Aneurysmal rupture of a mesodiverticular band has not previously been reported in the clinical literature. We are reporting a case of hemoperitoneum in a 51-year-old male after an aneurysmal rupture of a mesodiverticular band. This case demonstrates that in rare instances, a rupture of the mesodiverticular band leading to Meckel's diverticulum can lead to significant hemoperitoneum. This is usually caused by a traumatic injury but in our case was apparently caused by an aneurysm of the mesodiverticular artery. Patients with known Meckel's diverticula should be aware of the possibility of rupture, as should clinicians treating those with a history of this usually benign congenital abnormality. Rapid surgical intervention is necessary to repair the source of bleeding, as massive blood loss was encountered in this case. Meckel's diverticulum is the most common congenital malformation of the GI tract, occurring in between 0.6% and 4.0% of the population . Most paμL, hemoglobin of 15.7 g/dL, hematocrit of 47.8%, platelet count of 376 K/μL, and a normal coagulation profile. The patient stated that the day prior to the flight, he had a solid bowel movement with no blood or melena noted and was not vomiting at that time; he also denied any sick contacts.A 51-year-old male presented to Jamaica Hospital Medical Center for severe abdominal pain and nausea that began abruptly during a five-hour transcontinental flight. His past medical history included only asthma. Upon arrival at the hospital, he developed recurrent nonbloody, nonbilious emesis. His initial vital signs included a blood pressure of 133/75 mmHg, pulse of 83 BPM, and temperature of 36.3°C. A physical exam revealed rebound tenderness and guarding. His initial labs were as follows: white blood cell count of 15.3 K/After several hours in the Emergency Department, the patient attempted to stand and lost consciousness. An immediate blood pressure was noted to be 53 systolic. Repeat blood work was sent which revealed a decreasing hematocrit (48% to 30%) and hemoglobin (15.7 g/dL to 9.6 g/dL). Fluids were started and an emergent abdominal CT was performed. The CT scan revealed hyper-dense fluid present throughout the abdomen and pelvis, with heterogeneity of the fluid and an active contrast blush . A surgiIn the operating room, Meckel's diverticulum was encountered with a mesodiverticular band that appeared to have torn, resulting in massive intraperitoneal hemorrhage. A small bowel resection was performed to include Meckel's diverticulum, and continuity was reestablished with a side-side stapled enteric anastomosis. Total blood loss for the case was 2000 mL; 3 units of packed red blood cells and 3 units of fresh frozen plasma were administered. The patient was transferred to the SICU postoperatively and subsequently discharged on postoperative day 8.On gross examination, the diverticulum appeared to be completely intact and measured 7.5 cm in length and 4.0 cm in diameter. At the tip of the outpouching there was surrounding fatty tissue Figures  and 3, aDuring normal embryonic development, the left and right vitelline arteries originate from the primitive aorta; the left normally involutes and the right becomes the superior mesenteric artery. In cases where Meckel's diverticulum remains, a branch of this vitelline artery can result in a direct blood supply to the diverticulum through the mesodiverticular band . Cases oThe histology of the actual diverticulum in our case showed simple columnar intestinal mucosa. Meckel's diverticulum may contain various histologies including normal ileal tissue 26%) or have abnormal histology including ectopic tissue (43%), diverticulitis (25%), and enteroliths (6%) % or have. AlthougThis is the first reported case of a mesodiverticular band rupturing due to an aneurysm of the arterial vessel. Three PubMed indexed case reports have been written on a rupture of the mesodiverticular band due to trauma and one instance where it was injured during an operation. As of the writing of this report, no PubMed indexed cases were found that report a spontaneous rupture of the mesodiverticular band. These rare events can rapidly lead to significant hemoperitoneum and in delayed or severe cases potentially result in patient demise. They should be managed with immediate surgical intervention if a diverticulum with hemoperitoneum is suspected on imaging or by patient history.


Pyomyoma, also known as suppurative leiomyoma, is a rare clinical complication that occurs when a leiomyoma undergoes infarction and subsequent infection. A high index of suspicion is required to make the diagnosis and can be guided by a classic triad of symptoms that includes abdominal pain, sepsis without an obvious source, and a history of leiomyoma. In the vast majority of these cases, total abdominal hysterectomy is required to avoid severe morbidity and potential mortality. We present an unusual case of a postpartum pyomyoma that was successfully treated without the need for hysterectomy. With strong clinical suspicion, early diagnosis, and appropriate management, some affected patients may preserve fertility. Leiomyomata are common benign tumours of the uterus with an estimated cumulative incidence of 70% for Caucasian women and greater than 80% for Afro-Caribbean women . They arPyomyoma, or suppurative leiomyoma, is a rare complication of a leiomyoma. It results from infarction and subsequent infection of a leiomyoma. Since 1945, 16 cases of pyomyoma have been reported in the literature, including 7 related to pregnancy . Total aA gravida 3, para 2+0 Caucasian woman booked for antenatal care at 12-week gestational age. At her booking scan, a large intramural fibroid measuring 14 × 12 cm was noted. Fetal anatomical survey was normal at 22 weeks and the fibroid was noted to be above the vertex and not involving the placental site. The remainder of the pregnancy was uncomplicated. She had a spontaneous vaginal delivery at 41 weeks of gestation and was discharged with planned follow-up in gynaecology outpatient clinic 3 months later.At 5 weeks after delivery, the patient presented with abdominal pain and was generally unwell. She was noted to have pyrexia of 39°C. Examination revealed foul-smelling vaginal discharge. Transvaginal ultrasound revealed a 9 cm intramural fibroid. There was no evidence of retained products of conception. Blood cultures were positive for anaerobes for which she received IV antibiotics. After 72 hours, she clinically improved and was discharged on oral antibiotics.At 6 weeks postpartum, she re-presented complaining of the feeling of something coming down. On this occasion, she was afebrile. Examination revealed fleshy tissue protruding from the introitus. CT scan revealed a large collection in the lower segment consistent with pyomyoma. She was brought to theatre for examination under anaesthesia (EUA) and hysteroscopy. The cervical os was found to be open, and a large (27 × 10 cm), exceedingly malodourous mass was manually removed from the uterine cavity see .Her postoperative course was uneventful. She was later discharged with a course of oral antibiotics. Histology of the mass revealed degenerating leiomyomata. At her follow-up visit at 6 weeks postoperatively, she reported normal menses and transvaginal ultrasound scan revealed only a small remnant fibroid.Since 1945, only 16 cases of pyomyoma have been reported, with 7 cases being related to pregnancy . PresentThese cases carry a high rate of morbidity, including sepsis and potential loss of fertility, and possible mortality . A high


