[Pituitary tumor detected prenatally]. / Tumor hipofisiario detectado prenatalmente.

INTRODUCTION: Brain tumors are present in 2.9 per 100,000 newborn. Craniopharyngioma is a benign and slow growing brain tumor, frequently localized in the sellar and suprasellar region. There are few reports of pituitary tumor detected prenatally. CASE REPORT: We report a neonate with a craniopharyngioma detected prenatally as a pituitary tumor. In a 23 year old mother, second gestation, with no important history, was detected a sellar tumor at 31 gestation weeks, the obstetric ultrasound reported a suprasellar tumor of 2 per 3 cm diameter. Pregnancy ended in a vaginal delivery at 39 weeks, and obtained a 3.9 kg female, with cephalic diameter of 37.5 cm, the Apgar score was 8-9 at 1st and 5th minutes. In early neonatal period was scanned and confirmed a 3.2/2.3/2.9 cm suprasellar tumor with calcium deposits. The Paediatric Oncology department suggested a surgery and was realized a craniotomy at 3rd week of age. The surgery allowed to obtain 30% of the tumor and confirmed by histology craniopharyngioma. Patient had favourable evolution and was discharged at 3 months of age. CONCLUSIONS: We report a neonate in who was detected by prenatal ultrasound the presence of a suprasellar solid tumor, scan and magnetic resonance images in neonatal period defined its size and location and a craniopharyngioma was confirmed by histology. Patient had a satisfactory postsurgical evolution and was discharged at 3 months of age.

Anatomic relations of the arachnoidea around the pituitary stalk: relevance for surgical removal of craniopharyngiomas.

PURPOSE: The growth pattern of craniopharyngiomas (CP) is yet to be understood due to challenges arising from the diversity of morphological features that exist. This in turn has had implications on the development of safe surgical strategies for management of these lesions. The aim of this study is to propose a morphological classification of CP based on their tumor-membrane relationship. It is hoped that this will contribute to better understanding of CP morphology and prediction of the intraoperative classification. METHODS: Histological techniques were used to study eight fetuses. Following Masson staining, the membranes around the pituitary stalk were observed under microscope. Pre-operative MRI and intraoperative images of 195 patients with CP were also analyzed. FINDINGS: The arachnoidal sleeve around the pituitary stalk (ASPS) was noted to be comprised of a compact fibrous component and a related loose trabecular component. The pituitary stalk was divided into four segments in accordance with the folds of the ASPS. Correspondingly, the growth of CPs was divided into four basic patterns-infra-diaphragmatic (ID), extra-arachnoidal (EA), intra-arachnoidal (IA) and sub-arachnoidal (SA) growth. The IA growth pattern can be further subdivided into two subtypes-namely, IA1 (with tumor growing within the fibrous component of the ASPS) and IA2 (with tumor growing within the trabecular component). This method of topographical division can be used to understand the growth of CP-infra-diaphragmatic CP show growth pattern ID or ID together with EA. Suprasellar CP can show an extra-ventricular growth pattern (EA or IA2), an extra- and intra-ventricular (IA2 + SA) growth pattern, a trans-infundibular growth pattern (ID + IA1 + SA) and an infundibulo-tuberal growth pattern (SA or SA + IA1). There is a statistically significant difference between CP growth patterns in children and adults. A predominance of ID growth is noted in children while adults tend to show a pattern of predominantly Extra-ventricular (EV) growth. CONCLUSION: Our proposed classification details the relationship of the surrounding structures to CPs and purports to predict and identify the intraoperative anatomical stratification. It also attempts to help predict the growth patterns of these tumors. A knowledge of the intimate relations of the tumor and its key surrounding structures allows for safe surgical removal.

Craniopharyngioma: modern concepts in pathogenesis and treatment.

PURPOSE OF REVIEW: Craniopharyngioma is a benign tumour. Its tendency to recur after excision and the high surgical risk due to involvement of the most vital structures of the brain mean that alternatives to radical surgery should be considered, namely limited surgical procedures followed by radiotherapy. Since both options present inherent risks, optimal craniopharyngioma treatment remains controversial. This paper aims to critically review the recent literature on craniopharyngioma. RECENT FINDINGS: The management of children with craniopharyngioma has benefited from concerted efforts by national and international groups to improve outcome and reduce morbidity. From the current literature it is evident that there is a trend to better integrate all treatment modalities available, tailoring therapies to specific risk factors. Modern imaging and new surgical and radiotherapy techniques are increasing the possibility of cure. Biological markers are under investigation and this will increase our knowledge on craniopharyngioma. SUMMARY: Studies on treatment, biology and pathogenesis of craniopharyngioma, available in the current literature, grew considerably in the last year. Although a consensus has not been reached on all aspects of this complex disease, there is a trend in the field to move quickly towards a better understanding of the disease to improve treatment strategies and to produce clinical cooperative trials.

The craniopharyngioma.

