected patients with recurrence. Pinsolle et al believe that surgery and radiotherapy (50 Gy postoperatively) should be used for (1) mandibular recurrences when the first surgical treatment was adequate, (2) for all recurrences and (3) when soft tissue involvement or positive surgical margins are present after a wide resection.28An ameloblastoma is an epithelial tumor similar to a basal cell carcinoma histologically. Therefore, some investigators contend that their radiosensitivities must also be similar.22 Therefore, it is important to emphasize the need for long-term periodic follow-ups.This case report illustrates the importance of adequate radical resection to avoid recurrence. All efforts must be made to excise the recurrent lesion because death can occur as a result of damage to vital structures by massive local disease and metastasis. Recurrences often present after 15 years or more.