But a population based study from Newcastle upon Tyne, UK has found the incidence to be 1 in 27,000 live births [1].Various interdependent clinical and epidemiologic variables such as the age at diagnosis, sex, tumor site, histology which all correlate to different cytogenetic and molecular biologic aberrations; are considered to modify biologic behaviour of teratomas. Size of a sacrococcygeal teratoma  does not predict its biological behaviour. Histological grading of SCT does not seem to correlate with prognosis [2].Antenatal diagnosis is being more commonly made nowadays. Gabra et al found that before 1988, 2 of 18 were diagnosed antenatally compared with 8 of 15 between 1988 and 2001 [1].Fetal ultrasound and MRI are the mainstay of antenatal diagnosis of SCT. Compared with sonography, MRI more accurately characterizes the intrapelvic and abdominal extent of the tumors and provides more information on compression of adjacent organs. Additional anatomic resolution provided by MRI results in more accurate prenatal counseling and improved preoperative planning for surgical resection [1]. Tumor steals blood from fetal circulation, causing the heart to work extra hard and making cardiac failure possible. Cardiac failure manifests as hydrops, Association of fetal hydrops and SCT is usually fatal and always fatal prior to 30 weeks gestation. For the mother, there is the risk of “maternal mirror syndrome” in which the mother’s condition parallels that of the sick fetus.Fetal hydrops (excess fluid retention from high output cardiac failure) is the sole indication for fetal surgery, the only exception being drainage/ decompression of cystic teratoma. If hydrops develops, close maternal–fetal observation is needed. If the condition of the fetus is stable with no high output cardiac failure, then only regular antenatal monitoring is required. Maternal corticosteroids are advisable to accelerate fetal pulmonary maturation. If the condition of the fetus  places him or her in jeopardy, immediate intervention is recommended. If the fetus is mature, there will be an emergency Cesarean section. Termination of pregnancy may have to be considered in cases with bad prognostic signs . In some cases fulfilling the following criteria, fetal surgery to remove the SCT will be recommended. Accurate prenatal diagnosis and well defined natural history to allow the confident diagnosis of a correctable lesion that will otherwise prevent fetal survival; Absence of other life threatening or debilitating anomalies;Ability to perform the procedure without increased risk to the mother's life or her future fertility.The following factors are considered to help outcome in these cases of hydrops fetalis.The increased blood volume represented by tumour circulation may have exceeded the capacity of baby's lungs to arterialise blood; Venous blood from the tumour may have contributed to a low mixed venous PO2 and pulmonary vasoconstriction; excision may have removed the source of desaturated acidaemic blood and relieved pulmonary vasoconstriction; Fetal ascites and abdominal distension may have embarrassed lung development in utero, resulting in reduced lung volumes;Ascites raised the diaphragm after birth and paracentesis thus improved pulmonary function.General anesthesia is administered to provide complete pain relief for mother. Although general anesthesia will alleviate most of the pain of the fetus, additional anesthesia is provided to fetus during the operation. Uterus is partly lifted out of the abdomen. Uterus is opened and SCT is exposed. Tumor is removed and wound is closed. Management at deliveryManagement of SCT depends on fetal gestational age and well being, presence of associated anomalies, tumour vascularity assessed by colour flow Doppler. Preterm labor and premature delivery may occur due to polyhydramnios (excess amniotic fluid) and/or the mass of the tumor. Highly vascular tumours should be delivered by caesarean section to avoid risk of haemorrhage during the second stage of labour. Delivery should be conducted in a tertiary hospital. Size of SCT determines mode of delivery. Non-vascular tumours and those less than 10 cm in diameter may be delivered vaginally. Tumour size has been more than 10 cm in almost all reported cases with difficult deliveries. Teratomas should be well protected after delivery because erosion of the surface may precipitate bleeding before surgical excision is undertaken. Blood loss in large and even benign tumours has been reported to be substantial, sometimes equaling the patient's blood volume. Most reviews report mortality ranging from around 5% to 9% following exsanguinating haemorrhage.Baby is examined for other associated anomalies. Routine investigations are done as part of anesthesia evaluation and surgical fitness. Blood is cross matched. Abdominal ultrasound may reveal abdominal extension of tumor if the external component appears small. Additional investigations like CT scan or MRI will necessitate anesthesia and should be done only if necessary. Alpha feto protein (AFP) should be measured and interpreted carefully as normal newborns also have high levels of AFP. AFP is secreted by fetal liver and yolk sac during intrauterine life. Postnatally, neonatal liver also secretes AFP, but it rapidly falls to normal adult level (10ng/ml) by 8 month of life. In case of SCT, microscopic foci of yolk sac or embryonal carcinoma are the main source of persistently high AFP. It has prognostic significance, and persistent high levels after excision of SCT may indicate recurrence.The technique of wide resection of benign lesions with coccygectomy is helpful in preventing recurrence and has changed little over the last four decades. It was concluded by Schropp et al in 1992 and stands true after 20 more years [2]. Coccyx must be excised in all patients. During surgery, precautions must be taken to prevent hypothermia, which is easily precipitated because of the large surface area and the vascularity of the tumour. Recurrence rate of 37% was reported if the coccyx was not removed completely. Patients with malignant SCT are managed after surgery with irradiation if residual disease is present, and always with combination chemotherapy. Standard surgical management includes complete excision of tumor, without rupture or spillage, without damaging bowel and bladder, with minimum resection of gluteal muscles and without injury to pelvic nerves. A chevron shaped incision is frequently used, but it could depend on the size and location of the tumor. Most tumors have a plane of dissection and can be removed without enblock excision. It is safer to catheterize the bladder to keep it away from the tumor and place a large rubber catheter in rectum for identification. Levator ani muscles are often stretched over tumor and should be reconstructed after tumor is excised. Drainage is necessary as there is a large raw area and collections should be avoided. Patient should be nursed in prone position for a week to prevent fecal soilage. Bladder can be kept catheterized till this time.Wakhlu et al have stated that none of the initial 14 patients with malignant lesions survived beyond 2 years. Of the latter 11 (who received cisplatinum-based chemotherapy), 10 were alive 1 year after surgery; thus highlighting the significant improvement in survival following chemotherapy [3]. Non-fatal hematologic toxicity was common, but deafness and pulmonary and renal toxicities were rare in the UK Children’s Cancer Study Group series [4]. Standard therapy for good-risk patients is four cycles of cisplatin, etoposide plus bleomycin (BEP); cure rates are close to 90%. After chemotherapy and normalization of markers, patients should generally undergo resection of residual masses. Approximately 75% of intermediate-risk and 45% of poor-risk patients group achieve a durable complete response with BEP. Potentially curative options in the salvage setting include ifosfamide plus cisplatin-containing standard dose therapy and high-dose carboplatin plus stem-cell rescue. However, surgery remains an essential component of care [5]. For recurrent tumors, VeIP  is capable of producing durable complete remissions in patients with disseminated germ cell cancer who relapse after cisplatin-etoposide-based induction therapy [6].Amoah M et al concluded in his study of 18 patients from Ghana that most patients do not comply with follow-up appointments [7]. This is the most likely situation in the subcontinent too. Three monthly clinical examinations in the first year are necessary to evaluate and manage urinary and fecal incontinence, if that should develop. Rectal digital examination should be done during every visit to check for local recurrence. If alphafeto protein levels were high, they should be monitored till they reach normal and then measured at 3-6 month intervals for three years. Suspicion of recurrent disease will make further investigations like abdominal USG and CT, CXR or chest CT, tumor markers; necessary.Four of 23 patients with immature teratoma and malignant foci developed recurrence, suggesting that surgical resection followed by close observation are effective treatment [8]. Calaminus et al found that in children with malignant SCT, treated with an intensive, short-interval, platinum-based regimen, the stage, extent of metastases, extension into bone, and AFP level had no prognostic significance [9]. Metastases at diagnosis and completeness of the first attempt of tumor resection were significant prognostic predictors; however, metastases were not predictive for patients treated with up-front chemotherapy [10]. Gobel et al conclude that even locally advanced and metastatic sacrococcygeal GCT can be successfully treated with up-front cisplatinum-based chemotherapy followed by delayed but complete tumor resection.In a retrospective study of 173 patients, Derikx et al have concluded that immature and malignant histology, or incomplete resection are significant risk factors for recurrence. Mature teratoma has the biological capability to become malignant [11].Other complications of SCT are not uncommon. Derikx et al have found that a large proportion of the patients with SCT have problems with defecation, urinary incontinence, or a cosmetically unacceptable scar that affects QoL. Patients who are at higher risk for the development of long-term sequelae cannot be clearly assessed using clinical variables [12].Gabra et al have noted that presentation beyond the newborn period was associated with malignant disease and poorer outcome. They also found that neuropathic bladder or bowel disturbance was identified in 7 of 20 patients on long-term follow-up [13].Rescorla et al in a study spanning 22 years and covering 126 patients found that benign teratomas have a significant recurrence rate mandating close follow-up for more than 3 years; surgical resection alone is adequate therapy for non-metastatic malignant tumors; survival for malignant lesions with metastases is excellent with modern chemotherapy [14]. The type of the teratoma and the surgical approach both contribute to functional sequelae. Tumours with a large presacral component may present with lower extremity weakness or paralysis, constipation, abdominal distension, and urinary tract symptoms secondary to bladder outlet obstruction. Isolated cases of lower extremity weakness or paralysis and bladder dysfunction have been described post-operatively, particularly in association with malignant lesions. Many of the previously reported large series did not report long term faecal and urinary incontinence. Electromanometrically detected rectal and bladder dysfunction in 40% of children following excision of SCT has been reported, though overt incontinence is rare. Tumours with large intrapelvic extensions requiring an abdomino-perineal approach for resection may be associated with a higher incidence (67%) of functional sequelae. It is difficult to determine retrospectively whether dysfunction was caused by the tumour itself or by the surgery. Others have found fecal soiling in more than 20 % patients after SCT resection. No correlation between the severity of anorectal malfunction and the degree of intra-pelvic extension of the tumour has been found. Other health problems, including urinary incontinence, were reported by 13 patients (50%). Hypergonadotropic hypogonadism and sperm abnormalities have been reported in men born with benign SCT. Such patients may have Leydig cell dysfunction, abnormal spermatogenesis, or both. The association of these with SCT may reflect a congenital germ cell defect.Some SCT have a large abdominal component. This tumor may press on bladder, bowel and cause symptoms, compression changes in kidneys and bowel. These may also cause weakness in lower limbs. Rectal examination is done in every patient with SCT to determine deep extension. If it is not possible to reach the upper limit of tumor on rectal examination, these patients should undergo abdominal USG and or CECT to determine extent of abdominal disease. These patients often require abdominal and perineal exploration for complete resection. If possible, abdominal component is performed early, and tumor is completely mobilized. Patient is then turned prone and perineal dissection is done. Tumor can then be delivered from perineal route. Depending upon the extent of tumor and size, the approach may have to be reversed.The American Academy of Pediatrics' surgical section (APPSS) classification helps in grading the extent of SCT, as followsType I—Predominantly external with minimal presacral component.Type II—Present externally but with significant intrapelvic extension.Type III—Apparent externally but predominantly a pelvic mass extending into the abdomen.Type IV—Presacral with no external presentation.SCT are graded histologically as follows Grade 0—Tumour contains only mature tissue.Grade 1—Tumour contains rare foci of immature tissues.Grade 2—Tumour contains moderate quantities of immature tissues.Grade 3—Tumour contains large quantities of immature tissue with or without malignant yolk sac elements.Source of Support: NilConflict of Interest: None


Liver abscess (LA) is an entity with high morbidity and mortality, and therefore require a diagnosis agile and dynamic, allowing appropriate management to avoid complications. The emergency ultrasound (US) allows a versatile and comprehensive management, improving the prognosis of this disease in the majority of cases.we present a case of LA, diagnosed at ED, through the use of US scanning used by Emergency Phisicians.a patient with abdominal pain, with a final diagnosis of a EC assessing US, performed by EP.a 20 year old woman with no history of relevance, came to the emergency room with fever of 1 month duration and abdominal pain that persists after analgesic treatment. On arrival had malaise, hypotensive, febrile, tachycardic to 110 sqm, oriented in all three spheres, with no cardiorespiratory examination findings. Nontender abdomen, without peritonitis. The rest of the examination was irrelevant. Laboratory tests showed leukocytosis and neutrophilia, and abdominal and chest radiography was usual. She underwent a bedside abdominal ultrasound, que injury showed a complex, heterogeneous, poorly defined right hepatic lobe inside anechoic and echogenic materials, 70 x 85 mm, compatible with liver abscess at this level and confirmed by abdominal CT. Developments after starting antibiotic therapy after early crops, and subsequent percutaneous drainage thereof, was satisfactory, with total resolution, remaining asymptomatic.Liver abscesses are rare pathologies, but lethal without prompt diagnosis and treatment, based this on antibiotics and percutaneous drainage after culture. Bedside abdominal ultrasound means applying an extraordinary advance in the diagnosis and evaluation of emergency physicians, being transcendental training of this technique for quality care, comprehensive and dynamic services. Its use and distribution must be paramount, since it represents a cost-effective measure quality front as shown in the case that concerns us.


