Nasopharyngeal soft tissue is most commonly adenoid hypertrophy in children. Although rare, nasopharyngeal carcinoma (NPC) does occur in children. Nasal obstruction,serous otitis media, hearing problems, and tinnitus are common signs and symptoms of allnasal diseases. For this reason, the majority of NPC presents with advanced disease at thetime of the diagnosis. This paper reported 7-year-old boy who was admitted to the hospital foradenoidectomy. He had recurrent adenoidectomy operation due to nasal obstruction. NPChad been diagnosed suspecting the hard mass in the nasopharynx during the operation. Adenoidectomy is the most commonly performed surgical procedure in ENT practiceand NPC is unlikely to be considered in the differential diagnosis. Surgeon should be carefulabout signs and symptoms that alert suspicion. Nasopharyngeal soft tissue is most commonly adenoid hypertrophy in children. However, NPC is rare accounting for 1–3% of all pediatric malignancies and pediatric NPC constitutes 20–50% of all NPC [According to WHO classification, NPC is histopathologically divided into three categories: keratinizing squamous cell carcinoma (WHO type I), nonkeratinizing squamous cell carcinoma (WHO type II), and undifferentiated carcinoma (WHO type III). Most of the cases are diagnosed as undifferentiated nasopharynx carcinoma (WHO type 3) in the advanced stage .Epstein-Barr virus (EBV) infection combined with frequent exposure to environmental carcinogens is suggested in the etiology . Five-yeRadiation therapy is effective in 75–100% of local control of the locoregional disease. Systemic metastasis at diagnosis is between 30–50% and this creates the main difficulty in the treatment.This study presents a case of childhood NPC suspected during adenoidectomy. This is a very common surgical procedure in daily practice and surgeons should keep this diagnosis in their minds. This study also reveals alarming signs and symptoms for diagnosis.Seven-year-old male patient was admitted with complaints of nasal obstruction and snoring. He had a complaint of nosebleeds for the last two days. ENT examination was unremarkable. Flexible nasal endoscopy showed a soft tissue mass compatible with adenoid hypertrophy in the choana. Lateral radiograph of the nasopharynx revealed obstruction in the nasopharynx, so adenoidectomy operation was planned. The patient had previous adenotonsillectomy and adenoidectomy, respectively, at the ages of 2.5 years and 5 years old without any pathological examination. Nasal congestion and obstruction were relieved postoperatively despite recurrence over time. Otoscopic examination was normal without effusion and there was multiple palpable lymphadenopathy less than 2 cm in the neck.Lateral pharyngeal bands were fibrotic and tight during surgery and there was a hard, dense mass in the nasopharynx. As the mass was harder than expected adenoid tissue, it was sent to postoperative pathology. After the immunohistochemical staining in the paraffin blog specimen, pathology has been reported compatible with undifferentiated NPC showing marked pleomorphism and hyperchromatic nuclei with atypical epithelial cells. The specimens were stained pancytokeratin (+), CD 3, and CD 20 (−).Contrasted neck MRI after the surgery revealed 3 × 3 cm axial size, left parapharyngeal space occupying lesion on the left infratemporal fossa adjacent to the medial-inferior border. The patient had bilateral, multiple, 2 × 2 cm maximum diameter lymphadenopathy, prominent in the left jugulodigastric region .In FDG PET, there was a 37 × 30 mm size centrally hypometabolic, peripherally hypermetabolic mass starting from the base of the nasopharynx occupying the left Rosenmuller fossa . The masTumor was extending to soft tissues of oropharynx and nasal fossa with parapharyngeal extension. There was 2 × 2 cm lymphadenopathy in the left upper jugulodigastric region. Tumor was evaluated as T2N1MO and Stage 2B.The patient had 50 Gy radiotherapy and chemotherapy had been planned after radiotherapy. The patient is under the one-year follow-up and had no recurrence.Childhood NPC varies widely according to race and geography. At presentation, NPC associated with hearing problems, serous otitis media, tinnitus, nasal obstruction, anosmia, bleeding, difficulty in swallowing and dysphonia, and even eye symptoms with diplopia and pain. These findings are common signs and symptoms of all diseases of the nose. For this reason NPC usually presents with advanced locoregional disease . The mosThe flexible fibreoptic endoscope examination and biopsy are golden standard to establish the diagnosis. However, it is very difficult to apply to the pediatric age group in routine. Also Kalcioglu et al. reported that microscopic analysis of routine tonsillectomy and/or adenoidectomy specimens may not be necessary unless clinically suspicious .Most of the time, NPC is unlikely to be considered in the differential diagnosis of the adenoid hypertrophy. Rhabdomyosarcoma or lymphoma is the more likely diagnosis in younger children whereas NPC or lymphoma is more common in adolescents.NPC initiates in the fossa of Rosenmuller and spreads intracranially or locally as a mass in the head. In our case, NPC was diagnosed suspecting the hard mass originating from the left Rosenmuller fossa. It can invade petroclival fissure, skull base, pterygopalatine fossa, and parapharyngeal space.Configuration of the mass is always asymmetric in carcinoma, while adenoid hypertrophy is symmetric, diffuse mass without invasion of environmental structures. However, magnetic resonance imaging (MRI) cannot be applied routinely to all adenoidectomy planned patients. MRI can be applied to suspected cases particularly to pathological lymphadenopathy. In our case, MRI performed after nasopharyngeal carcinoma diagnosis .The treatment of nasopharyngeal carcinoma in children is high-dose locoregional radiotherapy to the area as in adults. Several authors advocate the use of systemic adjuvant chemotherapy to reduce distant metastasis and combined modality therapy increases overall survival . HoweverEarly age EBV infection in nasopharyngeal epithelial tissues due to locoregional inflammation may cause NPC development. Transformation of epithelial cells into a malignant disease and premalignant dysplasia may progress rapidly into cancer. Lo et al. showed that EBV DNA was detectable in the plasma samples and can be used for screening .Due to the late signs and symptoms and difficulties in diagnosis and biopsy , screening tests are of utmost importance for early diagnosis .EBV viral capsid antigen and EBV DNA and EBVIgA serology may be used for early identification of individuals at risk for screening programs. EBER hybridization (EBERRISH) is considered the gold standard for detecting and localizing latent EBV in tissue specimens, whether frozen or formalin fixed and paraffin embedded . But it Early stage NPC is difficult to diagnose clinically because of its hidden localization in the nasopharynx. Although rare, nasopharyngeal carcinoma does occur in children and surgeon should be careful about this. Surgeons should always keep in their minds NPC in pediatric adenoid vegetation. The physician should be aware of early warning signs.


In the United States, renal cell carcinoma (RCC) accounts for approximately 3% of adult malignancies and 90–95% of all neoplasms arising from the kidney. According to the National Cancer Institute, 58 240 new cases and 13 040 deaths from renal cancer will occur in 2010. RCC usually occurs in older adults between the ages of 50 and 70 and is rare in young adults and children. We describe a case of a TFE3 translocation-associated RCC in a 19-year-old patient presenting as avascular necrosis of the femur. Due to the rarity of this malignancy, we present this case including a review of the existing literature relative to diagnosis and treatment. In the United States, renal cell carcinoma (RCC) accounts for approximately 3% of adult malignancies and 90–95% of all neoplasms arising from the kidney. The incidence varies depending on racial and ethnic characteristic . AccordiInformation about the natural history is sparse; however the evidence is mounting that patients with metastatic Xp11 translocation RCC have aggressive disease that usually presents at an advanced stage , 26–32. Our patient was a 19-year-old male who had approximately one year of mild-to-moderate low back pain, for which he was being treated by a chiropractor. After development of left hip pain, X-ray examination showed osteopenia of the left femoral head and neck. The diagnosis of Perthes' disease was made and treated accordingly. The patient was placed on nonweight-bearing status of the left hip after a fall. 3 months later he suffered a pathological fracture to the left femur neck . A CT scThe patient had no relevant past medical and surgical history. Family history was noncontributory, specifically no history of malignant diseases. Social history was also unremarkable, particularly no history of tobacco, alcohol, or drug use or exposure to toxins or carcinogens. Physical examination revealed a normally developed 19-year-old male. Tenderness of the left hip region on deep palpation was encountered, as well as tenderness to palpation in the lower back. A palpable firm mass was encountered in the left-sided abdomen. Admission laboratory studies were WBC 7.7 kU/L, HGB 13.1 g/dL, RBC 4.01 M/UL, platelets 241 kU/L, and hematocrit 37.7%. His blood urea nitrogen was 11 mg/dL, creatinine 0.7 mg/dL. Urinalysis was positive for nitrite, blood, and leucocytes. Liver function test was ALT 10 intl units/L, AST 54 intl units/L, LD 327 units/L, total bilirubin 0.4 mg/dL, total protein 6.9 g/dL, albumin 3.9 g/dL. Alkaline phosphatase was 79 intl units/L.Initial differential diagnosis included possible metastatic renal cell cancer, Wilm's tumor, or lymphoma. CT scan of the abdomen and pelvis showed a large heterogeneously enhancing exophytic left renal mass measuring 11.6 × 10.7 cm, infiltrating the renal pelvis and lower pole calyceal system. The mass had multiple small calcifications. The mass extended to the left lateral and posterior abdominal wall without evidence of infiltration or solid organ involvement and without significant lymphadenopathy. The findings could be confirmed by MRI . CT scanThere was an osteolytic lesion involving the L4 vertebral body anteriorly with prevertebral soft tissue component and osteolytic lesions involving the left proximal femur and left acetabulum.The bone scan demonstrated a very mild salt-and-pepper appearance to the skull, sternum, and posterior ribs. The axial skeleton showed increased radionuclide activity throughout the entire diaphysis of the left femur as well as the lower lumbar spine and midsternum. There was also increased radionuclide activity at the posterior left hemipelvis, suggestive of metastatic disease to the axial skeleton and the lower appendicular skeleton .Percutaneous biopsies of the tumor showed a mixture of spindle cell (predominant) and epithelioid cells with abundant amphophilic, clear, and focally granular pink cytoplasm. Epithelioid cells often line spaces, spindle cells form fascicles. Necrosis is not present . The selThe patient was discussed at a multidisciplinary tumor board meeting, where it was agreed that a radical tumor nephrectomy with