The craniopharyngioma is one of the most common destructive lesions of the hypothalamus and pituitary gland. It still remains one of the most difficult tumors to treat effectively since complete resection is often impossible and is associated with frequent recurrence. Current therapy is multimodal and focuses on a combination of surgical decompression, medical treatment, as well as stereotactic radiosurgery. This chapter reviews the embryology, neuroanatomy, current treatment strategies, clinical features and the several surgical approaches to its treatment.

Suprasellar and infrasellar craniopharyngioma with a persistent craniopharyngeal canal: case report and review of the literature.

Craniopharyngiomas are usually confined to the sellar and suprasellar regions; infrasellar craniopharyngioma is rare. From an embryological point of view, this unusual localisation can be explained by Erdheim's theory that these tumours can arise anywhere along the craniopharyngeal canal (CPC). However, there has been no proof of this theory, because the CPC is usually obliterated during the 12th week of gestation. I present a case of supra- and infrasellar craniopharyngiomas with a persistent CPC. Imaging demonstrated an intimate relationship between the infrasellar tumour and the CPC, supporting Erdheim's view.

Embryonal craniopharyngioma. Case report of the morphogenesis of a craniopharyngioma.

BACKGROUND: Ordinary craniopharyngiomas affecting sellar regions of children and preadolescents are composed of ameloblastomatous and/or squamous constituents. The authors encountered an autopsy case of a stillborn infant with a large craniopharyngioma with unusual manifestations. METHODS: The craniopharyngioma was analyzed using detailed histologic and immunohistochemical techniques. RESULTS: An autopsy revealed a large tumor arising from the pituitary gland with associated severe hydrocephalus. Histologically, the cells of the tumor showed features of ameloblastoma and organoid structures simulating tooth buds and adenohypophyseal primordia in the stage of organogenesis. Cells of the latter were shown to be immunoreactive for chromogranin A and six adenohypophyseal hormones. CONCLUSIONS: Because embryology of the adenohypophysis and the teeth indicated that all of the epithelia in this tumor emerged during the normal development of the stomodeum, it was believed that an "embryonal form of craniopharyngioma" would characterize this organoid neoplasm best. The possible morphogenesis of craniopharyngioma is also discussed.

An entirely suprasellar symptomatic Rathke's cleft cyst: case report.

An entirely suprasellar symptomatic Rathke's cleft cyst in a 21-year-old woman is reported. An unusual feature of this cyst was the fact that the subepithelial tissues were composed of pituitary gland cells. High resolution magnetic resonance imaging is sensitive in the detection of subtle suprasellar abnormalities. We review the literature regarding the embryological pathogenesis of Rathke's cleft cyst, with special reference to the entirely suprasellar type.

Craniopharyngioma presenting as a nasopharyngeal mass: CT and MR findings.

Craniopharyngiomas usually arise in the suprasellar or parasellar region. The occurrence of the tumors in other locations is rare and they have to be distinguished from lesions in the suprasellar or sellar region with unusual extension. Thus far, few craniopharyngiomas of infrasellar origin have been described. We report a case in which the tumor presented as a nasopharyngeal mass and CT and magnetic resonance were used for evaluation. Review of the pertinent embryology and literature is also included.

Pituitary tumors composed of adenohypophysial adenoma and Rathke's cleft cyst elements: a clinicopathological study.

Nine cases of pituitary tumors composed of mixed tissue elements of adenohypophysial adenoma and Rathke's cleft cyst are reported. All cases were associated with hyperprolactinemia and were managed by transsphenoidal adenomectomy with good results. The clinical and pathological features are presented, and embryological pathogenesis for the occurrence of this unique association is discussed in connection with the high incidence of immunoreactive S-100 protein cells identified in tumor tissues. Patients with this kind of pituitary tumor can be managed in the same fashion as patients with other types of pituitary adenoma.

Craniofaringioma: relato de um caso / Craniopharyngioma: a case report

O autor discute aspectos embriológicos, clínicos e tratamento. Relata um caso de cranio faringioma operado e complementado com radioterapia

[One case of atypical craniopharyngioma located in sphenoïdal body and operated first by nasal then by maxillary sinus route (author's transl)]. / Craniopharyngiome ectopique basi-sphénoïdal. Abord par voie basse, naso-septale, puis antro-ethmoïdale.

This case of retro-pharyngeal craniopharyngioma was treated through an unusual combination of approaches first by naso septal then by the maxillary sinus route of Denker. This last exposure provided wide access and permitted a complete removal of this difficult tumor. It is usually meant for cancer of upper maxillary bone structures. In this indication, it is evidently followed by a very bad cosmetic result, but this did not happen with our patient.

[Topgraphical classification of craniopharyngioma]. / Beitrag zur topistischen Einteilung der Kraniopharyngiome

Squamous cell nests of Erdheim have been observed along the lateral blood vessels to the adenohypophysis in 4 cases. Their developmental origin and topographical distribution correlate with the sites in which craniopharyngiomas are found. The theory of dysontogenic origin of cranopharyngiomas is supported by these findings. Metaplasia of cells of the adenohypophysis is not considered to be a likely cause of craniopharyngioma formation.