Trigeminal schwannomas represent between 0.07% and 0.36% of all intracranial tumors and 0.8% to 8% of intracranial schwannomas. Selection of the appropriate management strategy requires an understanding of the tumor’s natural history and treatment outcomes. This report describes the case of a 36-year-old male who presented with a three-month history of progressive headaches, dizziness, loss of balance, decreased sleep, and cognitive decline. Magnetic resonance imaging revealed a large enhancing lesion centered around the left Meckel’s cave and extending into both the middle and the posterior fossa with obstructive hydrocephalus secondary to compression of the fourth ventricle. Resection of the posterior fossa component of the tumor was performed in order to relieve the mass effect upon the brainstem without attempting a radical removal of the middle fossa component and a potential risk of further cognitive impairment. The pathological exam confirmed the diagnosis of a trigeminal schwannoma. The residual tumor showed progressive spontaneous volumetric shrinkage after a subtotal surgical resection. This case shows the value of a planned conservative surgery in complex schwannomas and highlights the challenges in interpreting the treatment responses in these benign tumors, whether approached surgically or with stereotactic radiation techniques. According to the most recent data from the Central Brain Tumor Registry of the United States (CBTRUS) , schwannThe management of trigeminal schwannomas generally includes the following options: observation, microsurgical resection, radiosurgery, fractionated conformal radiotherapy, or a combination approach with surgery and radiotherapy. Selection of the appropriate management strategy is based on a detailed understanding of the natural history and treatment outcomes, as well as on the specific clinical presentation. The presented clinical case provides an example of a challenging clinical situation where all the relevant factors  were considered in order to optimize the treatment.A 36-year-old previously healthy male presented with a three-month history of progressive headaches, dizziness, loss of balance, decreased sleep, and cognitive impairment with psychosocial problems. On physical exam, the patient had normal mental status but had difficulty with tandem gait, truncal ataxia, and mild left sixth nerve palsy with diplopia. There was mildly decreased sensation on the left face in all three branches of the trigeminal nerve with an intact corneal reflex. Informed patient consent was obtained.On an initial computed tomography (CT) scan, a middle and posterior fossa mass was noted with a significant remodeling of the petrous bone Figure .Magnetic resonance imaging (MRI) revealed a contrast-enhancing T1 hypointense and a fluid-attenuated inversion recovery (FLAIR) hyperintense mass in the left middle and posterior fossa Figure .The extra-axial lesion extended into the left cavernous sinus from Meckel’s cave, along the course of the trigeminal nerve. The mass measured approximately 4.3 x 3.1 x 4.5 cm (39.50 cc), and exerted a significant mass effect on the pons and fourth ventricle with subsequent obstructive hydrocephalus. A mild mass effect was also present on the mesial temporal lobe  that had further regressed 69.7% (3.72 cc) at his one-year follow-up Figure .The patient reported transient facial numbness postoperatively but had a steady improvement of cognitive function with a return to baseline function within three months.Given their slow growth, trigeminal schwannomas frequently present with a gradual onset of symptoms with cranial nerve involvement  and headaches . HoweverA microsurgical middle fossa approach for lesions of this nature is often advocated -8. HowevThe exact natural history of schwannomas is unclear, as untreated tumors may grow in some instances, yet may remain dormant for many years and even regress in other cases. Around 6-26% of patients show spontaneous regression of acoustic schwannomas without therapy ; howeverTo the best of our knowledge, this is the first reported case of a trigeminal schwannoma presenting with obstructive hydrocephalus. This case illustrates that a limited surgical approach, focusing on decompression of tumor mass effect and relief of obstructive hydrocephalus, is of value in the treatment of a complicated schwannoma and that some tumor regression may occur postoperatively. As such, favorable outcomes can be associated with careful planning and execution of specific goal-oriented surgical approaches.


Accurate glioma sampling is required for diagnosis and establishing eligibility for relevant clinical trials. MR-based perfusion and spectroscopy sequences supplement conventional MR in noninvasively predicting the areas of highest tumor grade for biopsy. We report the case of a patient with gliomatosis cerebri and multifocal patchy enhancement in whom the combination of advanced and conventional imaging attributes successfully guided a diagnostic biopsy. Brain tumors are imaged with magnetic resonance imaging (MRI), given its exquisite anatomic definition and diagnostic versatility. Many pathologies can be suggested on the basis of features seen on standard T1- and T2-weighted sequences alone, for example, diffuse intrinsic pontine glioma or juvenile pilocytic astrocytoma. Diffusion-weighted imaging demonstrates hypercellular and ischemic areas, with restricted Brownian motion correlating with a higher grade in gliomas [p) have been used as tumor neovascularity surrogates. MR spectroscopy adds five to 10 minutes and noninvasively quantitates brain metabolites. N-acetyl aspartate , choline , creatine (a metabolic surrogate), and lactate/lipid quantification (indicative of necrosis) improve upon standard diffusion and contrast mapping in predicting areas of low versus high (WHO II vs. III) histologic grade in astrocytoma [Supplemental MR perfusion imaging aids in tumor diagnosis, adding three to four minutes to the scanning time. In primary brain tumors, dynamic susceptibility contrast-enhanced relative cerebral blood volume (DSC rCBV) and dynamic contrast-enhanced plasma volume , spectroscopy (MRS), and post-contrast T1 imaging findings are presented in Figure Informed patient consent was obtained for this patient's treatment. No reference to the patient's identity was disclosed in the figure displayed in this paper.This patient’s gliomatosis cerebri is unresectable, and the primary concern is establishing an accurate diagnosis via a minimally-invasive biopsy. Anatomic MR establishes this as an intra-axial tumor, most likely a glioma, given its confinement to and expansion of normal sulcal boundaries and diffuse infiltration of multiple lobes. Extension through multiple vascular territories and discontiguous gray and white matter involvement exclude ischemia and demyelination while viral encephalitis can be excluded clinically. By definition, this constitutes Type I gliomatosis cerebri as it diffusely involves ≥ 3 cerebral lobes . Importap and spectroscopic choline corroborated that area as the most likely to harbor high-grade features.Standard contrast-enhanced T1- and diffusion-weighted sequences are typically adequate for biopsy planning. In this patient, where tissue adequacy for molecular profiling is critical, the only diffusion-restricting and contrast-enhancing area, which was in the right periatrial white matter, was small and in an area where a needle biopsy might return a non-diagnostic sample. However, elevated perfusion V11C-MET, have been shown to be more sensitive and specific in gliomatosis [Additional advanced imaging modalities include CT/MR-fused positron-emission tomography (PET), a noninvasive adjunct examination for brain lesion diagnosis. Lacking the resolution of anatomic or perfusion MRI, PET may be useful in distinguishing radiation necrosis from an active tumor or biopsy planning in multifocal metastatic cases with unknown primary and sufficient spatial separation. While standard FDG-PET may be falsely negative and nonspecific for brain tumors, other amino acid tracers, including omatosis . DiffusiThis patient underwent navigation-assisted open biopsy with awake cortical language mapping to minimize the risk of postoperative dysphasia, given the proximity to Wernicke's area, and local sampling of the posterior left temporal lesion, which revealed anaplastic oligodendroglioma (WHO Grade III). Open biopsy was selected in this case over stereotactic biopsy given the small and deep target volume (< 0.5 cc^3) and the patient's desire for thorough tissue sampling and genetic sequence analysis for potential future clinical trial eligibility. Fluorescence in situ hybridization demonstrated 1p/19q codeletion (favorable), and immunohistochemistry was negative for IDH1 R132H mutation (wild-type is a negative prognostic) -8. On thAdvanced MR-based imaging techniques may aid in glioma surgical planning. In gliomatosis cerebri, which is characterized by infiltration of ≥ 3 brain lobes, a stereotactic biopsy  should be directed at the areas of predicted highest grade for treatment stratification. Lesional enhancement signifies breakdown of the blood-brain barrier, which is seen in tumors, but can also be seen in active demyelination, infection, and radiation necrosis, among other causes. Diffusion restriction is present in regions of tumor hypercellularity as well as infarction or purulence. The degree of Cho elevation and NAA depression on MR spectroscopy as well as plasma volume on DCE MRP, correlate with glioma grade.