the intention of tumor debulking was indicated. The patient was also scheduled for curettage and bone grafting of the metastatic femur lesion and left periacetabular region and calcar-replacing left total hip arthroplasty. On hospital days 5 and 10 the patient underwent left radical nephrectomy via chevron incision and orthopedic surgery. Intraoperatively the retroperitoneum demonstrated extensive number of dilated blood vessels and dramatically dilated lymphatics, measuring up to 6–8 mm in diameter coursing over the surface of the kidney, Gerota's fascia, and the mesentery to the left colon. These dilated lymphatics extended over the aorta and up to the renal hilum. Both surgeries went uneventful. Pathological examination showed an intact kidney without perinephric fat measuring 18 × 12 × 7 cm and weight of 920 g. Approximately 90% of the kidney had been replaced by the tumor, except for a rim of upper pole that measured 6 × 3 × 2 cm. The tumor was unifocal measuring 12 cm greatest dimension. The tumor was a solid, tan-pink mass with hemorrhage and necrosis, limited to the kidney, well circumscribed, and grossly confined by the renal capsule. The uninvolved renal parenchyma was unremarkable . Microscopically, the renal tumor was composed of relatively large and uniform epithelioid cells with clear to vacuolated to eosinophilic cytoplasms. The tumor showed a nesting or trabecular growth patterns. The nuclei show intermediate grade features but overall have a bland appearance. Mitotic activity was sparse. (bone) .The immunostainings were negative for melan-A and the melanoma cocktail. This tumor was also nonreactive for EMA, CD10, pancytokeratin, CK7, CD68, CD34 and E-cadherin Mart 1, HMB45, and CKAE1/AE3. Vimentin highlighted an occasional tumor cell. PAS with and without diastase and colloidal iron stains was equivocal . After rAREN0321Treatment Regimen for High Risk Renal Tumors was initiated. The treatment regimen of a 4-week cycle included Cyclophosphamide: IV over 1 hour 1200 mg/m2/day, week 1; Doxorubicin: IV over 120 minutes on Day 1 dose 45 mg/m2/day; Vincristine: IV Day 1 wks 1–3 = 1.5 mg/m2/day; Carboplatin: IV over 1 hour on Day 1 of week 4; cyclophosphamide: IV over 15–30 minutes on Days 1–4 of week 4 dose 440 mg/m2/day. Additionally, the patient received palliative radiation therapy with a total dose of 3500 cGy in 15 fractions using the 3D conformal technique with the 15 MV unit to the pelvis/left hip and to the C-spine and left shoulder with a total dose of 3150 cGy given in 8 fractions also using the 3D conformal technique with the 6 MV unit. The patient developed disease progression under this chemotherapy/radiation regimen and was started on Vincristine: IV Day 1 of weeks 1-2 dose 1.5 mg/m2/dose and Irinotecan: IV 20 mg/m2/daily X 5 according to the COG protocol for unresponsive renal masses. No significant response was noted. It was decided to start the patient on a combination regimen consisting of Sorafenib plus Irinotecan. This combination resulted in stable disease for approximately 8 weeks. However, this combination caused significant diarrhea, and Sorafenib was stopped. Finally, the patient was treated with a combination of Irinotecan, Temsirolimus, and Bevacizumab. Despite a negative PET scan the patient developed clinically progressive disease under treatment and died secondary to multisystem failure. Based on the histopathological characteristics of the tumor (clear cell carcinoma with sarcomatoid features) and its metastatic spread, chemotherapy according to the n = 6) or complete (n = 1) response [Solid tumors of the kidney are the third most common malignancies in children and young adults. While Wilms tumor being the most frequent and well-studied entity, RCC is rare and is responsible for only 0.3% of all malignancies and up to 6.3% of all malignant renal tumors in this age group . Becauseresponse .TFE3 gene fusion-related renal tumors described in the literature, the exact biologic behavior and impact of current treatment modalities remain to be uncertain. Increased awareness among urologists, pathologists, and oncologist is necessary in order to help in identifying more cases of this phenotype in the future. Prospective randomized studies on novel targeted agents are needed to identify the optimal treatment strategy for this specific patient population. In addition, a better characterization of the genetics of these translocations will help to develop more specific drugs.In summary, TFE3 RCC is a rare malignancy where the genetic background may not only contribute to tumorigenesis, but also determine the response to chemotherapy and targeted therapy. Therefore it is necessary to diagnose this tumor entity accurately. Histomorphological and immunohistochemical features were previously described by Argani et al., which may help pathologists in distinguishing these neoplasms from their mimics , 39. Bec


Pedunculated hepatocellular carcinoma (P-HCC) has rarely been reported and is characteristically large and encapsulated. Only sporadic cases have been published, in which P-HCC was combined with other liver tumors (mostly benign), making the diagnosis difficult.We report a patient who was admitted to our hospital with clinical features of intestinal obstruction and a palpable mass in the right iliac fossa. Ultrasound, computed tomography and magnetic resonance imaging demonstrated an encapsulated mass of unclear origin and characteristics of liver hemangioma. Laboratory tests revealed elevated α-fetoprotein (> 800 ng/ml) and cancer antigen 125 (> 51.2 U/ml). With a possible diagnosis of giant liver hemangioma, we proceeded to surgery. During surgery, a giant pedunculated tumor was discovered on the inferior surface of the right lobe of the liver, hanging free in the right abdominal cavity towards the right iliac fossa. The macroscopic appearance of the tumor was compatible with liver hemangioma. Tumor resection was performed at a safe distance, including the pedicle. The rest of the liver appeared normal. Histopathological examination revealed grade II and III HCC (according to Edmondson-Steiner's classification) with nodular configuration, central necrosis, and infiltration of the capsule. Underneath the tumor capsule, residual tissue of a cavernous hemangioma was recognized. The resection margins were free of neoplastic tissue.This rare presentation of a giant P-HCC combined with a hemangioma with features of intestinal obstruction confirmed the diagnostic difficulties of similar cases, and required prompt surgical treatment. Therefore, patients benefit from surgical resection because both the capsule and the pedicle prevent vascular invasion, therefore improving prognosis. Pedunculated hepatocellular carcinoma (P-HCC) is a rare form of cancer, which protrudes from the liver as a massive tumor with or without a pedicle. The exophytic growth of the tumor lying beyond the confines of the liver occasionally poses a considerable diagnostic challenge. This is due to the uncertainty of the origin of the tumor and that it mimics other abdominal tumors. An incidence of P-HCC of 0.24-3.0% has been reported in Japan .Pedunculated HCC variants are classified as nodular, diffuse and massive ,3. The m1- and T2- weighted images compared with the surrounding lower-signal-intensity liver tissue. No invasion into the liver or adjacent organs was demonstrated  and cancer antigen 125 (51.2 U/ml). HBV and HCV serum blood tests investigated in the past were negative. Abdominal X-ray showed opacity in the right upper abdomen, displacing the small bowel loops, and a few radiological signs of intestinal obstruction. The patient had clinically abdominal distention and features of intestinal obstruction on physical examination therefore, ultrasound was not performed. She also reported a history of liver hemangioma that was diagnosed 10 years previously. Following computed tomography (CT) and magnetic resonance imaging (MRI), without administration of intravenous contrast agent, a large (14 × 12.5 cm) well-defined, heterogeneous mass of unclear origin and nature was detected between the right hepatic lobe and the colon. The mass appeared hyper-dense on TWith a possible diagnosis of giant liver hemangioma causing acute abdominal discomfort and intestinal obstruction, surgery followed. During the operation, an enormous pedunculated tumor with exophytic growth and a thin pedicle attached only to the inferior surface of the right lobe of the liver was found, hanging freely in the right abdominal cavity Figure .The remainder of the liver was of normal color and consistency. Grossly, the tumor was fairly well-circumscribed and encapsulated, invading veins in the periphery. This gave the impression of hemangioma Figure . No enlaFollow-up of the patient has been short, but 5 months after surgery there was no evidence of recurrent disease.HCC occasionally grows outside the liver in a pedunculated form, hence making preoperative diagnosis difficult ,2. To diIn the present case, the patient was aware of a liver mass of unknown nature, which was asymptomatic for over 10 years. Due to idiopathic hemochromatosis, the patient had regular follow-up investigations with ultrasound and CT, suggesting an exophytic mass arising from the inferior surface of the right lobe of the liver, and with imaging features characteristic of hemangioma. It is reported that Idiopathic hemocromatosis is associated with an increased risk for hepatocellular carcinoma ,14. The This patient unfortunately experienced a severe allergic reaction to radio-opaque medium during CT examination in the past and all CT and MRI studies were performed without administration of contrast medium. Therefore, typical enhancement patterns of the exophytic HCC could not be identified. It is well known that, most hepatic hemangiomas identified on CT or MRI can be diagnosed accurately from the characteristic imaging features of these lesions. On T1 weighted (T1W) images are hypointense to surrounding hepatic parenchyma with smooth, well-defined, often lobulated margins. On T2 weighted images (T2 W) they become significantly hyperintense compared to normal liver. In this case of a giant pedunculated HCC with hemangioma the MRI was not characteristic. According to Bader TR et al. , the exo1-and T2- weighted images, with the central portion of the tumor showing a high signal intensity area compatible with central necrosis. Although the imaging studies could not draw a definitive diagnosis of the nature of the liver tumor, we proceeded to emergency surgery based on the patient's clinical picture and the findings of a palpable abdominal mass. The exophytic growth of P-HCC usually tends to form a mass that expands into the surrounding organs, rather than infiltrating them.The patient's condition had shown a gradual deterioration in the previous 6 months, accompanied by general malaise, anorexia, severe weight loss, and persistent right hypochondrial pain. At admission, MRI examination showed an increased size of the pedunculated liver mass that appeared hyper-dense on TThe histological findings of this patient were very unusual, since P-HCCs are rare liver tumors and to our best knowledge there have been no reports in literature describing P-HCC combination to other tumors. The largest series dealing with the surgical results of pedunculated HCCs is reported by Yeh CN et al. , where nAccording to their macroscopic appearance, P-HCCs are classified into pedunculated type with a pedicle  and pedunculated type without a pedicle and attached to the liver surface (sub-type II) . Horie eIn cases of pedunculated hepatic tumors, correct diagnosis is difficult to obtain. Nevertheless, patients benefit from surgical resection, especially in cases with large P-HCC without vascular invasion.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interestsTK and DD performed the surgery and were involved in drafting the manuscript; AS, DP and SK organized the patient's data and figures and helped to draft the manuscript; KE carried out the histopathology examination; GK corrected and contributed to the final version of the manuscript; TK wrote the manuscript. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-230X/11/99/prepub


Encephalocele is a rare congenital abnormality of the central nervous system, where brain tissue protrudes from a defect in the skull. Some anomalies are associated with encephalocele. However, the association of spinal teratoma and encephalocele has not been reported in the English literature.We report the case of an Iranian girl with a history of encephalocele surgery, who, at the age of four years, developed an intramedullary spinal teratoma, and discuss the pathogenesis of this association.To the best of our knowledge, this is the first report of an association between encephalocele and spinal teratoma. Encephalocele refers to a group of rare congenital anomalies of the central nervous system (CNS), where brain tissue protrudes from a defect in the skull . Its preTeratomas are tumors derived from all three germ layers . In chilWe report a case of encephalocele and lumbar intramedullary teratoma and discuss the possible etiology.A four-year old Iranian girl was referred to the neurosurgical department with severe back pain and motor regression. She was the second child of nonconsanguineous parents, and was delivered by elective Cesarean section due to being repeat. She had a history of occipital encephalocele, which was treated surgically during the neonatal period and she later received a shunt to treat progressive hydrocephalus. She could sit at nine month of age and stand at two years, but was unable to walk. Six months before her referral, she had developed back pain, which was particularly severe at night, and after three months, she was unable to stand.On physical examination, our patient was found to be generally normal, with good mental performance, and normal results from a neurological examination of the arms. She had a head circumference on the 75th percentile and a functional ventriculoperitoneal shunt. She could move her legs, but was unable to keep them up against gravity. Her deep tendon reflexes in the legs were exaggerated, and her sensory level was undetectable. She had urinary and fecal incontinence.Spinal MRI revealed an intradural mass Figure  extendinThe child underwent an osteoplastic laminotomy extending from T11 to L2. The dura matter was severely tense at the level where the laminotomy was opened. There was a white to creamy mass that was extramedullary at the distal level but intramedullary at the L1 and T12 levels. There was no real capsule around the mass, which contained small fine hairs and creamy fatty material. There was a small lipoma on the dorsal surface of the mass at the level of the L1 spine body.The lesion was excised completely. Histopathologic examination of the mass revealed a variety of tissues including skin, fat, connective and adipose tissue, and vascular structures Figure . A pathoOur patient's post-operative period was unremarkable. One year after the operation she was able to stand by herself and to walk with the aid of a brace and walker. She was continent during the day but had nocturia.Encephalocele is a cystic congenital malformation in which the cranial contents herniate through a defect in the cranium. Although encephalocele is typically classified as a neural tube defect, its underlying mechanism may differ from that of myelomeningocele, and probably occurs after neural tube closure . Encephaet al. suggested that a dysembryogenic process forms the basis of development of teratoma, especially those arising from spinal dysraphism. They proposed that the combination of mutated genes important for normal early neural development and cellular differentiation, and/or absent or deficient inductive signals, can lead to the formation of teratoma [Teratomas are tumors composed of derivatives of all three germ cell layers, and can be classified into mature and immature types based on the degree of differentiation . The oveteratoma . MoreoveThis case emphasizes that, when dealing with a patient with a congenital anomaly who presents with new signs and symptoms or loss of developmental abilities that had already been acquired, it is essential to investigate if the new symptoms are due to causes other than the already existing anomaly, as in our patient. It is possible that another anomaly may be causing the symptoms, and the necessary investigations should be performed.In any patient with a congenital central nervous system anomaly who presents with new neurologic problems, the possibility of another anomaly, especially those that are believed to arise from the same pathogenic pathway, should be considered.The exact pathogenic pathway of association between encephalocele and spinal teratomas remains to be elucidated. Although the possibility of two different pathogeneses could not be ruled out in our patient, it is more likely that the same genetic and molecular defects are responsible for this spectrum of findings. Further studies are needed to elucidate the probable genetic and molecular defects underlying these conditions.The authors declare that they have no competing interests.Written informed consent was obtained from the parents of the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.FR and FN made major contributions in patient care, literature review and drafting of the manuscript. MEK made a substantial contribution to the literature review, correction and final approval of the manuscript. FM made the pathological exam and description. All authors read and approved the final manuscript.


Benign esophageal tumors are uncommon, leiomyomas being the most frequent. However, esophageal schwannomas are exceedingly rare. We report here on two instances of large esophageal schwannomas treated by enucleation. A 63-year-old male and a 32-year-old female were referred to us for abnormal chest X-rays. Computed tomography of the chest documented sizeable growths in the upper thoracic esophagus, resulting in compression of membranous trachea posteriorly. By positron emission tomography, the tumors appeared hypermetabolic. In both instances, successful surgical enucleation was achieved. Histologic examination confirmed spindle cell tumors positive for S-100 protein by immunostaining. Benign esophageal tumors are uncommon and only 1% are detected clinically. Schwannomas are neurogenic tumors typically originating in the mediastinum. Esophageal schwannomas seldom occur and are difficult to diagnose by imaging. The sole treatment for such benign lesions is enucleation, which proved successful in the two patients reported here, each of whom presented with large esophageal schwannomas.A 63-year-old male was referred to our care for mediastinal widening by chest X-ray. On routine physical examination, no abnormalities were found and laboratory tests were unremarkable. Endoscopy revealed only superficial gastritis. Computed tomography (CT) of the chest disclosed a well-defined mass of 9.4 × 8.9 cm in the upper thoracic esophagus, with heterogeneous density after injection of contrast. No necrosis or hemorrhage was evident. This tumor was obstructive, causing proximal esophageal dilatation and tracheal compression Figure , withoutRight lateral thoracotomy was performed via the fourth intercostal space. Pleural effusion and adhesions were absent. The 10 × 10 cm mass protruded into the retrotracheal space, assuming a dumbbell shape. It was firm, well-encapsulated, and hypervascular. A 3-cm vertical esophageal myotomy was performed at the tumor level followed by tumor enucleation, but the surgical plane skirting a mass of this size was difficult to define. During extensive mucosal dissection, a mucosal tear (1 × 1 cm) was sustained, requiring primary repair. The muscle layer was approximated with non-absorbable sutures and regional lymph nodes were also removed. There was no malignancy including the mass and lymph nodes on the frozen section.The resected tumor formed two distinct lobes measuring 9.5 × 7.0 × 6.5 cm and 8.8 × 5.0 × 5.5 cm, respectively Figure ; the oveA 32-year-old female was admitted for mediastinal widening by chest X-ray. She had given birth 2 weeks previously and now complained of intermittent chest pain. Endoscopic ultrasonography and esophagography revealed a large, obstructive submucosal tumor of the upper thoracic esophagus (20–29 cm from incisor) without invasion. Endoscopic biopsy showed only inflammation, but a 6.0 × 8.5 × 4.0 cm homogenous mass of the upper thoracic esophagus was seen on chest CT, compressing the trachea (Figure After general anesthesia, ventilation was difficult due to airway distortion. The tumor may have originated from the left esophageal wall, since radiolucency was visible at the right esophageal lumen. Submucosal tumor enucleation was attempted via the left cervical incision Figure . We idenThe ovoid submucosal mass measured 8.7 × 5.9 × 2.4 cm. Its cut surface was yellowish and rubbery. Microscopically, an innocuous proliferation of spindle cells was observed, devoid of mitotic figures and positive for S-100 protein by immunostain Figure . The patSchwannomas are the most common type of neurogenic tumor, derived from proliferating Schwann cells. Typically, the mediastinum is the site of origin. The majority of esophageal submucosal tumors are leiomyomas. Esophageal schwannomas are in fact exceedingly rare; approximately 30 cases have been reported in the literature . The difSchwannomas are usually benign, displaying one or two histologic patterns: Antoni A and B. Antoni A areas are compact zones with palisading of spindle cells, whereas loosely arranged tissue with variable cystic change and hemorrhage is designated Antoni B. Schwannomas tumors show positive immunostaining of S-100 protein and are negative for smooth muscle markers. Malignant transformation, albeit rare , is indiTumor enucleation, as opposed to complete curative resection, is generally sufficient for benign schwannomas . Due to Large esophageal schwannomas were successfully treated by enucleation. Despite the short-term encouraging outcomes, extended follow-up is mandatory due to the rarity of these neoplasms.Written informed consent was obtained from the patients for publication of this case report and images.CT: Computed tomography; PET: Positron emission tomography; SUVmax: Maximum standardized uptake value; VATS: Video-assisted thoracic surgery.The authors declared that they have no competing interests.HWJ: 1st. author, review of medical records, writing. KSK: review of medical record. KYH: revision. JKP: corresponding. All authors read and approved the final manuscript.


Congenital high airway obstruction syndrome is a rare but life-threatening condition. Therefore, prenatal diagnosis is important. The obstruction can be due to laryngeal/tracheal atresia or external compression. While a differential diagnosis with congenital cystic adenomatoid malformation (CCAM) type III may be difficult, it is still possible with ultrasonography. In this study, we report a case of bilateral echogenic lungs with hydrops fetalis. After the prenatal diagnosis of laryngeal atresia, the couple opted to have an elective termination of pregnancy performed at 20 weeks of gestation. The diagnosis was confirmed by a complete pathological examination. Congenital atresia of the larynx is a rare abnormality of upper airway obstruction. For such a condition, prenatal diagnosis is difficult. Nevertheless, the diagnosis is crucial because affected fetuses face a high risk of demise. Sonographic findings showed increased lung echogenicity and size coexisting with fetal ascites. However, the findings may not always be typical. We reported a 20-week-old male fetus with congenital laryngeal atresia diagnosed prenatally by the findings of fetal hydrops, hyperechogenic lungs and other abnormalities without the fluid-filled trachea. After the elective termination of pregnancy, the diagnosis was confirmed by a complete pathological examination. A 29-years-old, gravida 1, woman was referred to our perinatal unit at 20 weeks of gestation on suspicion of fetal anomaly. Her family history was noncontributory. Physical examination revealed a 20-week-sized uterus with audible fetal heart sound. Transabdominal ultrasound showed oligohydramnios and fetal hydrops with marked ascites, generalized skin edema and placentomegaly. The fetal chest circumference was larger than 95th percentile of 20 weeks gestation. Both lungs became severely enlarged and highly hyperechoic . InversiThe aborted fetus weighed 450 g and had a distended thorax and abdomen and deformation both ear lobules. Postmortem examination revealed generalized edema and markedly distended abdomen compatible with hydrops fetalis . The abdCongenital high airway obstruction syndrome (CHAOS) is a rare and usually lethal abnormality. Three possible presentations include (1) complete laryngeal atresia without an esophageal fistula, (2) complete laryngeal atresia with a trachea-esophageal fistula, and 3) near-complete high upper airway obstruction  near-com. The sonLaryngeal atresia may be associated with other structural and genetic abnormalities , such asAlthough laryngeal atresia is usually lethal, it has recently become possible to bypass the airway obstruction and establish adequate ventilation by the EXIT procedure (Ex Utero Intrapartum Treatment) or fetoscopic laser decompression, while the fetus is still connected to the placenta. Several successful cases have been reported , 13–18. In this case, we observed enlarged bilateral echogenic lung masses, inversion of the diaphragm with nonimmune hydrops fetalis. Oligohydramnios can be present in the context of hydrops due to the impairment of renal function. The findings were difficult to differential with congenital cystic adenomatoid malformation (CCAM) type III. However, bilateral CCAM was very rare; therefore, the diagnosis of CHAOS was considered. Moreover, our case had distinct abnormalities, including deformed ears, a single lobe in the left lung, and intestinal malrotation. Lastly, our case did not have an enlarged fluid-filled trachea, a unique sonographic feature, although the laryngeal stenosis without fistula was confirmed by the pathological examination.


Midgut carcinoids are neuroendocrine tumors that commonly metastasize to the intestinal mesentery, where they predispose to intestinal obstruction, ischemia and/or congestion. Because of their location, many mesenteric carcinoid tumors are deemed unresectable due to the risk of uncontrollable bleeding and prolonged intestinal ischemia.We report the case of a 60-year-old male with a mesenteric carcinoid tumor obstructing his superior mesenteric vein, resulting in intestinal varices and severe recurrent GI bleeds. While his tumor was thought to be unresectable by conventional techniques, it was successfully resected using intestinal autotransplantation to safely gain access to the tumor. This case is the first described application of this technique to carcinoid tumors.Intestinal autotransplantation can be utilized to safely resect mesenteric carcinoid tumors from patients who were not previously thought to be surgical candidates. We review the literature concerning both carcinoid metastases to the intestinal mesentery and the use of intestinal autotransplantation to treat lesions involving the mesenteric root. Carcinoid tumors are the most common neuroendocrine neoplasm, typically arising in the respiratory and midgut gastrointestinal tracts ,2. WhileA 60-year-old man presented to our unit in January 2008 with a mesenteric carcinoid tumor compressing the superior mesenteric vein, resulting in recurrent episodes of GI bleeding from mesenteric varices. His relevant medical history began in 2005, when he underwent a work-up at another institution for chronic diarrhea at that time. He complained of up to 15 watery bowel movements a day for the past several years. He underwent an unrevealing esophagogastroduodenoscopy (EGD) and colonoscopy, but an abdominal CT scan showed a 4.6 × 2.5 cm mesenteric soft tissue mass encasing the superior mesenteric vein and extending along the mesenteric root to the base of the pancreas  and mesenteric lymph nodes (9 of 18 nodes positive). All resection margins were negative for malignancy. By TNM staging (as defined by ENETS), his tumor was stage IIIB. The patient had a complicated post-operative course. A second-look laparotomy performed on postoperative day 1 revealed all of the bowel to be viable. The patient was brought back to the operating room on postoperative day 4 with plans to connect the cecum and descending colon. At this laparotomy, the ileojejunal segment of bowel looked dusky, albeit not frankly necrotic. The ileocolic segment remained pink and well-perfused. A near-occlusive clot was found at the superior mesenteric artery anastomosis. Fogarty thrombectomy restored good flow to that bowel segment. Continued acidosis and hemodynamic instability required re-exploration the next day, at which point a large thrombus was again palpated in the SMA, and the ileojejunal segment of bowel was found to be nonviable. This segment of transplanted bowel was therefore resected, requiring take-down of the pancreaticojejunostomy, choledochojejunostomy and gastrojejunostomy. Reconstruction could not be performed with the ileocecal segment of bowel in its current location, so this segment was explanted, flushed with ice-cold lactated Ringer solution containing mannitol and then kept on ice. The venotomy and arteriotomy on the vena cava and abdominal aorta were patched with pieces of ileal vein and artery, respectively. The ileocecal segment (including ~70 cm of ileum) was then revascularized via the superior mesenteric artery and vein. The bowel was pink immediately upon reperfusion. A pancreaticoileostomy, gastroileostomy and choledochoileostomy were then performed in the standard fashion. Reconstruction was completed by anastomosing the cecum to the descending colon Figure . PostopeCandida albicans) and a final laparotomy for abdominal washout with saline containing amphotericin B and gentamicin. He did have a small leak at his pancreaticoileostomy that was managed conservatively with NPO status and initiation of TPN. He was discharged on TPN on POD 72, tolerating some tube feeds and enteral intake.His subsequent hospital course was remarkable for periodic febrile episodes requiring first IR drain placement into a pelvic fluid collection .e.g. small intestine and appendix) are by far the most common, with tumors distal to the jejunum representing 96% of carcinoids located in the gastrointestinal tract [Of the gastrointestinal carcinoids, those arising from the midgut  can become completely encased in tumor, causing regional portal hypertension and arterial insufficiency [Carcinoid metastases to the mesentery often grow far larger than the submucosal primary tumors in the small bowel wall, and they are responsible for much of the morbidity and mortality of gastrointestinal carcinoid tumor that is not attributable to carcinoid syndrome itself. Many patients with mesenteric carcinoid present with small bowel obstructive symptoms due to tethering and kinking of the small bowel to the rigid mesentery. In several surgical case series of patients with midgut carcinoids, 62-67% required laparotomy for either intestinal obstruction or abdominal pain, and of these patients, 67-79% had evidence of extensive mesenteric fibrosis upon surgical exploration ,13. In oficiency -16. The Although somatostatin analogs such as octreotide remain the mainstay of carcinoid therapy, surgical resection has emerged as a vital treatment in disease management. For metastatic disease, palliative cytoreductive surgery has been employed by some groups to specifically address mesenteric carcinoids. While some symptomatic improvement is often reported, long-term outcomes are compromised by recurrent disease ,17,18. Uen bloc and maintained in cold preservative fluid to limit warm ischemia injury. The mesenteric mass is then fully excised, and the intestine is autotransplanted back into the patient. This strategy was first employed by David Lai and colleagues to treat a nonfunctioning islet cell carcinoma [Recently, however, several groups have published reports outlining the use of intestinal autotransplantation to safely gain access and resect tumors that encase the vasculature of the mesenteric root. Utilizing techniques from intestinal allotransplantation, the patient's small intestine is harvested arcinoma . That pain situ resection of the carcinoid tumor, the patients in Tzakis' case series had ex vivo resection of their lesions on ice on the back table after their organs had been removed en bloc. The mesenteric lesions of his series did not apparently extend into the distal branches of the SMA, obviating the need to autotransplant multiple segments of intestine as in the case we present here. All of his patients had good outcomes except for the patient treated for pancreatic cancer, who died of hepatic metastases 7 months postoperatively. Two additional case reports of intestinal autotransplantation to treat locally-advanced pancreatic cancer extending into the mesenteric root were also complicated by rapid recurrence of hepatic or peritoneal metastases, and early death after the procedure [A similar approach was employed by Tzakis and colleagues to treat four patients with lesions involving the root of the mesentery. The indications for resection in these patients were pancreatic head fibroma, vascular malformation at the mesenteric root, desmoid tumor of the pancreatic tail, and locally advanced pancreatic adenocarcinoma ,21. Wherrocedure ,23. ThesHere we present the first description of partial abdominal evisceration and intestinal autotransplantation used to treat a mesenteric carcinoid tumor. This method may prove particularly useful for midgut carcinoid tumors, as this malignancy frequently extends into the mesenteric root, with enormous clinical consequences. While stenting of the superior mesenteric vein was recently described to palliate the symptoms of intestinal congestion that often accompany mesenteric spread of midgut carcinoids , intestiEGD: esophagogastroduodenoscopy; EUS: endoscopic ultrasound; SMA: superior mesenteric artery; SMV: superior mesenteric vein; TPN: total parenteral nutritionThe authors declare that they have no competing interests.WHK prepared the manuscript and all illustrations contained therein. MH was the primary surgeon and helped to develop the surgical technique. ABC and NE were co-surgeons involved in the development of the surgical technique, and ABC also provided critical help revising the manuscript. LSB was the medical oncologist of the patient and contributed to his preoperative and postoperative care. All authors read and approved the final manuscript.Written consent was obtained from the patient for publication of this case report and the accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Hydranencephaly is a rare congenital abnormality characterized by the absence and replacement