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According to the World Health Organization classification of pituitary tumors, only tumors with systemic metastasis must be considered as carcinomas. Invasive tumors with multiple recurrences are only classified as aggressive tumors or “atypical adenomas”. To illustrate the problems encountered in the pathological diagnosis of pituitary carcinoma and in patient management, we present two male patients operated on for an aggressive prolactin pituitary adenoma with and without metastasis. In case 1, 5 surgeries, 3 irradiations, increased doses of dopamine agonists, and trials of temozolomide and carboplatine-VP16 failed to control tumor progression and the appearance of metastases which lead to death 16 years after onset. In case 2, based on the initial diagnosis of an aggressive-invasive adenoma that was resistant to dopamine agonists, gamma-Knife irradiation was initially performed on the intra-cavernous remnant. Eight years after onset, the remnant remained stabilized and the plasma PRL normalized under dopamine agonist. From these 2 cases alongside other cases found in the literature, we propose that the association of certain clinical signs (male sex, dopamine-resistant hyperprolactinemia), radiological signs (invasive macro or giant tumor on MRI) and histological signs (angiogenesis, Ki-67 > 3 %, p53 positive, mitoses >2 per high power field, vascular invasion, up-regulation of genes related to invasion and proliferation, and allelic loss of chromosome 11) might suggest aggressiveness and be suspicious of malignancy before the appearance of metastasis. The early detection of an aggressive phenotype of a prolactin pituitary tumor should permit the earlier establishment of the optimum therapeutic strategy associating surgery and radiotherapy to delay or inhibit metastasis.

Von Hippel-Lindau disease is an autosomal dominant disorder involving the development of specific tumours in multiple organs, both benign and malignant. In the CNS, the syndrome is characterized by haemangioblastomas of the retina, spinal cord and brain. We report the case of a 15-year-old boy with the diagnosis of aggressive GH-PRL pituitary macroadenoma and a family history of VHL disease. Pituitary resection was performed, although complete excision of the lesion could not be confirmed by the neurosurgeon. A control MRI was done 6 months after surgery and the pituitary lesion was similar to the presurgical image. A second operation allowed partial resection of the tumour followed by targeted radiotherapy. Pituitary adenomas are rare benign tumours in children with macroadenomas observed mainly in boys. These tumours in adolescents often occur in a familial setting or in the context of known genetic defects. Angiogenesis is an important feature of pituitary adenomas and a possible inhibitory role of pVHL in pituitary angiogenesis has been suggested. This GH-PRL pituitary macroadenoma with a VHL mutation might be of particular aggressiveness. Pituitary adenomas are not classically described in VHL syndrome and the medical community should be alerted to its rare occurrence in this location.

Angiolipomas are rare tumors of the CNS that most frequently develop in the orbit, the cavernous space, and the epidural space of the spine. The authors report the case of a patient who presented with an angiolipoma of the cavernous space. Using data from the published literature and an experimental anatomical approach, they demonstrate that the cavernous space contains adipose tissue. Consequently, they suggest that angiolipomas constitute a characteristic tumor illustrating the interperiosteo-dural concept. The authors report the clinical, radiological, and histological data of a patient who presented with a tumor of the cavernous space. In addition, they prepared 2 encephalic extremities (4 cavernous spaces) using a special anatomical preparation consisting of an injection of colored neoprene latex followed by a 6-month immersion in a formaldehyde solution enriched with hydrogen peroxide to soften the bone structures (coronal sections) while leaving the fat in the cavernous space intact. This case report corroborates previously published clinical data and shows that the tumor was a hamartoma comprising mature fat cells associated with vascular proliferation. The tumor developed in the cavernous space, which is an interperiosteo-dural space extending from the sphenoid periosteum (osteoperiosteal layer) to the superior and lateral walls of the cavernous space (encephalic layer). This space represents an anatomical continuum extending from the coccyx to the orbit: the interperiosteo-dural concept. It contains fat tissue that is abundant at the level of the orbit and the epidural spinal space and sparser at the level of the cavernous spaces, as was shown in our anatomical study. The authors suggest that angiolipomas represent a characteristic tumor that illustrates the interperiosteo-dural concept because they essentially develop in the fat tissue contained in these spaces.