Pelvic actinomycosis comprises a rare, subacute to chronic bacterial infection characterised by suppurative and granulomatous inflammation. Diagnosis is difficult as it may simulate pelvic malignancies. Laboratory and radiological findings are non-specific. We reported on 2 cases of pelvic actinomycosis mimicking ovarian malignancy with different management approaches that lead to opposite outcomes. We reviewed the literature on pelvic actinomycosis imitating ovarian cancer with a focus on its surgical management. Despite agreement on the duration of antibiotic therapy following surgical management, consensus regarding surgical approach was rather equivocal. We concluded that pelvic actinomycosis should be strongly suspected in women with presumed ovarian cancer of atypical presentation and a history of intrauterine devices (IUD).The online version of this article (doi:10.1186/2053-6844-1-5) contains supplementary material, which is available to authorized users. Actinomycosis comprises a subacute to chronic bacterial infection caused by filamentous, gram-positive, nonacid-fast, anaerobic bacteria. It is characterised by contiguous, suppurative and granulomatous inflammation. Pelvic actinomycosis although rare, occurs almost only in women. It may simulate pelvic malignancies or retroperitoneal tumours , which oin situ for the last 9 years. Her family history was unremarkable.A 57-year old, mother of two, postmenopausal caucasian woman was referred to our centre following MDT discussion for a 4-month long, persistent, progressively worsening lower abdominal pain. This was associated with mild, offensive vaginal discharge but no vaginal bleeding. She had a significant weight loss (19 kg over 1 month), loss of appetite, dysuria and constipation. Her past medical history included bipolar disorder, hypothyroidism and hypertension. She had a recent psychotic episode following discontinuation of her medication due to lithium toxicity. She had no previous abdominal or pelvic surgeries, abdominal wall trauma or immunosuppression. The patient was previously on HRT for 8 years and had a copper IUD 3/μl).A pelvic US showed bilateral complex, predominantly cystic pelvic masses, inseparable from each other. This was confirmed by CT, which additionally reported a soft anterior abdominal wall mass 60 × 25 × 60 mm situated below the umbilicus involving the right rectus abdominis muscle. A small amount of fluid in the right paracolic gutter was seen in addition to generalised peritoneal disease and sigmoid involvement. The overall picture was suggestive of malignant ovarian disease , raised CRP at 118 mg/l and leucocytosis , pyrexia, night sweats, anorexia and altered bowel habits. Her medical history included asthma. She had no previous abdominal surgery, trauma or immunosuppression. She had a copper IUD 3/μl).Gastro-duodenoscopy revealed mild chronic gastritis and reactive gastropathy whilst colonoscopy ruled out the possibility of inflammatory bowel disease. CT thorax-abdomen-pelvis scan revealed a 75 × 61 mm complex right adnexal mass and a 52 × 41 mm heterogeneous pre-sacral mass. Multiple smaller masses were seen in the left flank, left iliac fossa and anterior pelvis, consistent with metastatic disease  of the left lower limb ruled out deep vein thrombosis and superficial thrombophlebitis. Laboratory investigations demonstrated anaemia (Hb: 7.9 g/dL), raised CRP at 133 mg/l and leucocytosis (WCC 14.3 × 10The case was discussed at the gynaecologic oncology multidisciplinary team (MDT) due to high suspicion of advanced ovarian cancer. Tumour markers included elevated CA125 at 83 U/ml, CA199 at 51 U/ml; AFP and CEA were normal. A US-guided percutaneous biopsy of the left adnexal mass was performed as per MDT decision, which was abandoned as there was no safe path to the lesion and hence diagnostic laparoscopy and biopsies were planned instead.At laparoscopy, there were multiple omental and large bowel adhesions to the anterior and lateral abdominal wall. The right adnexal mass was not visualised as the adjacent small bowel was obscuring the right ovary. A left pelvic side wall biopsy was taken and ascitic fluid was drained. Histology confirmed a benign peritoneal nodule showing fibrosing inflammation in association with actinomyces colonies. The latter were surrounded by acute suppurative exudate within chronic inflammation and prominent foamy macrophages  benzylpenicilline 1.8 mg 4-hourly followed by IV ceftriaxone 2 g daily for 6 weeks. She was then switched to oral amoxicillin 500 mg 3 times daily totalling 6 months. As a result, her weight increased steadily whilst her night sweats and abdominal discomfort subsided. All haematological parameters returned to normal. Abdominopelvic examination prior to discharge was unremarkable. She returned to follow-up appointments at 6 weeks and 6 months and was completely asymptomatic.We searched the MEDLINE and EMBASE databases for articles published between 1988 and 2013 using medical subject heading (MeSH) terms. Key terms included “pelvic actinomycosis” and “ovar* cancer or tumour or carcinoma or neoplasm”. The search was limited to the words “humans and adult female”. Additional publications were identified via cross-referencing from reference lists within the retrieved publications. Only case series published in English language but with no geographical restrictions were included in the literature review.The electronic search initially yielded 36 citations. Eleven reports were published in language other than english. There were two duplicate studies. Five studies were unrelated after screening titles; one study was a review, 3 studies referred to bladder cancer, intrauterine myomas and tubo-ovarian abscesses respectively and one study was on a pediatric patient. In 2 studies, abstracts were not available and they were further excluded. A total of 16 publications were finally included in the literature review Figure . The maiOur institution is a tertiary referral cancer centre; as large numbers of patients are referred with presumed metastatic ovarian or primary peritoneal cancer, it is important to determine the frequency and nature of diseases that mimic malignancy. In this context, pelvic actinomycosis, although difficult to diagnose by virtue of its rarity , should Both patients had a long standing history of copper IUD use. Pelvic actinomycosis accompanied by IUD accounts for about 3% of all actinomycosis . AlthougSeveral case reports of pelvic actinomycosis mimicking ovarian malignancies have been published , 25–27 aAccording to this review, there was no consensus with respect to surgical management of actinomycosis. Agreement came only with the approximate duration of antibiotic treatment. Once diagnosis was made, high-dose and long-term use of penicillin was recommended to eradicate actinomycosis. IV benzylpenicillin was administered daily for up to 6 weeks following surgery. Oral treatment should be continued for a period of at least 6 months due to low penetration in the fibrosis and the tendency to recur .Despite advanced imaging and diagnostics, it is important to suspect actinomycosis in women who present with a presumed ovarian cancer and a history of IUD. This will spare patients from unnecessary, potentially extensive surgery.Written informed consents were obtained from the patients for publication of their case report and accompanying images.


Benign odontogenic lesions are rare entities but are very important due to their locally aggressive nature. Odontogenic myxoma is even rarer in children than in adults. There is no evidence in the literature in regard to the best treatment approach, in terms of conservative or aggressive surgery, for this type of tumor. This paper reports a case of odontogenic myxoma in a child treated with a compromised approach through bone osteotomies and a review of the literature about this disease, especially in pediatric patients. Odontogenic myxoma is a rare tumor that occurs in the mesenchymal tissue of the mandible and maxilla and represents 3–6% of all odontogenic tumors. Despite its benign character, it can be locally aggressive and has a recurrence rate of 25% . It is uMost patients are asymptomatic at diagnosis and typically only present with image findings of the tumor. However, the tumor can also present as a facial deformity with no associated pain or inflammation .The diagnosis consists of clinical findings along with an image study  but can only be confirmed by histologic analysis. Radiography usually shows a mandibular or maxillary tumor that is unilocular with trabeculae and that is sometimes associated with tooth displacement. Tomography shows a radiolucent tumor with trabeculation, and although typical radiography can be enough for diagnosis, CT and MRI are essential to surgical planning and the evaluation of the tumor extension .There is no evidence-based consensus in terms of treatment of the odontogenic myxoma. A literature review demonstrated a large spectrum of surgical treatments varying from conservative enucleation of the tumor to aggressive resections such as mandibulectomy and maxillectomy. This study reports a rare case of odontogenic myxoma in a pediatric patient and discusses diagnosis and treatment options according to a literature review.This is a clinical retrospective study of medical records of one patient who underwent surgical treatment for odontogenic myxoma.Moreover, a review of the medical literature in PubMed and Scopus database was performed, covering articles published in Portuguese or English and using the following keywords (MeSH): “myxoma, odontogenic myxoma”.The medical records of the subjects were used in this review and all the parameters of the surgical procedure and related devices were detailed.The institutional review board approved this study, and all subjects gave written informed consent.A male patient, 2 years and 5 months old, presented at the otorhinolaryngology outpatient clinic with parents complaining of an enlargement and hardening of left maxilla region for 2 weeks . The parA face tomography was completed and revealed a maxillary sinus cyst with odontogenic origin and associated bone erosion . TherefoAccess was obtained through a sublabial incision at anterior left maxilla wall, and a dissection along anatomical planes through the cyst wall covered by a bone capsule was performed . ThereafThe patient was discharged from hospital at the first day after surgery with no complaints and with antibiotics and local care (cleaning of the incision site and ice compresses).The histology showed a lesion with an ill lobular appearance, surrounded by alveolar bone tissue. It is compounded by a cytologically bland spindled and stellated-shaped cells proliferation in an abundant, loose myxoid stroma that contains few collagen fibrils and delicate vessels . An immuTwo months after the surgery, the patient's parents denied complaints, and a new tomography was requested, which showed no evidence of recurrence .According to the World Health Organization (WHO), odontogenic myxoma is an intraosseous benign but infiltrative neoplasm, classified under odontogenic tumors derived from mesenchyme and/or odontogenic ectomesenchyme with or without odontogenic epithelium. Histopathological features include spindle-shaped and round cells in an abundant loose mucoid/myxoid stroma with few collagen fibrils. Capillaries, odontogenic epithelium, mast cells, plasmacytoid, and residual bony trabeculae can also be found . ImmunohDue to its slow growing characteristic, patients typically do not have many symptoms, and an investigation with CT and MRI is essential both for surgical planning and for differential diagnosis with lesions that could contraindicate biopsy . A wide Kleiber et al. presented a very aggressive case of myxoma with both mandible and maxillae commitment in a three-year-old patient who underwent two surgical procedures and previous chemotherapy. After a large resection, this patient did not experience disease recurrence over a 4-year follow-up period . KiresurKawase-Koga et al. presented a literature review of 44 cases with patients  presenting a median age of 31.9 years. Forty-five percent underwent conservative surgery, and, in this group, a 15% recurrence rate was observed. The other patients underwent radical procedures, and, in this group, no recurrence was presented. The follow-up was a median length of 46 months. Within those cases, three cases were children that were 6, 7, or 12 years old; one of those children underwent radical surgery, but none of the children presented with recurrence [Based on histopathologic features a myxoma may be confused with other myxoid neoplasms of the jaw, such as the rare chondromyxoid fibroma or myxoid neurofibroma; the first could have areas of cartilaginous differentiation, whereas myxoid neurofibromas tend to have scattered lesional cells, similar to myxoma, but both of these tumors are positive for S-100 protein, which is not observed on odontogenic myxoma . Also thThere is a large spectrum of differential diagnosis of odontogenic raging from benign to malignant diseases, in both children and adults. Especially concerning children, the diagnosis prior to surgery is essential because a functional and esthetic defect can be more complex, even considering a recurrence rate . ConsequOdontogenic myxoma at pediatric ages is a rare pathology; therefore, few studies have been performed in this field. Differential diagnosis is essential in children, and CT and MRI have important roles in this process.The evidence supports conservative resections in these patients due to the higher morbidity of aggressive surgeries. This paper also presents a middle ground between aggressive and conservative surgery by peripheral osteotomies. There is a need for evidence-based recommendation in terms of treatment of those cases. Especially in infants, long-term follow-up is important due to the recurrent nature of this disease and the long-term life expectancy in these patients.


Although gastric schwannomas usually are nonmalignant, these tumors can undergo malignant transformation. For diagnosis, endoluminal routes are believed to decrease the chance of cancerous cell dissemination. We present a case where a percutaneous route was utilized with supporting evidence for the safe use of this method for diagnosis. Although schwannomas are generally considered to be benign tumors, they can potentially undergo malignant transformation Patient was a 56-year-old white female. She was initially seen in the hospital emergency department for flank pain suggestive of nephrolithiasis. The patient had no abdominal symptoms or complaints except her flank pain. CT scan of her abdomen done at that time confirmed a small renal calculus in the distal portion of her left ureter with moderate hydronephrosis and hydroureter. There was an incidentally discovered homogeneous, solid mass that displaced the stomach posteriorly Fig.. The masSubsequently, abdominal MRI confirmed a large mass, discretely marginated, in the anterior stomach wall and the lesser curvature. The MRI findings were consistent with GS as described by Karabulut et al. Fig. and B 5.The patient's laboratory findings were normal. Although she was asymptomatic from the mass, she was sent for an upper endoscopy and a possible biopsy. Upper endoscopy failed to show any intraluminal pathology. Due to the benign attributes and fibrous appearance of the mass on imaging studies, it was felt a percutaneous needle biopsy would be safe and feasible. Therefore, an US-guided biopsy was suggested. Three biopsies taken from the extrinsic mass revealed a variable spindle-cell lesion composed of plump and wavy cells with tapered ends and inconspicuous cytoplasm, predominantly in short fascicles, with focal areas of nuclear palisading, hyalinized vessels, and admixed chronic inflammation. There was no significant nuclear atypia or mitotic activity, which would suggest malignancy. The immunohistochemical profile showed positive S100 staining and was negative for cKIT, SMA, Desmin, Caldesmon, and CD34 staining. Morphologic and immunophenotypic features of the specimen were identical to that of a peripheral nerve sheath tumor, most consistent with a schwannoma.Based on the findings, resection of the mass was recommended to the patient. At laparotomy, the abdominal mass was adherent to the anterior wall of the mid-stomach. It was pedunculated and easy to manipulate during the procedure. There was no invasion of any other organs and the base was noted to be thin and elongated. A stapling device was used to transect the mass with approximately 1-cm gastric margins. There were a few enlarged lymph nodes in the area and two biopsy specimens from the gastrohepatic ligament lymph nodes were taken for permanent sectioning.Specimen examination revealed an 11.2 × 8.9 × 8.0 cm ovoid mass with an attached 4.2 × 3.8 cm area of tan mucosa with somewhat flattened folds Fig.–C. No muSchwannomas (neurinomas/neurilemmomas) can originate in any nerve that has a Schwann cell sheath. They are generally benign and slow growing and develop more commonly in the stomach. GS are a rare, usually solitary, Gl mesenchymal tumor (0.2% of gastric tumors and 4% of benign gastric neoplasms) When patients suffer from upper GI bleeding, evaluation is usually by endoscopic examination The use of percutaneous biopsy for potentially neoplastic lesions has been controversial. A long-held theory is that there is a possibility of tumor seeding along the biopsy path, as well as a risk of tumor rupture into the abdomen and spreading of tumor cells through peritoneal or mesenteric seeding. Contrary to popular belief, recent evidence suggests this consequence is highly unlikely. In a review by Marco-Domenech et al. Based on current literature, the best treatment option for GC is complete surgical resection . Surgical resection is usually curative with prompt relief of symptoms.Percutaneous biopsy is better tolerated than the endoscopic route, safe and accurate, and can be obtained via a small gauge US-guided percutaneous needle with minimal side effects or long-term sequelae.None declared.


Its use is helpfulin benign diseases, such as caustic or peptic strictures, and malignancies3,especially when the stomach cannot be used, and also in children with congenitalanomalies4. However, this procedure is subject to earlycomplications, as ischemia of the colon and leakage5, or late problems as anastomosis stenosis, ischemic colitis,fistula due to diverticulitis and malignant lesions4.The surgical and anatomical basis for using the colon as a substitute for the esophaguswere established in 1911 by Kelling and Vuillet6 found 10 reported cases of adenocarcinoma in the transposed colonand Bando et al7 also reviewed 10cases in the literature, encompassing adenomas and adenocarcinomas.The transposed colon cancer is a rare complication. Since 2007, six new cases werereported and two reviews published. Hwang et alThe aim is to report an unique case of synchronous adenocarcinoma of the transposedcolon.Woman with 53-years-old diagnosed with congenital esophageal atresia, underwent toseveral surgical procedures in childhood, the latest was a cervical retrosternalesophagocoloplasty at 11 years old. After 42 years she was evolved with cervicaldysphagia, and an initial diagnosis of stenosis of the esophagocolic anastomosis wasperformed, treated with endoscopic dilation without improvement. Later, biopsies wereperformed in the area of stenosis in proximal colonic segment  and polySurgical treatment was performed with neck incision, sternotomy and laparotomy withresection of the colon transposed and a tactic transhiatal esophagectomy of the atresicesophagus in order to pull up the greater curvature gastric conduit obtained by theposterior mediastinum route. Resection of a portion of the left innominate vein whichwas invaded by the tumor was also performed. The pathological examination of surgicalspecimen showed moderately differentiated tubular adenocarcinoma invading pericolicaltissues and the left innominate vein, with no affected lymph nodes - p T4 N0 (0 / 42)M0.The patient developed postoperative superior vena cava syndrome, treated byanticoagulation. She had ischemia of the proximal portion of the stomach transposedbeing performed partial gastrectomy, and respiratory complications. She remained inintensive care and under multidisciplinary clinical support. Discharge of the hospitalwas after 128 days. Patient developed recurrent disease (lung metastases), startedchemotherapy, and died nine months after surgery due to pneumonia.8. For manyyears, the colon was considered the organ of choice, but the stomach has been the mostwidely used in recent decades due facility of preparation of the gastric conduit and itsmore robust vascular supply as a result of a rich submucosal vascular layer9. Resection of the gastric lessercurvature allows elongation and a safe cervical anastomosis11.There are basically three options for replacement after esophageal resection: stomach,colon and small bowel9. Colonic interposition may have earlycomplications as transposed colon ischemia and anastomotic fistula. Late complicationsas anastomotic stricture "redundant graft", ulceration, colitis, perforation,diverticulitis, or tumor in the colonic segment are reported5. Must beremembered that colorectal cancer has a high incidence; is the third leading cause ofcancer diagnosed in men and second among women in the world12 and this colonic segment has a risk for malignancy too.There are 21 cases of adenoma/adenocarcinoma in transposed colon described inliterature7.In cases of previous gastrectomy, gastric caustic or peptic strictures, tumorinvolvement of the stomach or failed gastroplasty the colon is usedThis case shows that all patient underwent to esophagocoloplasty and develops dysphagiaduring late follow-up should be investigated for malignancy and the initial diagnosis ofstenosis of the esophagocolic anastomosis without biopsy should be evoid.


Simple liver cysts occasionally cause pressure symptoms of the abdomen. We herein report an extremely rare case of spontaneous rupture of simple liver cysts. A 65-year-old woman suffered abdominal fullness and dyspnea. Laboratory examinations revealed general inflammation and mild hepatorenal dysfunction. Computed tomography revealed giant polycystic liver and ascites. Echinococcus antibody was not detected. Abdominal paracentesis provided dark brown transparent ascites in which any parasites or tumor cells were not observed. We diagnosed spontaneous rupture of isolated polycystic liver disease (PCLD) and continuously drained the ascites. After the symptoms and laboratory data were improved, resection of liver cysts and left lateral segmentectomy were performed. Histopathologically, simple columnar epithelia inside of cyst walls were observed. The patient remains well without recurrence of the symptoms 10 months after the surgery. We reviewed characteristics of PCLD and considered appropriate treatment for spontaneous rupture of simple liver cysts based on the previous case reports including the present case. Simple liver cysts occasionally cause pressure symptoms of the abdomen and require decompression. We report an extremely rare case of spontaneous rupture of multiple and giant liver cysts, followed by a review of the literature.A 65-year-old woman suffered general fatigue, abdominal fullness, and dyspnea for a month and visited our hospital. The dyspnea had prevented her from sleeping for several days. She had hypertension and underwent laparoscopic cholecystectomy for cholecystitis 20 years earlier. She denied history of trauma. She was 146 cm tall and weighted 97 kg . Her blood pressure, pulse, and oxygen saturation were 96/59 mmHg, 85 beats/min, and 90 %, respectively. Her body temperature was 37.5 °C. Her abdomen was highly distended, and she could not lie supine because she felt harder to breathe. Abdominal tenderness, muscular defense, or Blumberg’s symptom were not observed. Laboratory examinations revealed general inflammation , mild liver dysfunction , and mild renal dysfunction . Anemia was not indicated . Computed tomography revealed giant polycystic liver which occupied the large part of the peritoneal cavity, much ascites in the lower abdomen, and highly elevated right diaphragm  and spontaneous rupture of the cysts. The cause of leukocytosis and high serum CRP values was not apparent, but we denied infectious peritonitis on the ground of physical findings and appearance of the ascites. We continuously drained the ascites whose volume was totally 16,000 ml in 25 days. Her dyspnea, general inflammation, and hepatorenal dysfunction were improved . Her weight and body mass index downed to 82 kg and 38.5, respectively. Despite those remarkable improvements, abdominal fullness remained after the drainage.Twenty-five days after her visit to our hospital, resection of multiple liver cyst walls and left lateral segmentectomy were performed Fig. . We starn = 137) [n = 19) with those of patients with ADPKD (n = 34) [Simple liver cysts sometimes reach large sizes and cause pressure symptoms resulting from mass effect, vascular compression, and biliary obstruction . In the n = 137) . In summ(n = 34) . They co(n = 34) , 10. For(n = 34)  and soma(n = 34) .Spontaneous rupture of simple liver cysts including polycystic liver is extremely rare, and only 14 cases have been reported including this case –24 (TablPCLD is a different disease from ADPKD, tends to have greater and larger cysts and thus cause pressure symptoms more often than ADPKD, and provides better response to surgical reduction than ADPKD. For spontaneous rupture of simple liver cysts, surgical procedures such as cystic wall resection and hepatectomy generally seem to yield favorable outcomes without recurrence of pressure symptoms.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.