of the cerebral hemispheres with cerebrospinal fluid. Here, we present an ultrasonographic diagnosis of a case of a rare variant of fetal hydranencephaly at 38 weeks of gestation. Obstetric sonography revealed the absence of the cerebral cortex, thalami and basal ganglia with a disrupted falx and preserved posterior fossa structures. This is the first reported case of hydranencephaly with the absence of the thalami and basal ganglia, which was diagnosed prenatally. The diagnosis was confirmed with postnatal computed tomography. The early prenatal diagnosis allowed for prompt obstetric attention at a tertiary care hospital which had specialized pediatric facilities including prenatal counseling and support. Crome and Sylvester then reviewed the disease and defined it as a congenital condition2. It is an extremely rare and unique abnormality occurring in less than 1 per 10,000 births worldwide3. It is also said to be present in 0.2% of infant autopsies, and approximately 1% of babies diagnosed clinically as hydrocephalus4. It is critical to differentiate between hydranencephaly and extreme hydrocephalus as the latter carries a potentially better prognosis5. Here, we report a rare case of hydranencephaly, which was diagnosed in the late third trimester, followed by successful obstetric care and management.Hydranencephaly is a rare congenital abnormality characterized by the absence and replacement of the cerebral hemispheres with cerebrospinal fluid. It was first described by Cruveilher (1892) as “Anencephalie hydrocephalique” or “Hydroanencephalie”A 28-year-old women (Gravida 2 and para 1) at 38 weeks of gestation was presented to our obstetrics and gynecology unit with lower abdominal pain. She and her husband were Sinhalese and unrelated, with no family history of genetic or congenital anomalies. The patient didn’t smoke, and there was no history that suggested congenital infections or exposure to toxins. In her first pregnancy she delivered a normal healthy male child by an uncomplicated vaginal delivery. In her most recent pregnancy, she didn’t attend the local antenatal clinic regularly, and thus was recognized as a noncompliant patient. She had not had a routine dating scan, anomaly scan or growth scan of the fetus during her pregnancy. The clinical obstetric examination at presentation was unremarkable, and the cardiotocography of the fetus was normal.Upon inspection, the obstetric ultrasound scan showed fluid filled cranial cavity with an absent cerebral cortex, thalami and basal ganglia. The third ventricle appeared to be dilated and the remnants of midbrain structures were present see. The cerThe newborn was immediately transferred to the neonatal intensive care unit because of respiratory distress. A non-contrast computed tomography (CT) of the newborn’s head was performed two days after the delivery. The CT scan revealed that there was no cerebral cortex, thalami or basal ganglia. The third ventricle looked dilated and remnants of midbrain structures could be seen see. The cer6. The midbrain, thalamus, basal ganglia, choroids plexus, cerebellum and brain stem are usually preserved and contained within the skull. The falx cerebri is usually present but may be partially or entirely absent and the septum pellucidum may also be absent.Hydranencephaly is an encephaloclastic abnormality characterized by the absence and replacement of the cerebral hemispheres with cerebrospinal fluid and necrotic debris, covered by leptomeninges. Usually, there is no cerebral cortex but there may be partial preservation of a portion from the occipital lobe7. Myers described the etiology of hydranencephaly by experimenting on laboratory monkeys. In his study, monkey fetuses were subjected to ligation of the bilateral carotid arteries and jugular veins in the neck at different gestational ages. These fetuses were then restored to the uterus, brought to term and subsequently delivered. Examination of the baby monkey brains revealed hydranencephaly, which resulted from a vascular shutdown predominantly when carried out during earlier gestational age8.The etiopathogenesis of hydranencephaly is heterogeneous, and so several theories have been postulated for its occurrence. The most common etiology described is the occlusion of the supra-clinoid segment of the bilateral internal carotid arteries causing ischemic degeneration of structures supplied by them9. Other etiologies of hydranencephaly include intrauterine infections, which leads to the local destruction of brain tissue e.g. congenital toxoplasmosis10 or other viral infections 11. Another etiology that has arisen is the maternal exposure to carbon monoxide or butane gas, which can result in fetal hypoxia which in turn leads to massive tissue necrosis with cavitations, resorption of necrotized tissue and necrotizing vasculitis12.Some case reports suggest the occlusion of the internal carotid arteries due to a temporary spasm rather that direct occlusion leading to ischemic destruction of certain brain structures13. It has been revealed that vascular interchanges between monozygotic twinning can result in the transfer of intravascular coagulation materials from the deceased co-twin to the surviving twin causing thromboembolism15. Hydranencephaly has also been associated with various congenital anomalies, including Fowler syndrome, arthrogryposis, renal aplastic dysplasia, poly-valvular heart defect, and trisomy18.Monozygotic twinning has also been associated with congenital hydranencephaly19. The appearance of the thalami and brainstem protruding into the cystic cavity was characteristic, together with a midline echo from the remnants of the falx, the tentorium cerebelli and cerebellum20. The third ventricle and choroids plexus were often visible and the absence of the septum pellucidum may give rise to a single ventricle in the midline21. There is a main difference in the diagnosis of extreme hydrocephaly, alobar holoprosencephaly and porencephaly23. In these conditions, the above-mentioned structures will still be surrounded by a rim of cortex; as they carry a better prognosis, it is essential to try and differentiate them in the ultrasonographic imaging of hydranencephaly. In extreme cases of hydrocephalus, the thin cortical layer may be difficult to recognize sonographically, and thus Magnetic resonance imaging (MRI) or intrauterine CT scans can be used to support the diagnosis24.The cranial ultrasonographic imaging of hydranencephaly shows a large cystic mass filling the entire intracranial cavity with the absence or discontinuity of the cerebral cortex18; however there have been some cases of sonographic diagnosis of fetal hydranencephaly in the first trimester25. Hydranencephaly has a poor prognosis, as the majority of brainstem functions are missing. Affected newborns can die at birth, but most infants die within the first year of their life26. If a child does survive they will inevitably be severely handicapped.The majority of cases of hydranencephaly are detected in the second half of a pregnancy27. Counseling the parents regarding the poor prognosis and the potential management options is advised to help them prepare for the potential outcome. Education of the prognosis is a necessary step that allows the family time to prepare and come to terms with the eventualities and can help provide immediate support when required27. Because of the poor prognosis, termination of pregnancy is recommended once a definitive diagnosis has been established24. If macrocrania is identified in late pregnancy then cephalocentesis may be suggested as an option to aid the delivery24.The social and emotional problems that occur after a delivery of a child suffering from hydranencephaly can be terribly depressing for the family29 with the thalamus or basal ganglia (or both) structures preserved in the majority of cases. This is the first published case of prenatal diagnosed hydranencephaly with the absence of the thalami and basal ganglia along with ruminants of the midbrain in the same patient. Sonographic assessment is sufficient for the prenatal diagnosis of hydranencephaly in most cases, and MRI or an intrauterine CT should be used to support the sonographcic asssment and shouldn’t be considered as a first-line diagnostic tool.The ultrasonographic findings that we described, led to the diagnosis of hydranencephaly being the most likely outcome and this was confirmed by postnatal CT of the fetal head. Remnant or preserved midbrain structures were observed in the most reported cases of hydranencephalyTimely diagnosis is crucial in hydranencephaly cases as early treatment options can avoid obstetrics complications, and early diagnosis is particularly useful for anticipating the need to give appropriate counseling to the parents during the pregnancy. An early diagnosis is also fundamental for preparing the optimal conditions of delivery and allowing for a specialized pediatric delivery unit to be on hand for the delivery.Written informed consent 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 Editors. Line 1, remove the comma after "....described, ..."Line 3, remove "the" in "observed in the most reported...."Line 7, the correct spelling is "sonographic", not "sonogrpahcic"Otherwise, I approve of the latest version.There are three typographical errors in the Conclusion -I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. Reference 6 should read Chinsky JM, not Jeffrey MC.significantly higher prognosis” (line 6) to “potentially better prognosis”.In the introduction I would suggest changing “Hydranencephaly has a really poor prognosis" so that it simply reads "poor prognosis".In the Discussion, paragraph 6; remove "really" from "This second revision is fine except for the following suggestions/corrections:I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. The corrections made by the author are very satisfying. I now approve this article.I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. This case report is interesting. Except of some grammatical mistakes, I do have one suggestion.Petridis et al. (2011) deals with the social aspects of hydranencephaly and should be cited by the author.The authors should also discuss the social aspects and the social stress which accompanies families with hydranencephalic children. It is important to induce abortion when possible in such cases since the prognosis is very poor. Early diagnosis is the most important aspect in such cases. The paper of mine,I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard, however I have significant reservations, as outlined above. We would like to thank Dr Petridis for the valuable time spent reviewing our manuscript and the important comments he has made. We have attempted to address and incorporate most of the concerns that were raised in version 2 of our article. This case report presents an interesting case of apparent hydranencephalic changes, in addition to more extensive developmental brain anomalies . However, including the points made below would make the manuscript much more useful as an educational resource:The manuscript would be enhanced if the author’s did more than just itemize a list of associated etiologies for hydranancephaly. The authors should provide a paragraph explaining how any of these etiologies could specifically produce the anomalies observed  and perhaps contrast it with any syndromic or associated genomic alterations listings of similar brain findings (or at least provide evidence that they researched this aspect).I’m not sure all would accept this as a variant of the same processes that cause classic hydranencephaly since it appears to be so much more extensive anomalous formation of the final fetal brain. The authors may have to do a little more research and discussion to support their contention based on how others who have written about hydranencphaly and define the condition.There are several grammatical errors (e.g. in the Abstract “such that absent cortex” is an incomplete phrase to end the sentence) which should be addressed, and I suggest the article is proof read again to ensure it reads how a published manuscript should do.I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. We would like to thank the reviewer, Dr. Chinsky for the valuable time spent reviewing our manuscript and the important comments he has made. We have attempted to address and incorporate most of the concerns that were raised in version 2 of our article.


Isolated angiitis of the central nervous system (IACNS) is a rare but severe vascular disease, which could present like an isolated inflammatory lesion on magnetic resonance imaging (MRI). To date, only a few such cases with tumor-like IACNS have been reported.A 35-year-old woman presented with headache and left-sided weakness. MRI scans initially mislead us to a diagnosis of glioblastoma (GBM). Surgery was performed. The mass was sub-totally resected. Pathological examination confirmed a cerebral vasculitis. Radiological features, such as disproportionate mass effect, striped hemorrhage and abnormal enhancement of adjacent vessels, could be helpful to distinguish a tumor-like IACNS from a GBM. Single therapy with high doses of steroid did not improve the patient's condition. Combined therapy with prednisolone and cyclophosphamide showed great benefit to the patient. No relapse occurred during the period of 18 months follow-up.Although a tumor-like IACNS has no established imaging features, a diagnosis of tumor-like IACNS should be suspected when MRI shows inappropriate presentations of a tumor. Greater awareness of this potential manifestation of IACNS may facilitate more prompt diagnosis and treatment. Isolated angiitis of the central nervous systmen (IACNS) represents a rare and poorly understood form of vascular inflammatory disease restricted to the brain and spinal cord. An average annual incidence rate of 2.4 cases per 1,000,000 person-years was found by a report from US , particuIn April 2008, a 35-year-old woman was admitted to our hospital due to headache and left-sided weakness over the preceding 1 month. The headache was diffuse and did not have a burning or stabbing sensation. The weakness of the left arm and jaw was mild. Her mental status was clear with normal orientation and alertness. It was negative in speech disorder and perceptual disturbance. A review of systems at the time of presentation revealed no additional symptoms, except for mild hypomnesis. There was no history of alcohol or illicit drug use. Toxic exposure history was negative. On neurologic examination, the patient presented moderate weakness on the left side. An equivocal Babinski sign was elicited in the left foot. Findings on physical examination were normal. Admission MRI study of the brain Figure . IntraopIACNS, which was first reported as early as 1952 , can occClinical onset of IACNS is usually subacute, but it can have acute onset with rapid progress within a few days or weeks. The most frequent clinical manifestations at presentation are headache, altered cognition, hemiparesis or persistent neurological deficit or stroke . SubaracWith regard to imaging characteristics, IACNS always presents a challenge in radiographic diagnosis. Its MRI findings are highly variable, ranging from multiple irregular white matter changes to intracerebral hemorrhages ,10. The Moore  recommenNo clinical trials have been performed in patients with IACNS, nor is it possible to draw firm conclusions from the current study because of the non-standardized nature of the treatment protocols used in these cases. Fountain et al.  reportedThe prognosis of IACNS varies greatly and could be from months to years ,11,17. UIn conclusion, we suggest that correct diagnosis and appropriate treatment of tumor-like IACNS should be essential for prognosis. When the MRI shows a tumor-like subcortical lesion with disproportionate mass effect, and/or striped hemorrhage and/or abnormal enhancement of adjacent vessels, an IACNS should be include in the differential diagnosis. When high doses of steroids show no effect to the patient with tumor-like IACNS, combined treatment with cyclophosphamide followed by long term oral therapy is recommended.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.GY wrote the initial draft. WY drew the pictures. WZ revised the draft. SL described the MRI features. GL provided the pathological diagnosis. TJ was the surgeon and gave the final approval of the version to be published.


We report a case of a 33-year-old male with a mixed germ-cell testicular tumor. Postoperative follow-up FDG-PET revealed concentration of FDG in the left inguinal area which is not tumor metastasis or local recurrence but suture reactivity granuloma. In this paper, we reviewed suture granulomas associated with false-positive findings on FDG-PET after surgery. If FDG-PET will be used more frequently in the future, it will be necessary to refrain from using silk thread in order to prevent any unnecessary surgery. Imaging studies are fundamental to the diagnosis and planning of therapy for testicular tumors. FDG-PET (18F-fluorodeoxy glucose-positron emission tomography) is indicated extensively and serves a very useful purpose in detecting malignant tumors and evaluating therapy. However, the use of FDG-PET based on glucose metabolism understandingly produces false-positive results in cases with inflammatory or granulation tissue and can occasionally confuse clinicians.This report presents the case of suture reactivity granuloma showing a false-positive finding on postoperative follow-up FDG-PET after high orchiectomy in a patient with a mixed germ-cell tumor that complicated both the diagnosis of recurrent testicular tumors and the selection of the optimal therapeutic alternatives.β-hCG level was 19.5 ng/mL. The alpha fetoprotein level was 3 ng/mL, overlapping the normal range. The LDH level was increased to 1,690 IU/L. Abdominal-pelvic CT performed on the initial visit revealed a left testicular tumor with left inguinal and para-aortic lymph node swelling. The patient was diagnosed with a stage IIIc testicular tumor and immediately underwent high orchiectomy. The histopathological diagnosis was a mixed germ-cell tumor . A 33-year-old male was referred to the urological department for treatment of ongoing edema in both legs and left testicular enlargement. A physical examination revealed the left testis to be enlarged to the size of a fist with a hard, irregular surface and left subclavian lymph node swelling. A complete blood count was within the normal limits. The C-reactive protein level was increased to 3.84 mg/dL. The serum human chorionic gonadotropin (hCG) level was significantly elevated to 3,360 mIU/mL and the The patient received three courses of BEP  therapy, and one course of EP (Etoposide and Cisplatin) therapy was added as postoperative chemotherapy. The levels of tumor markers returned to the normal ranges and became negative one month later, and abdominal-pelvic CT revealed significant reductions of the lymph nodes and liver metastases. Four courses of VIP  therapy were administered as additional chemotherapy for residual para-aortic lymph node swelling. Although follow-up CT revealed no significant enlargement of lymph nodes or the presence of metastases, FDG-PET revealed a concentration of FDG in the left inguinal area, which was a surgical site . TherefoAlthough FDG-PET was originally thought to be less useful for detecting urological malignancies because FDG is eliminated via the kidneys, some reports have demonstrated the usefulness of FDG-PET for detecting urological malignancies. Hinz et al. suggested that FDG-PET is useful under specific conditions, such as when the size of the residual mass is greater than 3 cm; however, because the possibility of false-positives is not low, it is necessary to be careful when determining the indications for surgical treatment of residual masses . De SantThe treatment guidelines for testicular tumors issued by the European Association of Urology and The National Comprehensive Cancer Network do not recognize the usefulness of FDG-PET for staging or the postoperative followup of nonseminoma disease. This is because the use of FDG-PET can lead to the unnecessary treatment of patients due to the accumulation of inflammatory and granulation tissue. In fact, some reports of suture granulomas associated with false-positive findings on FDG-PET after surgery have been published . All of Chung et al. reported a case in which they performed an aspiration biopsy using ultrasound and diagnosed FDG-PET-positive findings as a suture granuloma without performing open surgery, while, in another case, they diagnosed a suture granuloma using open surgery –8. In caSome studies have reported that absorption thread, including manufactured silk, can be a more frequent cause of suture granulomas than nonabsorption thread , 10. Mor In conclusion, PET is useful for the postoperative followup of seminoma; however, sufficient evidence has not yet been accumulated for nonseminoma cases. Recently, other case reports similar to ours of suture granulomas in other organs have been published. Therefore, clinicians must consider the possibility for suture granulomas in cases with positive findings on PET detected within the surgical site during postoperative followup of nonseminoma. Of course, tumor markers must be evaluated in order to judge whether postoperative positive findings on PET indicate suture granulomas or tumor recurrence. However, clinicians have no choice but to perform tissue diagnosis at present. Therefore, large-scale studies of the effectiveness and impact of PET in the postoperative followup of testicular tumors are needed in the future.


A solid pseudopapillary neoplasm (SPN) of the pancreas has distinct histopathological features. A solid pattern of growth with pseudopapillary structures that result from degeneration is observed. On rare occasions, the tumor may vary from being entirely solid to completely cystic. The present study describes two unique cases of SPN. A 25-year-old male presented with a pancreatic tumor showing a predominantly solid pattern with no degenerative change, although the pre-operative cytological specimens that were obtained by endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) revealed pseudopapillary structures. The second case was of an 11-year-old female who presented with a pancreatic tumor with prominent degeneration. Nests and cords of the remaining neoplastic cells were located only at the periphery, with perineural invasion. An immunohistochemical analysis revealed that the tumor cells in the two cases were positive for CD10 and β-catenin and negative for trypsin. An awareness of the broad morphological variability of SPN and an immunohistochemical panel that includes CD10, β-catenin and trypsin are useful for establishing an accurate diagnosis. A solid pseudopapillary neoplasm (SPN) is a low malignant epithelial tumor of the pancreas that predominantly occurs in adolescent and young females ,2. In unA 25-year-old male was admitted to Gifu University Hospital  with a two-week history of diarrhea and abdominal pain. The results of routine laboratory tests were all within the normal range. However, an abdominal ultrasound revealed a mass in the pancreatic head, which was composed of high-echoic central and low-echoic peripheral areas. Computed tomography (CT) and magnetic resonance imaging identified the nodule to be predominantly solid and ~28 mm in maximal diameter. The cytological smear and cell block of the sample that was obtained by endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) revealed the presence of uniformly monomorphic tumor cells, several layers of which covered the central fibrovascular stalks and formed papillary-like structures . The patAn 11-year-old female presented to Gifu University Hospital due to a sudden onset of abdominal pain. Laboratory tests revealed a high level of serum amylase, suggesting acute pancreatitis. An abdominal CT scan disclosed a cystic mass  with a clear margin in the pancreatic tail. The follow-up examination revealed that the size of the mass had become smaller within the next three months with no treatment, attaining a size of 18 mm in maximal diameter. The lesion was excised by a distal pancreatomy. Prior to the surgery, no histological or cytological examinations were performed. On gross examination, the yellowish tumor was prominently degenerative with cystic and hemorrhagic changes . MicroscIn cases 1 and 2, a diagnosis of SPN was confirmed and no adjuvant therapy was administered. No residual tumor or metastases were identified during the follow-up period.et al that the EUS-FNA diagnosis of an SPN is considered to be accurate (et al also suggested that a cytological diagnosis of SPN may be rendered with great confidence in unusual presentations, including those that are identified in older patients, males, ectopic locations and metastatic sites (The microscopic appearance of an SPN is variable ,5. Histoaccurate . Pettinaic sites .The evaluation of neuroendocrine differentiation in the tumors using immunohistochemical markers, including synaptophysin, chromogranin, CD56 and NSE, is also important in order to differentiate between an SPN and an NET. Caution should be paid, however, since the cellular differentiation of SPNs remains to be elucidated and, therefore, immunohistochemistry may be of marginal use in this context . In geneIn certain instances, SPNs are prominently cystic, with only thin peripheral rims of the remaining tumor cells, as in case 2. Notably, perineural invasion was identified in the remaining tumor area of case 2, which may indicate malignant behavior . To diffet al, it was reported that CD10 immunopositivity was detected diffusely in all the SPNs that were examined, whereas the immunopositivity was scattered in 67% of ACCs and focal in 20% of NETs (Studies have shown that immunohistochemical CD10 and β-catenin are valuable to establish a diagnosis of SPN. CD10 is a cell-surface neutral endopeptidase, an antibody which is used in daily practice as a marker of several cell lineages, including germinal center cells, renal tubular or glomerular cells and endometrial stromal cells . In a st of NETs . Further of NETs ,17. Immu of NETs ,17. Theset al is that of a gender difference (The reason why SPN has numerous morphological variations remains undetermined. A plausible explanation is the size of the tumor. The solid variants of SPN represent a number of small tumors that have not grown large enough to undergo cystic degeneration . Anotherfference . The stufference . TherefoIn summary, the current study presents two cases of rare SPNs with unusual macroscopical and microscopical appearances. The literature suggests that when the solid or cystic area is predominant in an SPN, a detailed observation and careful interpretation of the cytological and immunohistochemical findings may be useful to avoid a potential misdiagnosis.