Object The dura mater has 2 dural layers: the endosteal layer (outer layer), which is firmly attached to the bone, and the meningeal layer (inner layer), which directly covers the brain. These 2 dural layers join together in the middle temporal fossa or the convexity and separate into the orbital, lateral sellar compartment (LSC), or spinal epidural space to form the extradural neural axis compartment (EDNAC). The aim of this work was to anatomically verify the concept of the EDNAC by using electron microscopy. Methods The authors studied the cadaveric heads obtained from 13 adults. Ten of the specimens (or 20 perisellar areas) were injected with colored latex and fixed in formalin. They carefully removed each brain to allow a superior approach to the perisellar area. The 3 other specimens were studied by microscopic and ultrastructural methods to describe the EDNAC in the perisellar area. Special attention was paid to the dural layers surrounding the perisellar area. The authors studied the anatomy of the meningeal architecture of the LSC, the petroclival venous confluence, the orbit, and the trigeminal cave. After dissection, the authors took photographs of the dural layers with the aid of optical magnification. The 3 remaining heads, obtained from fresh cadavers, were prepared for electron microscopic study. Results The EDNAC is limited by the endosteal layer and the meningeal layer and contains fat and/or venous blood. The endosteal layer and meningeal layer were not identical on electron microscopy; this finding can be readily related to the histology of the meninges. Conclusions In this study, the authors demonstrated the existence of the EDNAC concept in the perisellar area by using dissected cadaveric heads and verified the reality of the concept of the meningeal layer with electron microscopy. These findings clearly demonstrated the existence of the EDNAC, a notion that has generally been accepted but never demonstrated microscopically.

We present three cases of meningiomas developing at the site of an old head trauma. We then review the literature regarding the controversies on the development of post-traumatic brain tumors and, finally, we emphasize the medico-legal characteristics of post-traumatic meningiomas, particularly with respect to their cell type which is frequently atypical or anaplastic and which have a poor outcome.

Pontine cavernomas are benign vascular lesions whose surgical treatment is challenging due to their localization. We report our experience in the surgical management of these lesions exclusively using a lateral, subtemporal transtentorial approach in high pontine lesions and an anterior petrosal approach in low pontine lesions. We performed a retrospective study on a series of patients who were operated on for a pontine cavernoma in our neurosurgery department between 1987 and 2007. In the study, we detail the patients' clinical and preoperative radiological data and compare the two surgical techniques we used. Finally, we analyze the postoperative follow-up, the morbidity encountered according to the surgical approach used, and the long-term outcomes. We enrolled nine patients into the study. Six patients were operated on using an anterior petrosal approach. None of the patients died. Five patients were able to resume their former professional activity after surgery and were clearly improved following surgery. One patient was worse after surgery (hemiplegia and deafness). We used a subtemporal transtentorial approach in three of the patients. None of the patients died. Two of the patients were able to resume their prior professional activities without any sequels, and the third patient's condition worsened following surgery (temporal hematoma). The lateral surgical approach for pontine cavernomas constitutes a reasonable surgical alternative to the transventricular, suboccipital, retromastoid, or transclival approaches. Patient morbidity in both approaches is acceptable, and the long-term outcome is satisfactory with respect to sequels and the resumption of prior professional activity.