Granulosa cell tumors (GCTs) of the testis are rare sex cord-stromal tumors that are present in both juvenile and adult subtypes. While most adult GCTs are benign, those that present with distant metastases manifest a grave prognosis. Treatments for aggressive GCTs are not well established. Options that have been employed in previous cases include retroperitoneal lymph node dissection (RPLND), radiation, chemotherapy, or a combination thereof. We describe the case of a 57-year-old man who presented with a painless left testicular mass and painful gynecomastia. Serum tumor markers  and computed tomography of the chest and abdomen were negative. The patient underwent left radical orchiectomy. Immunohistochemical staining was consistent with a testicular GCT. He underwent a left-template laparoscopic RPLND which revealed 2/19 positive lymph nodes. Final pathological stage was IIA. He remains free of disease 32 months after surgery. Granulosa cell tumors (GCTs) of the testis are sex cord-stromal tumors that are represented by both juvenile and adult subtypes. Juvenile testicular GCTs account for only 1–4% of prepubertal testicular tumors but are the most common testicular stromal tumors in boys < 6 months of age . A literA 57-year-old male presented for evaluation of a painless left testicular mass. He discovered the mass on self-exam. The mass had been slowly increasing in size over the previous three months. He also noted painful bilateral gynecomastia. Review of systems was negative for fevers, chills, night sweats, nausea, vomiting, fatigue, anorexia, and shortness of breath. He noticed no change in libido or erectile function. He was not aware of any palpable lymph nodes. His past medical history otherwise included atrial fibrillation and obstructive sleep apnea. He had no history of abdominal or pelvic surgery. He denied history of cryptorchidism. Family history was notable for breast and lung cancer, but no urologic malignancy.On exam, the patient had a painless nodular mass in his left testis and a left-sided hydrocele. The right testis was unremarkable. His epididymides were normal bilaterally with no masses, tenderness, or induration. He had no palpable inguinal lymph nodes. His abdominal exam and the reminder of his genital exam were normal. Gynecomastia was present, and the breast tissue was tender to palpation.Serum blood counts and chemistries were normal. Alpha fetoprotein (AFP), human chorionic gonadotropin (hCG), and lactate dehydrogenase (LDH) were normal . A scrotal ultrasound showed a 4.5 cm hyperemic left testicular mass infiltrating and essentially replacing the left testicle, as well as a reactive left hydrocele . ComputeThe patient underwent left radical inguinal orchiectomy without complications. The left testis mass was 4.7 cm × 2.8 cm × 2.3 cm on gross examination. Histological examination revealed abundant neoplastic cells with scant cytoplasm and round-to-oval nuclei with longitudinal grooves. They infiltrated through the interstitium as nests, cords, and single cells, with focal rosetting, reminiscent of Call-Exner bodies. The immunohistochemical profile  was consistent with testicular GCT . NeuroenThe patient was counseled regarding the paucity of literature related to the management of GCT. Treatment options including RPLND, chemotherapy, and active surveillance were discussed. The patient elected to undergo left-template laparoscopic RPLND. Twelve interaortocaval lymph nodes and 7 periaortic lymph nodes were removed. Microscopic examination revealed 2/12 interaortocaval nodes with histology consistent with GCT metastases (both <2 cm in size) . The patFour months after his initial presentation, the patient complained of fatigue and decreased muscle mass. He was found to have decreased serum free and total testosterone , increased follicle-stimulating hormone (FSH) (53.6 mIU/mL), and increased luteinizing hormone (LH) (13.3 mIU/mL). Estradiol (18 pg/mL), thyroid-stimulating hormone (2.12 mIU/L), and free thyroxine (T4) (1.3 ng/dL) were normal. These results were consistent with primary hypogonadism. He declined testosterone replacement therapy. In the months following RPLND, he complained of worsening bilateral painful gynecomastia. He underwent bilateral mastectomy. Thirteen months after initial presentation, his free and total testosterone levels had normalized  with persistently elevated FSH and LH, indicative of compensated primary hypogonadism. Blood work drawn at 26 months showed stable levels of testosterone, FSH, and LH. Surveillance CT scans of his chest, abdomen, and pelvis obtained every 6 months following RPLND have been negative for recurrence. His most recent follow-up was 32 months after initial presentation.n = 29, with 6 being malignant) [Approximately 25% of adult testicular GCTs display malignant features, thus necessitating careful clinical and histopathologic investigations to evaluate metastasis and metastatic potential. However, due to the scant number of cases, factors predictive of malignancy have not yet been clearly defined. In 2011, Hanson and Ambaye reviewed all prior cases of adult GCTs (n = 3) progressed quickly and had limited survival (as little as five months following diagnosis). Conversely, patients with metastasis to retroperitoneal lymph nodes only (n = 2) tended to have a longer survival period  [The prognosis for patients with malignant GCTs is variable and not well-defined, again owing to its infrequency. Following a review of the literature, Hammerich et al. found that patients with distant or multiple metastases (diation) . The patTo date, there are no well-established treatment guidelines for the malignant variants of adult testicular GCTs. The rarity of the condition precludes definitive management algorithms. A review of reported cases by Hammerich et al. suggests that various options may be viable and that a combination of treatment modalities  may prove to be advantageous. Chemotherapy regimens have included bleomycin, etoposide, and cisplatin, or doxorubicin and cisplatin. In the absence of metastatic disease on cross-sectional imaging, active surveillance is also a reasonable option. It has previously been suggested, based on experience with sex cord-stromal tumors , that RPLND may be beneficial in stage 1 disease, but less effective in N1 cases . The patThe experience presented herein suggests that primary RPLND with or without neoadjuvant or adjuvant chemotherapy may be a viable option in select patients with testicular GCT. Patients may experience the sequelae of changes in testicular endocrine function and should be monitored based on symptomatology.


Adrenocortical oncocytic neoplasms (oncocytomas) are extremely rare; only approximately 159 cases have been described so far. The majority are nonfunctional and benign. We describe an unusual case of a functional oncocytoma secreting an excess of glucocorticoids (cortisol) and androgens (androstenedione and DHEAS), a pattern of plurihormonal cosecretion previously not reported in men, presenting with endocrine manifestations of Cushing's syndrome. The neoplasm was considered to be of uncertain malignant potential (borderline) according to the Lin-Weiss-Bisceglia criteria.  Adrenocortical oncocytic neoplasms (oncocytomas) are very rare tumours. Since the first description by Kakimoto et al.  in 1986,A 58-year-old man was referred in May 2013 with lumbar persistent pain on the left side. A recent diagnosis of hypertension and type 2 diabetes was established. The examination revealed some features leading to suspicion of Cushing's syndrome, such as central obesity, round face, fine skin, and capillary fragility.The endocrine study confirmed the suspicion of Cushing syndrome and showed cosecretion of increased amounts of DHEAS and androstenedione . The meaT score of −3.2 SD in the vertebral bone and −1.9 SD in the femur. A bone scan revealed pathological deposits in several vertebral bodies with vertebral crushing.An abdominal computed tomography (CT) scan revealed a 9.3 × 7 × 10 cm left adrenal mass. The radiological description was a heterogeneous mass, with intratumoral calcifications, hypodense areas suggesting necrosis, and enhanced imaging after contrast administration. The neoplasm was suspected to correspond to an adrenal carcinoma. Bone densitometry revealed osteoporosis with a A left adrenalectomy was performed. Macroscopically, the mass was adhered to the spleen and the tail of the pancreas and, therefore, considering the clinical and radiological suspicion of malignancy, apart from the adrenalectomy, both the spleen and the tail of the pancreas were excised. The postoperative course was uneventful.At the pathologic examination, macroscopically the tumour measured 15 × 10 × 8 cm and weighed 361.98 g . The micα + in 25% of tumoral cells, and MIB-1 in 10%. The cells were negative for melan A and calretinin.There were both capsular and sinusoidal invasion with extended necrosis and calcifications. Less than 5 mitoses per HPF were present. Neither atypical mitoses nor venous invasion was detected. Immunohistochemistry showed the tumour cells to be positive for synaptophysin, chromogranin A, inhibin-µmol/L. A control of bone densitometry, 15 months after surgery, revealed an improvement of 17.2% at vertebral bone without changes in the femur. The radiological follow-up of the patient included thoraco-abdomino-pelvic computed tomography scan that was performed every 3 months, as well as 18FDG-PET/CT showing only images of what remains of the surgical scars, without evidence of recurrence of Cushing's syndrome, the tumour, or metastasis 30 months after the surgical treatment.After the surgical intervention, the patient showed data of postoperative adrenal insufficiency that required corticoid replacement therapy. There was an improvement of the clinical features of Cushing's syndrome with normalization of the cortisol and the adrenal androgen plasma levels. The values of basal cortisol in plasma fell to 114 nmol/L, those of androstenedione to less than 1 nmol/L, and those of DHEAS to 1.1 This patient presented with an exceptional adrenocortical oncocytoma of uncertain malignant potential (borderline), according to the Lin-Weiss-Bisceglia criteria for the specific assessment of the malignant potential of oncocytic adrenocortical neoplasms , cosecreThe histological findings do not allow us to ascertain the evolution of these borderline adrenal oncocytomas and the low number of cases so far described precludes us from establishing an informed prognosis. In addition, little information exists about the natural history as well as the outcome of these borderline oncocytomas, creating uncertainties for both the patient and the physician . TherefoThe importance of the recognition of this subclass of oncocytomas derives from their uncertain biologic behaviour and the variable form of presentation in that hormone hypersecretion is not as infrequent as previously believed. Mearini et al. , after aIn a recent clinicopathologic study of 13 cases by Wong et al.  it was p