The adenomatoid odontogenic tumor (AOT) is a benign, nonneoplastic (hamartomatous) lesion with a slow progressing growth. It occurs in both intraosseous and peripheral forms. This paper reports the case of a female aged 16 years who presented with a swelling in anterior maxilla; canine was missing, and a supernumerary tooth was present in the mid line. Radiology revealed a well-defined radiolucent area associated with impacted canine and root resorption of adjacent teeth, which was diagnosed histopathologically as AOT. The patient was treated surgically and later rehabilitated with fixed prosthesis. Histological typing of odontogenic tumors,” adenomatoid odontogenic tumor (AOT) is defined as “A tumor of odontogenic epithelium with duct-like structures and with varying degrees of inductive change in the connective tissue. The tumor may be partly cystic, and in some cases the solid lesion may be present only as masses in the wall of a large cyst” [According to the second edition of the WHO “ge cyst” . AOT is ge cyst” , 3.Histological typing of odontogenic tumors, jaw cysts and allied lesions” in 1971 and was retained in the second edition in 1992.AOT is a relatively uncommon distinct odontogenic neoplasm that was first described by Steensland in 1905 . DreibalUnal et al. in 1995 produced a list containing all nomenclatures for AOT reported in the literature  like ade A 16-year-old girl presented with left anterior maxillary swelling of about 2 months duration. It was a slow growing swelling, without any other symptoms. On clinical examination, the left cheek was grossly deformed with normal overlying skin, and there was no neurological deficit over the affected area and no nasal discharge . The palThe radiographs showed a well-defined, unilocular radiolucency in maxilla with expansion and thinning of all its bony walls with the left upper canine tooth and without any evidence of calcifications . It alsoThe enucleation of the cyst was done under local anaesthesia along with the removal of the impacted canine, supernumerary, and first premolar tooth . It contMacroscopically, the specimen measured 4.5 × 3.5 × 4.0 cms with a smooth surface and was associated with a well-developed canine crown portion, circumscribed by cyst . The cysThe AOT is an uncommon cause of jaw swelling . The tumThere is a slight female over male predilection, almost 2 : 1 . If geogAlthough larger lesions have been reported in the literature , 27, theHistologically, the tumor is solid, and there is a cyst formation. The epithelium is in the form of whorled masses of spindle cells as well as sheets and plexiform strands. Rings of columnar cells give rise to duct-like appearance. Calcification is sometimes seen and may be extensive . InteresThe tumor is well encapsulated and showed an identical benign behaviour . Therefo


Castleman's disease is a distinct form of lymph node hyperplasia divided into a solitary and a multicentric type. The solitary type occurs most commonly in the mediastinum and is usually asymptomatic. We present a patient with Castleman's disease of the hyaline-vascular solitary type located in the retroperitoneum. The patient was a 38-year-old male, who presented to our hospital with fever. The imaging workup revealed a retroperitoneal mass, measuring 4 × 6 cm, located lateral to the aorta, inferior to the left renal artery and vein, and posterior to the left testicular vein. At workup, a solid hepatic lesion, 3 cm in diameter, located in the left lobe of the liver, segment IV, was also identified. Both lesions were surgically excised. The retroperitoneal tumor had the features of angiofollicular hyperplasia (Castleman's disease), hyaline-vascular type, whereas a diagnosis of focal nodular hyperplasia was made for the hepatic lesion. The patient is well at fourty months followup postoperatively. Surgical excision is the treatment of choice for unifocal Castleman's disease. Castleman's disease is a rare form of lymph node hyperplasia of unknown etiology, which has been divided into a solitary and a multicentric type. The solitary type is located mostly in the mediastinum, but it may also occur in other anatomical sites. It is usually asymptomatic. Occasionally it may cause symptoms from pressure of adjacent organs. The multicentric type of the disease is usually symptomatic, with severe systematic phenomena and may be associated with lymphoma, myeloma, and Kaposi's sarcoma. We present a rare case of solitary retroperitoneal Castleman's disease coexisting with focal nodular hyperplasia of the liver in an otherwise asymptomatic patient.A 38-year-old man with unremarkable medical history presented with fever. Clinical workup included an abdominal ultrasound scan which revealed a mass of 6,5 × 5 cm located in the left retroperitoneum and a lesion of 3,3 cm in diameter in the left lobe of the liver. The retroperitoneal mass was solid and was in contact with the left renal vessels, without any hemodynamic features of malignancy. The most probable imaging diagnosis was a teratoma. The hepatic lesion was solid and hypoechoic, with increased vascularization, most likely presenting focal nodular hyperplasia. No other findings were present. Consequently, an abdominal CT scan was performed to delineate the retroperitoneal pararenal mass and an abdominal MRI  for the Physical examination, routine blood count, and blood biochemistry were normal. In particular, no tumor markers  were detected and the viral markers  were negative. The chest X-ray was unremarkable. en bloc with the peri-renal fat and sent to the pathology department for intraoperative evaluation. The frozen section examination identified a lymphoid lesion and the definite diagnosis was deferred. Subsequently, the patient underwent wedge resection for the hepatic lesion. Histology confirmed the diagnosis of focal nodular hyperplasia. Paraffin sections of the retroperitoneal tumor revealed angiofollicular hyperplasia (Castleman's disease) of the hyaline-vascular type . It is usually unifocal, forming a mass up to 15 cm. It is usually asymptomatic and identified as an incidental finding in U/S or CT scans. When it is large, it may cause symptoms, such as cough, dyspnea, neuropathy, jaundice, postprandial discomfort, anorexia, vomiting, and urinary retention  due to p0%. It is The plasma cell type 10%) is usually associated with the multifocal form of the disease , 6, 7. H% is usuaIn the case we present, the patient was asymptomatic and the mass was discovered as an incidental finding, characteristics that are typical of the hyaline-vascular type of the disease. An interesting aspect of the case we present is the synchronous presence of a hepatic lesion, which was also difficult to define preoperatively. Additionally, imaging studies and perioperative findings do not distinguish retroperitoneal Castleman's disease from malignant retroperitoneal tumors, particularly in the presence of a hepatic lesion. Gross inspection also fails to distinguish the lesion from a malignant neoplasm, because it may develop dense adhesions to adjacent organs , 13. A qCastleman's disease is a lymph node disease. Its multicentric form seems to be related with human herpes virus 8. It presents mainly in two types: the unifocal  and the multifocal (plasma cell type). The localized hyaline vascular form presents as a solitary lesion which can be located mainly in the mediastinum, but also in the abdomen, retroperitoneum, or in many other locations. A good preoperative workup and an open biopsy during surgery, for an abdominal or retroperitoneal lesion if no definitive preoperative diagnosis has been established, can help to avoid extensive resection when dealing this benign disorder. Complete surgical excision is curative; recurrences have only been described after incomplete resection. The prognosis is excellent with a five years survival of nearly 100%.