Resume But Les meningiomes a cellules claires (MCC) sont une variante rare de meningiomes qu’il est important de distinguer en raison de leur potentiel recidivant. Soixante-huit cas ont ete decrits dans la litterature. Dans cette etude retrospective, nous rapportons sept cas de meningiomes a cellules claires operes de 1994 a 2008 au CHU de Tours. Methode Nous exposons les donnees cliniques, radiologiques et histologiques des differents cas. Une etude histologique standard a ete realisee, suivie d’une etude immuno-histochimique (marqueurs etudies : antigene epithelial de membrane (EMA), cytokeratine KL1, recepteurs a la progesterone, MIB-1 (Ki-67), proteine S100). Resultats Deux cas de MCC etaient de siege medullaire et cinq intracrâniens (deux dans le sinus caverneux, deux dans l’angle pontocerebelleux et un dans la fosse posterieure). L’âge moyen etait de 36 ans (deux a 70 ans) avec une predominance feminine (5/7). Trois patientes appartenaient a la meme famille, probablement atteinte d’une neurofibromatose de type 2. L’exerese etait complete dans trois cas. Le suivi etait de trois mois a 15 ans. Dans quatre cas des recidives sont survenues et trois d’entre elles ont entraine un deces. Discussion La frequence des MCC (0,6 % de tous les meningiomes operes) dans notre serie etait a peu pres identique a celle de la litterature. Nous avons observe des aspects histologiques comparables a ceux de la litterature. Nous avons discute de la valeur pronostique de l’index de proliferation (MIB-1), de la qualite de l’exerese et du terrain. Conclusion Comme dans la litterature, nous retrouvons une evolution moins favorable des MCC par rapport aux meningiomes de grade I de l’OMS. Cela est observe en l’absence d’atypie cytonucleaire, d’activite mitotique notable, de necrose. Il semble que l’index de proliferation soit independant de l’evolution.

To define magnetic resonance (MR) imaging criteria for the diagnosis of cavernous sinus invasion by pituitary adenoma.The MR images obtained in 106 patients (86 female, 20 male; age range, 16-71 years) were reviewed retrospectively by two physicians. The standard-of-reference criteria for invasion were the surgical findings. A chi(2) analysis was performed, and the sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) for nine groups of MR imaging signs were computed.Invasion of the cavernous sinus was certain (PPV, 100%) if the percentage of encasement of the internal carotid artery (ICA) by tumor was 67% or greater. It was highly probable if the carotid sulcus venous compartment was not depicted (PPV, 95%) or the line joining the lateral wall of the intracavernous and supracavernous ICAs was passed by the tumor (PPV, 85%). It was definitely not invaded (NPV, 100%) if the percentage of encasement of the intracavernous ICA was lower than 25% or the line joining the medial wall of the intracavernous and supracavernous ICAs was not passed by the tumor.The radiologic diagnosis of cavernous sinus invasion by pituitary adenoma remains difficult, but the above-mentioned criteria may be of assistance.

Object.The authors studied the heads of 17 adult cadavers and one fetus to clarify the anatomy of the sellar region, particularly the lateral boundaries of the hypophyseal fossa.Methods.Vascular injections and microdissection or histological techniques were used in this study. The roof of the cavernous sinuses and diaphragma sellae were part of a single horizontal dural layer that joined the two anterior petroclinoid folds. Laterally, the direction of this layer changed; it became the lateral wall of the cavernous sinus and joined the dura mater of the middle cerebral fossa. On the midline, this layer ballooned toward the sella through the diaphragmatic foramina, created a dural bag containing the hypophysis, and attached to the inferior aspect of the diaphragma sellae. As a consequence, no straight sagittal dural wall existed between the pituitary gland and cavernous sinus; the lateral border of the hypophyseal fossa was part of this anteroposterior and superoinferior convex bag. The authors stress the importance of the venous elements of the region and discuss the structure of the cavernous and coronary sinuses.Conclusions.Invasion of the cavernous sinus makes surgery more risky and difficult and may necessitate modification of the surgical treatment plan. The preoperative diagnosis of cavernous sinus invasion is thus of great interest, but the possibility of normal lateral expansions of the pituitary gland must be kept in mind. A lateral expansion of this gland into the cavernous sinus was encountered in 29% of the specimens, and an adenoma that developed in such an expansion could easily mimic cavernous sinus invasion.

We report a case of primary melanoma of the thoracic spinal cord revealed by progressive bilateral lower extremity weakness associated with sensory loss and urinary dysfunction. The preoperative MRI revealed an intramedullary tumour from T7 to T9. Treatment was by complete surgical excision without radiotherapy. Histopathology and immuno-histochemical studies confirmed the diagnosis. The postoperative course was satisfactory with no sign of recurrence after 28 months of postsurgical follow-up. Primary spinal melanomas are rare intramedullary tumours that can be cured by appropriate surgical treatment.

