1/3. Intra-sellar salivary gland-like pleomorphic adenoma arising within the wall of a Rathke's cleft cyst.Salivary gland rests occur in the posterior lobe of the pituitary gland near or often communicating with the Rathke's cleft or its cystic subdivisions, and are usually incidental autopsy findings. They are attributed to the oropharyngeal development of the Rathke's pouch and may rarely give rise to salivary gland-like tumors in the sella. We present a pleomorphic adenoma, a rare tumor of the sellar region, that has not been previously recognized in association with Rathke's cleft cyst. It occurred in a 44-year-old man who presented with hypopituitarism and reduced vision. magnetic resonance imaging showed a sellar mass with suprasellar extension which was totally removed. It consisted of segments of a cyst wall lined by focally ciliated columnar of cuboid epithelium containing goblet cells. An eosinophilic granular material with cholesterol clefts represented the contents of the cyst. Within its wall there was a tumor with ductal structures and non-ductal varied cellular components including hypercellular areas of spindle and ovoid cells forming interlacing fascicles. Individual cells appeared to float in abundant mucinous material. The appearances were those of a salivary gland pleomorphic adenoma arising within the wall of a Rathke's cleft cyst. The myoepithelial nature of non-ductal tumor cells was confirmed with immunocytochemistry. The existence of seromucous glands communicating with the Rathke's cleft remnants, explains the concomitant occurrence of the tumor and the cyst. This rare neoplasm from salivary gland rest should be considered in the differential diagnosis of unusual sellar and suprasellar tumors.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/3. Giant supratentorial enterogenous cyst: report of a case, literature review, and discussion of pathogenesis.OBJECTIVE AND IMPORTANCE: To describe a histologically well-documented adult case of a giant supratentorial enterogenous cyst (EC). Fewer than 15 cases of supratentorial ECs are on record: 8 associated with the brain hemispheres or the overlying meninges, 4 with the sellar region, and 2 with the optic nerve. CLINICAL PRESENTATION: A 31-year-old woman complained of long-standing mild left brachial and crural motor deficit precipitated by headache and signs of intracranial hypertension. magnetic resonance imaging revealed a huge cyst overlying the frontoparietal brain. INTERVENTION: Symptoms were relieved by evacuation of the cyst content by means of a Rickam's reservoir, and the lesion was subsequently removed in toto. Histological and immunohistochemical examination of the cyst wall clearly established the enterogenous nature of its epithelium. Follow-up for up to 2 years after intervention showed no sign of recurrence, and symptoms, including treatment-resistant seizures in the postoperative period, have entirely subsided. CONCLUSION: Supratentorial ECs, distinctly rare in adult patients, may in some cases present as giant lesions. Total removal seems to be curative once careful examination has eliminated the possibility of a metastasis from an unknown primary. A correct histological diagnosis is important because, in contrast to other benign cysts of similar location and size, ECs may be prone to intraoperative dissemination.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/3. teratoma or enterogenous cyst? The histopathological and clinical dilemma in co-existing occult neural tube dysraphism.BACKGROUND: Better understanding of embryology, histopathology and genetics of dysraphic conditions have lead to an expansion of this concept to entities with a similar microscopic appearance (e.g. enterogenous cysts, colloid cyst of the III-rd ventricle) or rated among neoplasms (e.g. mature teratoma), creating a certain conceptual confusion. Currently the diagnosis of "teratoma" is being substituted by "enterogenous cyst" or "teratomatous cyst". AIM OF paper: Clarification of concepts in this field and presentation of the experience of the Department of neurosurgery of the Children's Memorial health Institute associated therewith. MATERIAL AND METHOD: Since January 1990 through April 2005 we had treated 7 children with the final diagnosis of "an enterogenous cyst". The mean age of the children was 10.5 years and the mean follow-up time was 4.1 years. The study was performed by a retrospective analysis of medical records, imaging studies and histological preparations. RESULTS: the study group included: 3 cases of type I enterogenous cyst, 1 case of type II cyst and 3 cases of type III cyst (in 2 cases a former diagnosis of "mature teratoma" has been revised). Within this follow-up time, a good outcome was obtained in 3 cases, moderate disability - in 3 cases and severe disability - in 1 case. CONCLUSIONS: (1) Enterogenous cyst often co-exists with other dysraphic features; (2) Clinical signs of an enterogenous cyst are non-typical and depend on location of the lesion. The presence of secreting gastric mucosa may lead to chemical myelitis; (3) Severity of the postoperative neurological deficit is due to the developmental nature of the lesion and common vascular supply; (4) Late results of treatment are satisfactory, provided the lesion is excised radically; (5) Enterogenous cyst may be diagnosed in the case of a tumor composed of tissues originating from 1, 2 or 3 embryonic layers coexisting with dysraphic stigmata, congenital vertebral abnormalities or a mediastinal tumor of the same type. Types II and III of enterogenous cyst were historically diagnosed as "adult teratoma".- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |