Histopathological features of malignant craniopharyngioma: Case report and literature review.

Malignant transformation in craniopharyngiomas is a very uncommon event and scarcely mentioned in the World Health Organization (WHO) Classification of Tumors of the Central Nervous System (CNS). So far, there are only 34 reported cases. AIMS: We report an additional case in a 63-year-old woman who was diagnosed with craniopharyngioma 47 years ago. We reviewed the literature in order to define the histological features of malignant craniopharyngioma and its overlap with odontogenic tumors. RESULTS: Our case presented morphology of mixed odontogenic ghost cell/ameloblastic carcinoma. Analyzing all reported cases, 18 of them presented malignization as squamous cell carcinoma (SCC), 1 as odontogenic ghost cell carcinoma, 2 as ameloblastic carcinoma, and 10 cases were mentioned just as malignant craniopharyngiomas. CONCLUSION: We concluded that SCC represented only half of the malignant cases, while the morphology of ~ 11% of them was comparable with ameloblastic or odontogenic ghost cell carcinomas and 28% lacked a specific histological diagnosis. Most cases were fatal, which makes it necessary to include the entity of malignant craniopharyngioma in the WHO Classification of Tumors of the CNS as a high-grade tumor defining its histological variability.

Hub genes for early diagnosis and therapy of adamantinomatous craniopharyngioma.

BACKGROUND: Adamantinomatous craniopharyngioma (ACP) is a subtype of craniopharyngioma, a neoplastic disease with a benign pathological phenotype but a poor prognosis in the sellar region. The disease has been considered the most common congenital tumor in the skull. Therefore, this article aims to identify hub genes that might serve as genetic markers of diagnosis, treatment, and prognosis of ACP. METHODS: The procedure of this research includes the acquisition of public data, identification and functional annotation of differentially expressed genes (DEGs), construction and analysis of protein-protein interaction network, and the mining and analysis of hub genes by Spearman-rho test, multivariable linear regression, and receiver operator characteristic curve analysis. Quantitative real-time polymerase chain reaction was used to detect the level of mRNA of relative genes. RESULTS: Among 2 datasets, a total of 703 DEGs were identified, mainly enriched in chemical synaptic transmission, cell adhesion, odontogenesis of the dentin-containing tooth, cell junction, extracellular region, extracellular space, structural molecule activity, and structural constituent of cytoskeleton. The protein-protein interaction network was composed of 4379 edges and 589 nodes. Its significant module had 10 hub genes, and SYN1, SYP, and GRIA2 were significantly down-regulated with ACP. CONCLUSION: In a word, we find out the DEGs between ACP patients and standard samples, which are likely to play an essential role in the development of ACP. At the same time, these DEGs are of great value in tumors' diagnosis and targeted therapy and could even be mined as biological molecular targets for diagnosing and treating ACP patients.

Ghost cell odontogenic carcinoma.

Ghost cell odontogenic carcinoma (GCOC) is the malignant counterpart of calcifying cystic odontogenic tumour and dentinogenic ghost cell tumour. This is the case of a middle-aged male who presented with a slow-growing maxillary tumour. He was asymptomatic until pain symptoms developed prior to initial presentation. The excised tumour was diagnosed as a ghost cell odontogenic carcinoma. More case reports are needed for further understanding of this rare malignant odontogenic tumour.

Granulomatous hypophysitis caused by a ruptured intrasellar Rathke's cleft cyst: report of a case and review of the literature.

OBJECTIVE AND IMPORTANCE: Ruptured Rathke's cleft cyst is a rare cause of giant cell granulomatous hypophysitis. Chronic inflammatory reaction is caused by extravased cyst content into the adjacent gland. We provide a demonstration that mucins produced by cells lining the cyst wall caused the granulomatous giant cell reaction. CLINICAL PRESENTATION: A 37-year-old nonpregnant woman presented with a 3-year-history of headache and amenorrhea. She had experienced normal sexual maturation, and her medical history was unremarkable. Radiologically, the lesion appeared as an intrasellar mass with a cystic component indistinguishable from a pituitary adenoma with cystic degeneration. TECHNIQUE: The patient underwent a transsphenoidal approach. Because no demarcation between normal and affected tissue was evident at surgery, the lesion and residual pituitary were radically removed. Tissue was studied using routine hematoxylin and eosin and histochemical stainings for mucins and immunocytochemical techniques. CONCLUSION: This study demonstrates that mucins that had spilled out from the cyst caused the granulomatous reaction. Using computed tomography, magnetic resonance imaging, and gross inspection, distinction between granulomatous hypophysitis and pituitary adenoma was virtually impossible. Nevertheless, a granulomatous reaction of the pituitary gland should be suspected in a case of a sellar mass having a cystic area. In such cases, intraoperatory diagnosis on frozen sections is mandatory because adoption of a conservative treatment allows preservation of the gland.

Infrasellar craniopharyngioma presenting as epistaxis, excised via Denker's medial maxillectomy approach.

Infrasellar craniopharyngioma is an uncommon neoplasm. Rarely, it presents with epistaxis. To date, 39 cases of infrasellar craniopharyngioma have been reported in the world literature. Involvement of the maxillary sinus has only been reported once. We report a second case exclusively involving the nasal cavity, ethmoid sinus and maxillary sinus. Excision was performed using a Denker's medial maxillectomy approach.

[Sphenoido-nasopharyngeal craniopharyngioma. A case with radical excision by Le Fort I-type maxillotomy]. / Crâniopharyngiome sphénoïdo-naso-pharyngé. Un cas avec exérèse radicale par maxillotomie de Le Fort I.

A case of infra-sellar (sphenoidal nasopharyngeal) craniopharyngioma is reported. A 16 year-old girl presented with progressive nasal obstruction. CT and MR images showed a heterogeneous tumor originating from the sphenoid bone with no intra-cranial or supra-sellar extension. On a biopsy specimen, the histologic examination was consistent with a craniopharyngioma. The tumor was successfully removed via an inferior route using a Le Fort I maxillotomy. Plates and screws were used to achieve osteosynthesis. The post-operative course was uneventful and there was no residual tumor on post-operative CT. Craniopharyngiomas with strictly infra-sellar development are very rare. Up to now, 19 cases have been reported in the literature. This condition likely results from growth of embryonic remnants lying along the craniopharyngeal duct. Several surgical approaches have been proposed, most of those giving poor exposure to the clival and lateral tumorous extensions. Of particular interest in the authors' experience was the use of a Le Fort I maxillotomy which provided optimal exposure to the skull-base and facilitated radical excision. CT and MR studies have proved of upmost importance in delineating the area of bone to be removed at operation.

[Cholesterol granuloma at the sella region: a new method of the differential diagnosis of craniopharyngioma]. / A sella-tájék koleszterin-granulomája: új alternatíva a craniopharyngeoma elkülönító kórisméjében?

Cholesterol-granuloma is a pseudotumoral mass that is believed to enlarge by a self-perpetuating sequence of repeated hemorrhages and reparative tissue reaction. Albeit an almost ubiquitous phenomenon throughout the body, cholesterol-granuloma has recently been appreciated as a distinctive lesion mimicking or associated with craniopharyngiomas. Upon review of a surgical series of 15 purported craniopharyngiomas, the authors identified 3 such occurrences. All were characterized by a predominance of slit-like cholesterol clefts with multi-nucleated giant cells embedded in a fibrotic stroma permeated with lipid laden macrophages, lymphocytes, as well as organizing hemorrhage. Non-craniopharyngioma specific cuboidal epithelium was present in one case. The mean age of patients--all males--with cholesterol-granuloma was 26 years, and all but one had an intrasellar tumor component. Clinical symptoms referrable to hypopituitarism predominated. At variance with the above, patients with adamantinomatous or papillary craniopharyngiomas were 23.5 and 46 years old, respectively, and presented with neurological deficits or ones due to hypothalamic involvement by their tumors. With marginal central nervous tissue present in 53 percent of the specimens, 75 percent of adamantinomatous craniopharyngiomas, but only 12 percent of cholesterol-granulomas showed invasive growth. At present cholesterol-granulomas are conceived as a clinicopathologically distinctive lesion of uncertain origin. They most probably represent a clinically relevant entity in the ontogenesis of adamantinomatous craniopharyngiomas with predisposing factors yet to be elucidated.