Primary ectopic atypical meningioma involving the renal hilum is rare. This is, to our knowledge, only the second case report of a primary retroperitoneal meningioma and the first case of an atypical subtype in this location.en bloc along with the kidney via laparotomy. According to the World Health Organization criteria, the tumor was histopathologically classified as a meningioma . Five years later, the tumor recurred at the primary site with a similar histopathology. The patient received palliative resection, followed by radiotherapy (4500 cGy in 25 fractions). No relapse was found at 6-month follow-up.A 53-year-old Han Chinese man presented with a 2-year history of left-side flank pain. An oval-shaped retroperitoneal mass was found in the left renal hilum on computed tomography, which was resected We describe the clinical, radiographic and histopathological features of an unusual case of aggressive ectopic meningioma in the renal hilum. The patient presented with a massive retroperitoneal tumor without primary cerebral or secondary metastatic lesions; the preoperative diagnosis was naturally confined to the common retroperitoneal malignancies. This case is of interest to oncologists, because of both its rare location and aggressiveness; it not only enriched the spectrum of primary ectopic meningioma, but also reminded us of potential recurrence of an atypical meningioma. This case raises the issue of the etiology of such a rare tumor that needs further investigation, and more importantly demands long-term follow-up result. Meningioma is a central nervous system (CNS) tumor arising from arachnoidal or meningothelial cells . It is oDespite being a common intracranial tumor, meningioma has been reported to originate from unexpected sites such as bone, scalp, paranasal sinuses, parotid gland, parapharyngeal space, mediastinum, lung, and even the abdomen , 2. EctoA 53-year-old man attended our clinic with a 2-year history of left-side, dull flank pain. The progressive pain was aggravated by being in the recumbent position, without groin-radiating pain. No emaciation, fever, hematuria or CNS symptoms were present. Physical examination revealed a large solid mass with an impalpable border in the left upper quadrant of the abdomen. Abdominal computed tomography (CT) scanning confirmed an irregular mass with soft-tissue density near the left renal hilum, invading the parenchyma. On imaging, the bulky mass had transient heterogeneous enhancement, which was more prominent in the center. There was no lymphadenopathy or metastasis Figure A and B. en bloc without violation of the capsule. Frozen sections showed that the tumor was renal spindle cell carcinoma. The operation was not eventful and the patient was timely discharged.During the operation, a well-defined, oval tumor measuring 10.9 × 8.5 × 17.5 cm was found in the left renal hilum. It infiltrated the renal parenchyma, encased the renal vessels, and significantly displaced the pancreas and splenic vessels. However, there were no enlarged lymph nodes in the retroperitoneum. Radical nephrectomy was performed, and the tumor was resected He was periodically followed up in our clinic. Multiple CT scans of the abdomen were performed, with the most recent being 2 years postoperatively. Five years after surgery, an asymptomatic recurrent lesion was identified attached to the psoas muscle and jejunum in the left renal fossa. The patient was asymptomatic. On CT scan, the tumor was shown to have delayed moderate enhancement, which implies underdeveloped vasculature . As the largest subtype, clear-cell RCC is a heterogeneous regular mass with destruction of the parenchyma and kidney contours, and it shows strong contrast enhancement and rapid attenuation on CT. Papillary and chromophobic RCCs tend to be homogeneous and undergo mild enhancement without frequent necrosis. After a RCC invades the renal pelvis, it is often accompanied by hematuria. Large RCCs often portend nodal disease, extension into vessels, and distant metastasis.Epithelioid angiomyolipoma, an unusual subtype of renal angiomyolipoma, has been recently identified as a potential malignancy. These non-fatty lesions invariably appear as heterogeneous soft-tissue masses on CT; some giant angiomyolipomas even exhibit local invasiveness and lymph node metastasis. Enlarged vessels, hemorrhage, and spontaneous kidney rupture are helpful for diagnosis if identified .Leiomyosarcomas are arguably the most common retroperitoneal sarcomas. These hypervascular malignancies, mainly originating from vessel smooth muscle cells, are more frequently located in the uterine or gastrointestinal tracts. They appear as poorly circumscribed, lobulated masses with iso/hypodensity and delayed circumferential contrast enhancement on CT. They may also have extensive necrosis, cystic degeneration, and hemorrhage. Imaging findings of compression, invasion, or obstruction of the vena cava could be regarded as a characteristic of leiomyosarcoma .Liposarcomas, another group of common sarcomas of the retroperitoneum, are considered relatively easy to recognize. However, the rare pleomorphic liposarcoma may pose a challenge for differentiation because of a non-fatty appearance. Extensive necrosis, hemorrhage and metastasis are inadequate to discriminate them from other soft-tissue malignancies .Considering the non-pathognomonic imaging presentations of this case and unexpectedly overlap in imaging features of many retroperitoneal tumors, pathological analysis through core biopsy is still a determinate diagnostic tool.According to the WHO 2007 standards, meningioma is histologically classified into three types: predominant Grade I, benign tumors; and rare Grade II and III, aggressive tumors. Histological Grade II meningioma, also termed atypical meningioma, is estimated to account for 4.7–7.2% of the entity . It is dAlthough the pathological guidelines are straightforward, the criterion resembles a series of carcinomas and sarcomas that need differentiation. In this case, the tumor should be distinguished from several retroperitoneal masses by means of immunohistochemistry and fluorescence in situ hybridization. The biphasic subtype of synovial sarcoma exhibits epithelioid and mesenchymal differentiation, typically supported by positive staining of EMA, vimentin, and CK. However, the cells grow in nested acinar patterns, and more importantly, the characteristic SYT-SSX fusion gene is diagnostic , 14, whiThe microscopic presentation and clinical features of the tumor in our case were in line with the WHO 2007 Meningioma Classification (Grade II), and it could be distinguished from the differential candidates above. Despite a controversial marker to predict tumor prognosis, the relatively high level of Ki67 did indicate later recurrence in our case . In addiThe only definitive treatment for meningioma is curative resection. However, no successful precedent could be found for treating retroperitoneal meningioma. In the case reported by Huszar et al., the patient relapsed 6 months after surgical resection and survived for only 2 years under chemotherapy . The outTo the best of our knowledge, this is only the second case report of a primary retroperitoneal meningioma and the first case of an atypical subtype in this location. Like the previously reported one, our case presented with invasiveness and gross volume, which warranted regular examination and timely treatment. Considering its rarity and non-pathognomonic presentation, the best method of preoperative diagnosis might be core biopsy. Surgical resection combined with adjuvant radiotherapy may be an alternative treatment, but urgently needs assessment.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 of this journal.


