1/97. Immature teratoma originating from the pituitary gland: case report.OBJECTIVE AND IMPORTANCE: Recently, it has been suggested that the primary site of suprasellar germinomas is the neurohypophysis, but nongerminomatous germ cell tumors originating from the pituitary gland have been rarely reported. CLINICAL PRESENTATION: A 27-year-old man presented with panhypopituitarism, bitemporal hemianopsia, and mild right oculomotor nerve paralysis. diabetes insipidus was not observed. The tumor was shown by magnetic resonance imaging to extend and invade bilateral cavernous sinuses, sellar and clival dura mater, and the sphenoid sinus mucosa. INTERVENTION: Transsphenoidal removal yielded a diagnosis of immature teratoma. serum alpha-fetoprotein was prominently elevated. Magnetic resonance imaging and surgical findings of the superiorly displaced residual pituitary gland strongly suggest the pituitary origin of this rare tumor. CONCLUSION: In contrast to the neurohypophyseal germinomas, nongerminomatous malignant germ cell tumors originating from the pituitary gland tend not to be associated with diabetes insipidus and thus mimic adenomas. Evaluation of the tumor markers is necessary in young patients with cavernous sinus syndrome and invasive pituitary tumors with heterogeneous intensity revealed by magnetic resonance imaging.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/97. 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 = 1.5183541776236keywords = nerve, organ (Clic here for more details about this article) |
3/97. Intracavernous teratoma in a school-aged child.An eight-year-old boy presented with left eye pain, photophobia, proptosis, third nerve paresis and decreased visual acuity. magnetic resonance imaging revealed a nonenhancing mass filling the cavernous sinus. Using an extradural fronto-orbitozygomatic approach, the cavernous sinus was approached laterally, and a teratoma was removed from within the cavernous sinus. This is the first case of a truly intracavernous teratoma in a child and the fourth case of a teratoma reported in the cavernous sinus region overall. This report outlines the diagnosis and treatment of this unusual cavernous sinus tumor.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
4/97. Anaesthetic management of an infant with anterior mediastinal mass.A substantial mediastinal mass in a small infant can create a dilemma regarding the safest mode of airway management. To ensure safety at all times, we adopted one lung ventilation for fear of compression of the carina and/or both main bronchi. Anaesthesia was maintained at a very light plane by the use of local nerve blocks to secure the airway and epidural analgesia for surgery until the tumour was mobilized.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
5/97. Congenital orbital teratoma.A case of congenital orbital teratoma is described in which there was no organized eye only microscopic evidence of ocular tissues within the disorganized teratoma. A baby boy presented at birth with a 10-x-8-cm mass extruding from the left orbit. magnetic resonance imaging (MRI) showed a mixed cystic-solid orbital mass containing areas of calcification and deforming the bony orbit around its margins. There was no organized eye and no intracranial extension. The eye was removed with reconstruction of the eyelids. Histopathology showed representation from all three germ cell layers consistent with a teratoma. There was no organized eye, but some disorganized ocular structures within the teratoma. Follow-up has been uneventful. Neonatologists and pediatricians should be aware of the possible diagnoses in a newborn presenting with an orbital mass, so that early definitive surgery can be performed with preservation of the globe where possible.- - - - - - - - - - ranking = 2.591770888118keywords = organ (Clic here for more details about this article) |
6/97. Benign mediastinal teratoma complicated by cardiac tamponade: report of a case.We present herein the case of a 48-year-old woman with a benign mediastinal teratoma that had been followed up for 3 years, who developed acute cardiac tamponade. The patient had initially undergone an exploratory sternotomy, at which time the tumor was histologically diagnosed as a benign mature teratoma that could not be resected due to its severe, wide adhesion to the surrounding organs. However, following the development of cardiac tamponade, both sternotomy and right intercostal thoracotomy were employed, and the tumor could be excised with cardiopulmonary bypass standby. High levels of amylase and carbohydrate antigen 19-9 were revealed in the pericardiac effusion fluid. The mRNA expression of inflammatory cytokines including interleukin-1 (IL-1), IL-6, and IL-8 in the tumor tissue was also demonstrated by a reverse transcriptase-polymerase chain reaction analysis. This case illustrates the ultimate natural course of benign mediastinal teratoma and emphasizes the importance of early surgical excision, even when this tumor is asymptomatic.- - - - - - - - - - ranking = 0.51835417762361keywords = organ (Clic here for more details about this article) |