✓ The case is described of a granular cell tumor of the pituitary stalk in a 40-year-old woman with secondary amenorrhea. A computerized tomography scan showed a large contrast-enhancing mass, and T1-weighted magnetic resonance images demonstrated a tumor isointense to the brain parenchyma with nonhomogeneous enhancement after intravenous administration of gadolinium diethylenetriamine penta-acetic acid. Histopathological examination, especially immunocytochemical and electron microscopic studies, showed elements supporting an astrocytic origin for this type of tumor, a hypothesis advanced by many authors but still controversial.

Context and Objective: Predicting pituitary tumor behavior remains a challenge. This multiparameter investigation aimed to identify markers for recurrence and progression in prolactin tumors. Design: From a cohort of patients treated for prolactin tumors by surgery, we retrospectively studied clinical data, tumor characteristics, clinical outcome, and the expression of nine genes by quantitative RT-PCR. Results: This study included 94 patients (62 females and 32 men), with long postoperative follow-up periods (mean, 138 ± 46 months); 54.3% of patients had a macro or giant adenoma. Tumors were classified into three pathological groups based on their radiological and histological characteristics (noninvasive, 61; invasive, 22; and aggressive-invasive, 11). Immediately after surgery, 60 patients (63.8%) went into remission (prolactin level normalization). Persistently elevated prolactin levels (36.2%) were associated with increasing age, male sex, high preoperative prolactin levels, large tumor size on univariate analysis, and invasion and pathological classification on univariate and multivariate (P = 8 × 10 -10 and 3 × 10 -8) analysis. During follow-up, 19 patients (20%) had tumors that recurred or progressed under dopamine agonist treatment. Invasion and pathological classification were associated with recurrence or progression on univariate analysis. Seven genes (ADAMTS6, CRMP1, PTTG, ASK, CCNB1, AURKB, and CENPE) were associated with tumor recurrence or progression and five of these (ADAMTS6, CRMP1, ASK, CCNB1, and CENPE) were associated with the pathological classification. Conclusion: This study identifies both the clinical and histological factors that relate to prolactin tumor recurrence or progression. Molecular markers give additional information for prognosis of such tumors. Altogether, our results could influence the management of patients with pituitary tumors. Copyright © 2010 by The Endocrine Society.

Purpose To evaluate the operative results of spheno-orbital meningiomas regarding resectability, recurrence and ophthalmological outcome. Methods Retrospective study of 40 patients who underwent surgical resection of spheno-orbital meningiomas followed by bony reconstruction between 1992 and 2008. Results Mean age of 50 years and sex ratio of 37 women and 3 men. The most common preoperative sign was proptosis (90%), visual acuity (VA) and visual field deficits (55%). Periorbital tumor infiltration is a predictive factor of decreased VA in case of initial VA deficit. Optic canal invasion and resection don’t influence VA recovery. Postoperatively, 10 patients showed severe permanent visual field deficits, versus 17 patients showed no visual field deficits. After surgery, proptosis improved for all patients with a preoperative mean proptosis of 5,6 +/- 3,6 mm versus 2,2 +/- 2,7 mm postoperatively (p 90%) tumor resection gives a long-term survival free of clinical recurrence for 80 % of our patients.