Based on exclusion criteria in the landmark NINDS-rtPA trial, current expert consensus guidelines preclude the use of intravenous recombinant tissue plasminogen activator (IV rtPA) in acute ischemic stroke (AIS) patients with intracranial neoplasm. There are only 3 published cases of administration of IV rtPA to AIS patients with intracranial neoplasms in the literature. Two of these published cases involved malignant brain parenchymal lesions discovered only after rtPA was inadvertently given, and one of these cases was associated with hemorrhage within the tumor. In this paper, we report two cases of administration of IV rtPA in AIS patients with intracranial neoplasms observed on neuroimaging prior to IV rtPA administration. In both cases, the tumor was outside of the brain parenchyma. The first case was an acoustic schwannoma and the second a falcine meningioma. Neither case was associated with intratumoral hemorrhage as of at least one week following IV rtPA treatment. More published cases are definitely warranted, but our experience with these two cases suggests that administration of IV rtPA to AIS patients in the presence of extraparenchymal brain tumors may not necessarily precipitate intra-tumoral bleeding and thereby worsen clinical outcomes. Fifteen years after Food and Drug Administration approval of intravenous recombinant tissue plasminogen activator (IV rtPA) for treatment of acute ischemic stroke (AIS), only a substantial minority of AIS patients <2%) of strokes receive this proven therapy . While s% of stroA 77-year-old man developed sudden onset left upper, and lower extremity weakness. His past medical history included hypertension, hyperlipidemia and type 2 diabetes. He had experienced two prior strokes (the more recent one two years prior to this presentation) which resulted in residual mild left hemiparesis. On admission, blood pressure was 146/50 mmHg, and pulse was 56 beats per minute and regular. Neurological examination revealed flaccid left lower extremity weakness affecting proximal and distal muscles. He was unable to walk. National Institutes of Health Stroke Scale Score (NIHSSS) was 4. Noncontrast head CT demonstrated a hyperdense right anterior cerebral artery (ACA) and a heterogeneous lobulated mass within the left cerebellopontine angle measuring 3.3 × 1.3 cm. There was mild mass effect on the adjacent pons . CT angiA 74-year-old woman arrived after a fall witnessed by family members. Past history included type 2 diabetes, hypertension, and hyperlipidemia. Initial blood pressure 165/77 mmHg, and pulse was 83 beats per minute and irregularly irregular. Neurological evaluation revealed right gaze deviation, flattening of the left nasolabial fold, and dysarthria, flaccid left arm with no strength, weakness of left leg (2/5 strength), and left-sided neglect. NIHSSS was 13. Noncontrast head CT demonstrated a hyperdense right middle cerebral artery, bilateral basal ganglia calcification, and an occipital falcine mass . She recWe report two cases of safe administration of IV t-PA in acute ischemic stroke patients with intracranial neoplasm. In both cases, the tumor was outside of the brain parenchyma. The first case was possibly an acoustic schwannoma, and the second a falcine meningioma. Neither case was associated with intratumor hemorrhage. As already noted, there are extremely few cases of IV t-PA use in AIS patients with intracranial neoplasms reported in the literature. Two of these cases involved initially undetected malignant intracranial neoplasms, while the remaining one involved a benign intracranial neoplasm seen prior to treatment. For instance, a 57-year-old man with a left temporal lobe glioblastoma multiforme (GBM) (subsequently diagnosed after magnetic resonance imaging and neurosurgery days later) was inadvertently given IV rtPA after a normal initial head CT. There was no evidence of blood by that MRI or follow-up head CT. The patient became asymptomatic on the seventh day . More siAdmittedly, the number of reported cases of rtPA treatment for AIS in patients with intracranial neoplasms is far too small to make any definitive inferences, but there is a suggestion, based on previous cases as well as our current report, that the presence of a presumably benign intracranial neoplasm seen on neuroimaging prior to IV rtPA treatment may not necessarily contribute to unfavorable imaging or clinical outcomes in AIS and perhaps should not preclude treatment. Further supportive evidence of possibly little harm from using intravenous thrombolysis among vascular disease patients with benign intracranial neoplasm may be seen among the few reported cases of i.v. rtPA use in nonstroke patients , 9. Thre


We report a case of mixed capillary venous hemangioma of the retroperitoneum in a 61-year-old man. Abdominal ultrasonography showed a mass to be hypoechoic with increased flow in color Doppler imaging. Dynamic contrast-enhanced computed tomography revealed a centripetal filling-in of the mass, located anterior to the left psoas muscle at the level of sacroiliac joint. On the basis of imaging features, preoperative diagnosis of hemangioma was considered and the mass was excised by laparoscopic method. Immunohistochemical studies were strongly positive for CD31 and CD34, and negative for calretinin, EMA, WT1, HMB45, Ki67, synaptophysin, and lymphatic endothelial cell marker D2–40. Histologically, the neoplasm was diagnosed as mixed capillary venous hemangioma. Primary retroperitoneal hemangioma is an extremely rare entity, of which, the most common is cavernous hemangioma. We report a case of retroperitoneal hemangioma which was diagnosed preoperatively by contrast-enhanced scan performed on a 256-slice multidetector computed tomography (CT). This is the first case report to our knowledge that describes a mixed capillary venous hemangioma in the retroperitoneal space.A 61 year-old otherwise healthy male presented with complains of intermittent, dull lower left abdominal pain, and back discomfort for 2 months. The patient has no other associated symptoms. A physical examination revealed a firm, mobile palpable mass 2 cm × 2 cm in size in the left lower quadrant of abdomen, associated with mild tenderness. Laboratory and biochemical examinations were reported normal. Abdominal ultrasonography (US)  showed a round, well-defined, mild heterogenous hypoechoic mass with posterior acoustic enhancement, anterior to the left psoas muscle with increased flow on color Doppler imaging . DynamicPrimary retroperitoneal tumors are relatively rare entity and constitute 0.07% to 0.6% of all tumors and around 62–82% are malignant, with liposarcoma being most common . Benign Although US is helpful in differentiating cystic from solid tumors, US appearance and echogenicity of hemangioma can be variable, from hyperechoic to anechoic, typically with posterior acoustic enhancement. CT images of previously reported cases showed cystic mass with minor or poorly enhanced lesion  or calciVirtually there is no risk of malignant transformation of hemangioma. Surgical treatment is usually indicated for large size tumor that has risk of rupture or bleeding , 2. WhilIn conclusion, we highlight a case of a benign vascular neoplasm in the retroperitoneal space. Although retroperitoneal hemangioma is rare entity, it should be considered in the differential diagnosis of retroperitoneal mass. While imaging feature of hemangioma can be typical occasionally, it should be correlated with histology to make a correct diagnosis.


Primary spinal glioblastoma (GBM) is a rare disease, with an aggressive course and a poor prognosis. We report a case of a 19-year-old male with a 4-week history of progressive weakness in both lower limbs, which progressed to paraparesis with a left predominance and difficulty in initiating urination over a week. Spine magnetic resonance imaging (MRI) showed an intramedullary expansile mass localised between T6 and T11. We performed a laminotomy and laminoplasty between T6 and T11 and the tumour was partially removed. Histopathological study was compatible with GBM. The patient was administered focal spine radiotherapy with chemotherapy with temozolamide. Serial MRI performed after the initial surgery demonstrated enlargement of the enhancing mass from T3 to T12 and subarachnoid metastatic deposits in C2 and C4, the pituitary stalk, inter-peduncular cistern, left superior cerebellar peduncle and hydrocephalus. We review the literature with regard to the disease and treatment options, and report the unique features of this case. Primary spinal GBM is an extremely rare entity with a poor prognosis and a short survival time. An aggressive management of the different complications as they arise and improvement of current modes of treatment and new treatment options are required to improve survival and ensure better quality of life. Astrocytoma is the most common primary tumour of the central nervous system . Spinal A 19-year-old male was transferred to our institution in May 2010 with a 4-week history of progressive weakness in both lower limbs, which progressed to paraparesis with a left predominance and difficulty in initiating urination over a week. Examination revealed spastic paraparesis  and hypoesthesia below the T10 sensory dermatome.The study was approved by the Ethics Committee of Hospital de Braga, Braga, Portugal. Informed consent was obtained from the patient’s family.The patient underwent brain and spine magnetic resonance imaging (MRI). The brain and cervical spine were negative for masses and signal intensity alterations, whereas from T1 to L1 there was a marked spinal cord signal intensity and morphology alteration, with notable spinal cord expansion between T6 and T11 and contrast enhancement between T6 and T9 .We performed a laminotomy and laminoplasty between T6 and T11, and partial tumour removal under motor-evoked potential monitoring. We were unable to distinguish the tumour margin from the spinal cord and decided to partially remove the mass.Histopathological study confirmed the diagnosis of GBM , with hiPost-operatively the patient had transient neurological deterioration with worsening of paraparesis, but with intensive rehabilitation his condition returned to baseline. Thoracoabdominal CT scan for extra-neuronal metastases was negative. The patient was administered spinal radiotherapy between T1 and L1 (45 Gy in 28 fractions) with chemotherapy with temozolamide (completed 6 cycles).Serial MRI at 3, 6 and 17 months was performed . Six monNeuroaxis MRI performed 17 months after surgery revealed an enlargement of the enhancement mass from T3 to T12 with less perilesional oedema and a new metastatic deposit in the interpeduncular cistern and left superior cerebellar peduncle.On the follow-up 20 months after surgery, the patient presented with a left third cranial nerve paralysis and bilateral mydriasis without signs of increased ICP or mental status change. CT scan showed larger ventricles and enlargement of the pituitary stalk and left superior cerebellar peduncle metastatic deposits. Given the disease progression and the Karnofsky score (40%), we did not consider further treatment and the patient succumbed to the disease 1 month later.Intramedullary GBM is a rare disease entity. It develops primarily from the spinal cord or as a secondary metastasis from the brain, which covers up to 25% of the total occurrences. Intramedullary GBM has a predilection to develop from the cervical region in primary cases, and has a tendency to develop at a young age (<30 years old). Despite the best treatment (surgery and adjuvant therapy), the estimated survival barely exceeds 6 to 16 months –11.Certain patients with malignant spinal cord astrocytomas develop hydrocephalus ,7, whichSeeding of an intracranial GBM along the spine occurs in 25% of cases, but the reverse process is extremely uncommon ,3. PatieAll current therapeutic measures have produced disappointing results and few data concerning their real value are available, with survival times between 6 and 16 months with a mean survival period of 12 months after diagnosis ,7–10 RadMost authors suggest focal spine radiotherapy and chemo-therapy with temozolamide, while others recommend a more aggressive approach with whole-brain irradiation in addition to focal spine irradiation, even if there is no evidence of intracranial dissemination. Others suggest intrathecal administration of interferon-β via an Ommaya reservoir in conjunction with cranio-spinal irradiation ,7.MRI is considered the gold standard imaging modality to diagnose intramedullary tumours ,14, and To the best of our knowledge, only 16 cases of spinal GBM involving the conus medullaris have been previously reported –19,20–24In conclusion, primary spinal GBM is an extremely rare entity. Despite aggressive treatment with radical surgery, radiotherapy and chemotherapy, this disease progresses rapidly with a poor prognosis and a short survival time. We advocate an aggressive management of the different complications as they arise  to extend the patient’s survival as long as possible with the best quality of life. Improvement of current modes of treatment and new treatment options  are required to improve survival and ensure better quality of life.