1/22. An unusual case of the complete Currarino triad: case report, discussion of the literature and the embryogenic implications.OBJECTIVE AND IMPORTANCE: We present and illustrate an unusual case of the complete familial Currarino triad (an association between a bony sacral defect, a presacral mass, and an anorectal malformation) in which the teratoma arose from the conus medullaris and contained mature neurons, glia, and branching ependymal canals that were in communication with a terminal syrinx. The embryogenic implications are discussed. CLINICAL PRESENTATION: The patient was a term neonate when discovered to have imperforate anus. Further workup revealed lumbosacral dysraphism with a presacral mass, a rectovaginal fistula, and a single pelvic kidney. The family pedigree revealed a familial transmission pattern; the patient had a second cousin with anal atresia and a first cousin with similar sacral anomalies. The motor level was L4 with trace L5, and there was absent sensation in the sacral dermatomes. INTERVENTION: A diverting colostomy was performed on Day 14, and the infant returned at 3 months of age to undergo near-total resection through the previous abdominal approach. Only a subtotal resection was possible because the mass arose from the low-lying conus and was firmly adherent to the sacral nerve roots and iliac vessel. Follow-up magnetic resonance imaging performed 18 months after surgery revealed that the residual tumor had not progressed. CONCLUSION: Complete Currarino triad is rare and is familial in half of the cases. The special features of the tumor in our case were the presence of mature neurons with ependymal canals and its origin from the conus. The possible embryogenesis may provide evidence that the caudal notochord is important for organized secondary neurulation.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/22. Radiofrequency ablation of human fetal sacrococcygeal teratoma.OBJECTIVE: Fetuses with solid, highly vascularized sacrococcygeal teratomas can die as a result of the vascular steal syndrome. This is the first report in which a percutaneous technique, radiofrequency ablation, was used to interrupt blood flow to a sacrococcygeal teratoma in 4 human fetuses. STUDY DESIGN: A radiofrequency ablation probe was percutaneously inserted into the fetal tumor under ultrasonographic guidance. In 2 fetuses a significant portion of the tumor mass was ablated, whereas in the other 2 fetuses only the major feeding vessels were targeted. RESULTS: Two infants were delivered at 28 and 31 weeks' gestation, respectively, and are doing well. In 2 other cases hemorrhage into the tumor led to an unfavorable fetal outcome. CONCLUSION: Ablation of a majority of the tumor tissue in sacrococcygeal teratoma is not necessary and proved fatal in two instances. Targeted ablation of the feeding tumor vessels diminishes blood flow sufficiently to reverse high-output fetal heart failure.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
3/22. Thermocoagulation of fetal sacrococcygeal teratoma.Fetal sacrococcygeal teratoma can lead to a high output cardiac failure resulting in hydrops fetalis. One of the prenatal therapeutic options is to occlude the feeding vessels by radiofrequency ablation. We present a case of fetal sacrococcygeal teratoma diagnosed at 13 weeks of gestation. The tumour increased in size more than 100 fold over 5 weeks causing polyhydramnios and cardiac and placental enlargement. Thermocoagulation was performed at 18 weeks' gestation by passing an insulated electric wire through an 18 gauge needle placed close to the feeding vessels of the tumour at its neck. Blood supply to the tumour was successfully reduced. However, fetal death was diagnosed 2 days after the procedure. We speculate that it may be safer to limit the extent of coagulation in one attempt but to repeat the procedure at a later stage when necessary.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