Odontomas, the most often seen ones among odontogenic tumors, are usually asymptomatic and discovered in routine radiographic examinations. Frequently it may interfere with the eruption of teeth. The purpose of this article is to present and discuss the case of a 12-year old child with a complex odontome, obstructing the eruption of left maxillary permanent central incisor. Radio opaque calcified masses were revealed in the radiograph and the masses were surgically removed to facilitate the eruption of the tooth.How to cite this article: Shetty RM, Halawar S, Reddy H, Rath S, Shetty S, Deoghare A. Complex Odontome associated with Maxillary Impacted Permanent Central Incisor: A Case Report. Int J Clin Pediatr Dent 2013;6(1):58-61. Odontomes are generally discovered through routine radiographic examination in dental treatment. An odontome is essentially a benign lesion, but often causes disturbances in the eruption of its associated tooth.56An odontoma is a mixed tissue benign tumor of odontogenic origin, which exhibits complete dental tissue differentiation. Complex odontome constitute about 5 to 30% of all odontogenic tumors of the jaws.This paper discusses a case of an unerupted left maxillary permanent central incisor due to the presence of complex odontome in a young patient with clinical presentation, radiographic features, histopathological features and surgical removal of odontome.A 12-year-old female patient reported with a complaint of missing tooth in the upper front region of the jaw. Past family and medical histories were not relevant. Her general medical history was noncontributory.Intraoral examination revealed a permanent dentition with unerupted left maxillary permanent central incisor. On inspection a swelling was noticed on the labial side of the unerupted tooth . A firm Intraoral periapical and panoramic radiographs revealed that radiopaque structures were present obstructing the eruption of left maxillary permanent central incisor . On basiThe patient was subjected to surgical removal of the odontome under local anesthesia. Full thickness mucoperiosteal flap was reflected from the labial surface of right maxillary permanent lateral incisor to left maxillary permanent canine. The layer of bone overlying the mass was removed and the calcified masses were exposed . The fouOne week postoperative panoramic radiograph revealed the absence of radiopaque masses confirming the successful removal of the odontomes which obstructed the eruption of left maxillary permanent central incisor .7 For tooth impaction, numerous local etiologic factors have been described. These include odontomes, odontogenic tumors, ankylosis, trauma and dentigerous cysts.10 Among these factors, odontoma is the most common etiological factor.In clinical setting, dentists often encounter the problem of tooth impaction, which has been defined as a situation where a tooth fails to erupt into a normal functional position by the expected times.11 It was in 1867 that Paul Broca first used the term ‘odontome'.The term odontome by definition alone refers to a tumor of odontogenic origin. In a broad sense, it means a growth with both the epithelial and mesenchymal components exhibiting complete differentiation with the result that functional ameloblasts and odontoblasts form enamel and dentin. This enamel and dentin were usually laid down in an abnormal pattern because the organization of odontogenic cells failed to reach the normal state of morphodifferentiation.12 odontomes can be divided into three groups:According to WHO classification,Complex odontome: When the calcified dental tissues are simply arranged in an irregular mass bearing no morphologic similarity to rudimentary teeth.Compound odontome: Composed of all odontogenic tissues in an orderly pattern that results in many teeth-like structures, but without morphologic resemblance to normal teeth.Ameloblastic fibro-odontome: Consists of varying amounts of calcified dental tissue and dental papilla-like tissue, the later component resembling an ameloblastic fibroma. The ameloblastic fibro-odontome is considered as an immature precursor of complex odontome.11 It had been suggested by Hitchin, that odontomes are inherited or are due to a mutagene or interference, possibly postnatal, with the genetic control of tooth development.13The exact etiology of odontome is unknown. However, it has been suggested that trauma and infection may lead to the development of such a lesion.14 When these buds are divided into several particles they may develop individually to become numerous, closely positioned malformed teeth or tooth-like structures. When the buds develop without such uncommon division and consists of haphazard conglomerates of dental tissues, they may develop into complex odontomes. However, the transition from one type to another is commonly associated with varying degrees of morphodifferentiation or histo-differentiation or both, and it is often difficult to differentiate between both the types.14The etiology of odontome is that most result from extraneous odontogenic epithelial cells.278 However, underlying impacted teeth are sometimes extracted in association with the removal of odontomas.15The treatment for odontomes in both primary and permanent dentition is their surgical removal. If odontomes are extirpated early without disturbing the underlying tooth germ, the eruption of the impacted teeth can then be expected spontaneously or after orthodontic traction.16 Interestingly, orthodontic therapy is not usually necessary and is not applied to improve the malocclusion caused by odontome after extirpation of the tumor.17 The reason is that most odontomes are very small, and the influence of the tumor on occlusion might be improved without orthodontic therapy. Hisatomi et al18 reported that, the impacted tooth tended to erupt regardless of the degree of root formation after extirpation of the odontoma interfering with tooth eruption, although some teeth showed infraversion and/or crowding. In the case presented, root formation of the impacted incisor was not complete. Therefore, it is anticipated that impacted left maxillary permanent central incisor may erupt spontaneously. However, there have also been some reports about orthodontic therapy which might lead the impacted permanent tooth to a satisfactory postoperative occlusion. In the present case, if the eruption does not occur within 2 to 3 months, orthodontic traction will be done making use of the ligature wire hook on the bonded bracket. If the tooth does not erupt, suspended ligature wire hook will be used for orthodontic traction with no necessity for repeated flap reflection.In this case, the overlying odontomes were surgically removed and the impacted central incisor has been kept under observation to monitor its eruption. If the root of the impacted tooth is still developing, the tooth may erupt normally; but, once the root apex has closed, the tooth has lost its potential to erupt.Radiographic examination of all pediatric patients that present clinical evidence of delayed permanent tooth eruption or temporary tooth displacement, with or without history of previous dental trauma should be performed. Early diagnosis of odontomes allows adoption of a less complex and less expensive treatment and ensures normal eruption pattern of permanent teeth which may otherwise get impacted.


The postoperative histopathological diagnosis was MPNST with deletion of NF1, confirmed by dual-color fluorescence in situ hybridization (FISH) analysis. The tumor recurred 1 month following the surgery. Although adjuvant chemotherapy was administered, the patient succumbed due to intramedullary dissemination and carcinomatous meningitis 8 months following the initial consultation. NF1 deletion by FISH analysis may be particularly useful in distinguishing MPNST from other high-grade malignancies with overlapping morphological features.A malignant peripheral nerve sheath tumor (MPNST) is defined as any malignant tumor that develops or differentiates from cells in the peripheral nerve sheath. This tumor is commonly associated with neurofibromatosis type 1 (NF1) and previous radiotherapy treatment. Primary intraosseous MPNSTs are extremely rare and a case of the lumbar spine in a patient without NF1 is reported in the present study, with a review of the intraosseous MPNST literature. A 45-year-old female presented with a 1-month history of severe lower back pain and pain radiating to the left leg. A total  A malignant peripheral nerve sheath tumor (MPNST) is a rare tumor that accounts for ≤5% of soft-tissue sarcomas, often arising from Schwann cells . ApproxiAn additional case of intraosseous MPNST of the lumbar spine in a patient without NF1 is presented in the current study, and the relevant literature on intraosseous MPNST is reviewed. The study was conducted following a clinical research review by the ethics committee of Toyama University Hospital . Informed consent was obtained from the patient, who was advised that the data from the case would be submitted for publication.A 45-year-old female presented with a 1-month history of severe lower back pain and pain radiating to the left leg. No ‘café au lait’ spots or neurofibromas were present and the patient confirmed there was no family history of NF1. The neurological examination revealed a decreased sensation to a pin prick in the left L4 area. There was no obvious motor weakness of the leg, and bladder and bowel dysfunction was evident. Plain radiographs of the lumbar spine showed an osteolytic lesion at the L4 vertebral body. Computed tomography revealed compression of the spinal canal resulting from destruction of the posterior elements of L4 . Magnetien bloc spondylectomy was performed according to a previous study  was performed for maintenance of spinal stability and prevention of tumor dissemination. The tumor appeared slightly adherent to the dura. The tumor mass was not obviously connected with the bilateral L4 spinal nerve roots or the dura. The boundary between the tumor and the L4 vertebral body was unclear. Histologically, the tumor consisted of coagulation necrosis and sheets of tumor cells with alternating areas of hyper- and hypocellularity. The majority of the tumor cells were atypical spindle cells. The spindle cells exhibited mitotic figures and pleomorphism. Immunohistochemically, the spindle cells were positive for vimentin and smooth muscle actin and negative for S-100, epithelial membrane antigen (EMA) and cluster of differentiation 34 (CD34). The histological diagnosis was undifferentiated pleomorphic sarcoma. Postoperatively, the symptoms of the patient were dramatically relieved. However, they recurred two weeks following the surgery, and the patient presented with progressive hypoesthesia and motor weakness in the legs. A total us study  11). Br. Bren bl2 doxorubicin, 1.5 g/m2 ifosfamide and 300 mg/m2 dacarbazine; 3 days] was administered following the second surgery  and two green [myeloperoxidase (MPO) gene region on 17q22 as the control probe), demonstrating a deletion of the NF1 gene  according to the manufacturer’s instructions. Hybridization signals were assessed in 200 interphase nuclei with strong, well-delineated signals and distinct nuclear borders by two individuals, as previously described . Dual coNF1 gene . These hMPNSTs are rare malignant tumors arising from peripheral nerve sheath cells. These tumors typically present as an enlarging mass originating from a peripheral nerve root of the trunk (~50%), in the extremities (~30%), or the head and neck region ~20%) (0% (2). SDue to a wide morphologic spectrum and lack of specific markers, the diagnosis of intraosseous MPNST is extremely difficult, particularly in a case lacking the manifestations of NF1 and classic histopathological features. High-grade MPNSTs may resemble other malignancies, including fibrosarcoma, synovial sarcoma and malignant fibrous histiocytoma. In the present study, although the initial histological diagnosis was an undifferentiated pleomorphic sarcoma, the histological diagnosis of the recurrent mass was MPNST with positive immunohistochemistry for vimentin and bcl-2, focally positive for EMA, but negative for AE1/AE3, desmin, S-100, CD99 and CD34. Numerous antigens are useful in the identification of nerve sheath differentiation, including S-100 protein, Leu-7 and myelin basic protein. The protein S-100 is the most commonly used antigen for neural differentiation, and it can be identified in ~50% of MPNSTs, although the staining is typically focal and limited to a small number of cells. As encountering an MPNST with a strong and diffuse immunoreactivity for the S-100 protein is uncommon, such a staining pattern always indicates that other benign diagnoses should be reconsidered, particularly cellular schwannoma. The proteins myelin basic protein and Leu-7 have been identified in ~40 and ~50% of MPNSTs, respectively .NF1, has been detected in 25–50% of reported sporadic and NF1-associated cases, often in the form of chromosomal monosomy. Similarly, NF1 deletion in FISH analysis may aid in distinguishing MPNSTs from other high-grade malignancies with overlapping morphological features. Perry et al , while S-100-negative nuclei were observed in all eight MPNSTs. These results indicated the prevalence of NF1 deletion in MPNSTs, regardless of S-100 protein expression (NF1 deletion by FISH was simultaneously detected. Therefore, MPNST in the present case was believed to be a high-grade malignancy from the viewpoint of the S-100 protein-negative cells, which represent dedifferentiated Schwann cells.The molecular pathogenesis of MPNST remains poorly understood. In contrast to numerous other sarcomas, there is no pathognomonic chromosomal translocation and conventional cytogenetic studies typically yield complex, often near-triploid karyotypes without specific aberrations . Howeverry et al  demonstrpression . In the et al (en bloc resection in 128 patients with MPNSTs was 83% and, of these patients, only 48% had negative surgical margins. However, en bloc resection is often extremely complicated in cases of spinal MPNST due to the surrounding spinal cord, dura mater and large blood vessels. In previous studies, four out of the five vertebral MPNSTs underwent subtotal resection, and the outcomes of three cases included two who succumbed to disease and one who remained with disease (Surgical resection is the treatment of choice for MPNSTs. Complete tumor resection with negative margins remains the most effective treatment. A study by Wong et al  reported disease . Adjuvan disease . The eff disease . Howeveren bloc resection of the tumor and adjuvant chemotherapy were performed, the patient succumbed to carcinomatous meningitis. Since the outcome remains poor, further studies on genetic therapy of the tumor based on its molecular pathogenesis are required.In conclusion, the current study presents a case of intraosseous MPNST arising in a lumbar vertebra. Although