7/97. Congenital nasopharyngeal teratoma (epignathus).The case report of a congenital nasopharyngeal teratoma presented as an intra-amniotic sac mass was described. Histologically, the tumor was composed of highly differentiated tissue derived from three germ layers. Those tissues were partially developed as infant organs with a central core made up by bone. Haphazardly arrangement of such tissues with disorganized sketal axis helped in differentiating the mass from an asymmetric twins. The evidence of tumor attachment was seen at the retro-uvular region.- - - - - - - - - - ranking = 1.0367083552472keywords = organ (Clic here for more details about this article) |
8/97. Extraneural metastasis of pineal tumor.The case of an eight-year old male with a pineal tumor is reported, in which metastases occurred to extraneural organs. The pineal tumor consisted of portions of benign teratoma, malignant ependymoma and embryonal carcinoma. Only the embryonal carcinoma metastasized. The intracanial and spinal subarachnoid space alos was invaded by disseminated embryonal carcinoma. There have been seven other similar cases reported. All patients were males from five to 34 years of age.- - - - - - - - - - ranking = 0.51835417762361keywords = organ (Clic here for more details about this article) |
9/97. Spinal teratoma: is there a place for adjuvant treatment? Two cases and a review of the literature.Spinal teratomas are rare intradural spinal tumours, commonly of benign histological profile. The main treatment remains surgical excision which often is incomplete as the tumour is frequently intimately related to surrounding nerve roots and adjacent spinal cord. The value of adjuvant treatment in the form of radiotherapy has not been clearly identified. We report two patients with spinal teratomas in the lower thoracic and conus region, who were treated with surgical excision and did not receive radiotherapy. One of the tumours recurred at 38 months following surgical excision and required a second operation. We review in addition another 20 patients reported in the literature with such tumours. With a mean follow-up of 25 months a local recurrence rate of 10% was observed, the same in both total and subtotal excision groups. No deaths were reported. Analysis of cumulative survival from surgery to treatment failure demonstrated that recurrence occurs late, verifying a slow biological progression of this tumour type. Although no reliable information of the effect of radiotherapy can be obtained, it is difficult to see how radiotherapy could have an impact on such a slow growing tumour. Initial treatment of choice remains surgical excision. If the teratoma shows any malignant histological features or germ cell elements radiotherapy should be employed after surgical excision, even if apparently totally excised. In the presence of benign histology it would seem appropriate to avoid radiotherapy after initial surgery. In case of tumour recurrence or progression to explore the possibility of further surgery first, before considering radiotherapy which even then may have doubtful efficacy.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
10/97. Ectopic cerebellum presenting as a suprasellar mass in infancy: implications for cerebellar development.A 4-month-old infant with a history of nasopharyngeal teratoma developed progressive optic neuropathy. neuroimaging studies demonstrated a solid, isointense, suprasellar mass impinging on optic nerves and chiasm superiorly. The mass was subtotally resected. No attachment of the mass to brain stem or cerebellar structures was noted. Histological examination identified the tissue as developing cerebellum. The cytoarchitecture and cellular constituents of the cerebellar tissue were only slightly distorted. All cerebellar cortical constituents were arranged anatomically, and an external granular cell layer was present superficially. The latter was actively proliferating and appropriately cellular for the infant's age. The clinical presentation of ectopic cerebellum as a suprasellar mass in an infant is highly unusual. Moreover, this example illustrates the ability of cerebellar tissue to mature appropriately in a site distant from the posterior fossa, removed from ascending and descending afferent projections. Intrinsic signaling mechanisms appear sufficient to direct histogenesis in developing cerebellar cortex.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
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