4/22. Mediastinal immature teratoma with yolk sac tumor and myelomonocytic leukemia associated with Klinefelter's syndrome.A 14-year-old male presenting with a short history of right subclavicular chest pain was found to have a mediastinal tumor. Hematologic investigations and bone marrow examination showed features of myelomonocytic leukemia. The mediastinal tumor was excised, but the surgery was complicated by massive hemorrhage. The patient's condition deteriorated postoperatively and he died a week later. The histology of the mediastinal tumor showed the typical features of an immature teratoma with a yolk sac tumor. A prominent infiltrate of leukemic blast cells was present within blood vessels and in close proximity to the yolk sac component. The karyotypic analysis of leukemic cells isolated and cultured from the bone marrow showed 50XXY, 8, 21, iso G-group marker chromosome karyotype.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/22. A multidisciplinary team approach for management of a giant congenital cervical teratoma.Congenital cervical teratomas are associated with a high rate of perinatal mortality due to airway obstruction. We describe a multidisciplinary management of a neonate with prenatal diagnosis of giant cervical teratoma. An 'operation on placenta support' (OOPS) technique was carried out during delivery, and intubation was successfully performed with no perinatal anoxic damage. Postnatal computed tomography and angiography showed a huge teratoma covering both sides of the neck with agenesis of the big blood vessels on the left side. A rapidly developing third space phenomenon and deterioration in the general status of the neonate, required early surgical intervention. During surgical excision, the left carotid artery and internal jugular vein, the left lobe of the thyroid gland and the left recurrent laryngeal nerve were not detected. The left vagus, accessory and hypoglossal nerves were positioned between the skin and the tumor, at a distance from their normal anatomical location. Pathologic examination confirmed the diagnosis indicating immature teratoma with no signs of malignancy. The postoperative period was complicated by neurological deterioration, pharyngo-cutaneous fistula and paresis of the left hypoglossal nerve. However, all the symptoms resolved spontaneously. tracheotomy was performed when the baby was 6 weeks old due to paralysis of the left vocal cord and to severe laryngo-tracheomalacia. She was decannulated when she was 3 years old. Today, she is suffering only from dysphonia. This report confirms the efficacy of a multidisciplinary team-approach and the usefulness of the OOPS technique in prenatally diagnosed cervical masses. It emphasizes the extraordinary characteristics of this case, mainly the development of a third space phenomenon and the unusual surgical findings.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/22. Use of activated recombinant factor vii (NovoSeven) during neurosurgery.Diffuse bleeding during neurosurgical operations is a major complication that can be difficult to control. In extreme circumstances, it may be necessary to clamp cerebral blood vessels or resect additional tissue. Both of these procedures can result in brain damage and, ultimately, in disability for the patient. We report on the use of recombinant activated blood coagulation factor vii (rFVIIa) to control bleeding during neurosurgical procedures in three patients undergoing neurosurgery for brain tumors who received intravenous rFVIIa 4.8 mg (NovoSeven, NovoNordisk, Copenhagen, denmark) for diffuse intracranial bleeding after conventional hemostatic techniques had been exhausted. All patients (one man and two women, age range 23-40 years) were otherwise somatically healthy with a mean weight of approximately 50 kg. Bleeding ceased 12 to 20 minutes after administration of rFVIIa. All three patients recovered with no further bleeding. In our initial experience, rFVIIa appears to be efficacious for terminating bleeding during neurosurgical procedures. The use of rFVIIa made it unnecessary to resort to procedures carrying a risk of long-term sequelae.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/22. Mature ovarian cystic teratoma with a highly differentiated homunculus: a case report.BACKGROUND: Mature ovarian cystic teratomas, which are commonly observed benign ovarian tumors, consist of ectodermal, mesodermal, and endodermal components that are generally disorganized. In this report, we document a case in which the solid portion of an ovarian teratoma demonstrated considerable differentiation, forming a doll-like structure. CASE: A 25-year-old virginal Japanese woman underwent surgery for an ovarian tumor that was diagnosed as a mature teratoma. A solid mass within the tumor was found to have a head, trunk, and extremities. Consequently, this mass was diagnosed as a mature fetiform teratoma (homunculus). brain, eye, spinal nerve, ear, teeth, thyroid gland, bone, bone marrow, gut, trachea, blood vessels, and phallic cavernous tissue were confirmed microscopically. Distinctive features were the clear anterior-posterior, ventral-dorsal, and left-right axes, with a spatially well-organized arrangement of the organs. An eye was located on the front of the head, a spinal nerve lay dorsal to the spinal bones, the thyroid gland was anterior to the trachea, and the gut was deep inside the trunk. CONCLUSIONS: These findings indicate that the information necessary for organization of the body plan may be conserved and transmitted, even with parthenogenesis. Mature cystic teratomas of the ovary are mostly benign and do not always attract detailed attention. However, precise analyses of such tumors may significantly enhance our understanding of both parthenogenetic and normal human development.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/22. Giant, highly vascular sacrococcygeal teratoma: report of its excision using the ligasure vessel sealing system.Reports about giant, highly vascular sacrococcygeal teratoma (GHV-SCT) are uncommon. A boy with a prenatally diagnosed GHV-SCT was born at 34 weeks' gestation weighing 3,716 g. At birth, the GHV-SCT was 20 x 17 x 14 cm. After initial devascularization using an aortic snare, excision of the GHV-SCT was started using monopolar diathermy, but hemorrhage from cutaneous collateral vessels was difficult to control, and individual ligation of vessels was extremely time consuming. The authors then used the LigaSure (LS) vessel sealing system (Valleylab, Boulder, CO) to control intraoperative hemorrhage. hemostasis was achieved easily with LS, without any need for vessel ligation; the operative field was dry and the efficiency of excision enhanced. Total operating time was 255 minutes, but excision of the GHV-SCT itself took only 16 minutes. The GHV-SCT weighed 1,208 g or 325.1 g/kg body weight. Total blood loss was 77 mL or 20.7 mL/kg body weight, which was minimal compared with other cases in the literature. At the last follow-up examination, 7 months after surgery, he remains well with no evidence of recurrence. LS was extremely useful for controlling intraoperative hemorrhage in this case, and we strongly recommend its selective use for excising highly vascular SCT.- - - - - - - - - - ranking = 8keywords = vessel (Clic here for more details about this article) |
9/22. Placental site trophoblastic tumor of the mediastinum.choriocarcinoma has been described as the most frequent subtype of mediastinal germ cell tumors showing trophoblastic differentiation. We report a unique case of a placental site trophoblastic tumor, which developed in the mediastinum of a 14-year-old boy 2 years after the resection of a mature teratoma. The recurrent tumor was composed of a grossly hemorrhagic and necrotic mass. Histologically, diffusely infiltrating large polygonal cells with focal nodular growth and a teratomatous part containing mature intestinal, respiratory, and squamous epithelium with adjacent cutaneous adnexal structures were found. The typical morphologic features included vessel wall infiltration by the neoplastic cells with fibrinoid deposits and geographic necroses within the tumor masses. Characteristic diffuse positivity for melanoma cell adhesion molecule and human leucocyte antigen G was found on immunohistochemical investigation, confirming the diagnosis of placental site trophoblastic tumor. The patient died 1 year later after polychemotherapy. The outcome of this rare tumor is similar to the reported poor clinical outcome in patients with mediastinal choriocarcinomas.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/22. Preoperative angiography with embolization and radiofrequency ablation as novel adjuncts to safe surgical resection of a large, vascular sacrococcygeal teratoma.Sacrococcygeal teratomas (SCTs) can present a challenging problem and can be associated with significant perinatal morbidity and mortality. A female child was born at 36 weeks' gestation with a large, vascular Type 1 SCT originally identified by prenatal ultrasound. A CT scan showed two large feeding vessels arising from both internal iliac arteries that were successfully embolized during angiography. A radiofrequency probe was then used to ablate a zone between normal tissue and the tumor. The SCT was subsequently surgically excised with minimal blood loss. This case is presented to illustrate two useful and previously unreported postnatal adjuncts to the surgical treatment of massive, hypervascular sacrococcygeal tumors.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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