Prolactinomas usually exhibit a benign course and can be safely and effectively managed by dopamine agonists (DA). However, some are locally invasive and may show resistance to DA therapy, and the management of such cases remains controversial. The aim of the present study was to determine whether histological features and markers of cell proliferation correlated to the clinical behaviour of prolactinomas and with DA resistance.This retrospective study included 74 cases (36 men and 38 women) who had monohormonal prolactinomas removed by transsphenoidal surgery. The prolactinomas were categorized on the basis of tumour size (48 macroadenomas), invasion of the cavernous sinus (n = 31), and resistance to bromocriptine (BRC) therapy (n = 14). Group 1 consisted of non-invasive microprolactinomas (n = 24), group 2 of non-invasive macroprolactinomas (n = 19), group 3 of invasive non-BRC-resistant tumours (n = 19), and group 4 of invasive BRC-resistant tumours (n = 12). The later group included one case of carcinoma with bone and lung metastases. Seven additional parameters were studied, these being age, sex, basal prolactin (PRL) levels, the Ki-67 and PCNA labelling indices (LI), mitotic count, and cellular atypia.Age and preoperative PRL levels did not correlate to the histological parameters studied. Tumour size and invasion were related to cellular atypia and the Ki-67 LI. BRC-resistant tumours were more frequently invasive (12/14) than BRC-responsive tumours (11/30; p = 0.002) and were more frequent in men than in women (33 versus 5%; p = 0.003). BRC-resistant tumours had a higher Ki-67 LI and mitotic count (4.2+/-2.0% and 4+/-1, respectively) than other tumours (0.7+/-0.2% and 1+/-0, respectively; p0.05). The strongest correlations with tumoural staging were seen with male sex and high mitotic activity. Six out of the 12 invasive BRC-resistant macroprolactinomas, including the PRL secreting carcinoma, exhibited histological features of aggressiveness (a mitotic count/=3 [i.e. in the fourth quartile] and/or a high Ki-67 LI and cellular atypia).In this surgical retrospective series, histological signs of aggressiveness are present in 50% of invasive and BRC-resistant prolactinomas, which are more frequent in men than in women. This fits with the behaviour of BRC-resistant prolactinomas, which can continue to grow despite DA treatment. These findings justify the long-term follow up of these tumours, and the use of surgery and/or radiotherapy if there is concern about the control of tumour growth.

We report a case of radiation-induced meningioma that appeared 12 years after the discovery and treatment of a posterior fossa medulloblastoma. The features of the case that support the diagnosis of radiation-induced meningioma are discussed.

Seeding on surgical pathway is a rare form of clival chordoma treatment failure. We report the case of a 42-year-old male with a clival chondroid chordoma removed by a sublabial transsphenoidal approach followed by proton beam radiotherapy, who developed a maxillary bone recurrence 3 years after surgery.

2. Kramer IRH, Pindborg JJ, Shear M: Histological Typing of Odontogenic Tumours (ed 2). Geneva, Switzerland, World Health Organization, 1992, p 20 3. Buchner A: The central (intraosseous) calcifying odontogenic cyst: An analysis of 215 cases. J Oral Maxillofac Surg 49:330, 1991 4. Praetorius F, Hjorting-Hansen E, Gorlm RJ, et al: Calcifying odontogenic cyst: Range, variations, and neoplastic potential. Acta Odontol Stand 39:227, 1981 5. Pindborg JJ, Kramer IRH, Torioni H: Histologic Typing of Odontogenic Tumours, Jaw Cysts and Allied Lesions (ed 1). Geneva, Switzerland, World Health Organization, 1971, p 28,35 6. Ikemura K, Horie A, Tashiro H, et al: Simultaneous occurrence of calcifying odontogenic cyst and its malignant transformation. Cancer 56:2861,1985 7. Ellis GL, Shmookler BM: Aggressive (malignant?) epithelial odontogenic ghost cell tumor. Oral Surg Oral Med Oral Path01 61:471, 1986 8. Grodjesk JE, Dolinsky HB, Schneider LC, et al: Odontogenic ghost cell carcinoma. Oral Surg Oral Med Oral Path01 63:576, 1987 9. Scott J, Wood GD: Aggressive calcifying odontogenic cyst: A possible variant of ameloblastoma. Br J Oral Maxillofac Surg 27~53, 1989 10. Alcalde RE, Sasaki A, Misaki M, et al: Odontogenic ghost cell carcinoma: Report of a case and review of the literature. J Oral Maxillofac Surg 54:108, 1996 11. Folpe AL, Tsue T, Rogerson L, et al: Odontogenic ghost cell carcinoma: A case report with immunohistochemical and ultrastmctural characterization. J Oral Path01 Med 27:185, 1998

Study design This study described the course of a case of massive osteolysis (Gorham's disease) of the cervical spine and discussed the literature data. Objectives To describe a case of massive osteolysis of the cervical spine with fatal outcome and to discuss the classification of the disease among osteolysis and its therapeutic modalities. Summary of background data Massive osteolysis is a rare condition (fewer than 100 cases reported in the literature) of unknown etiology, which may involve any bone in the body, with a propensity for the shoulder and pelvic girdle. Few cases of cervical spine involvement were reported. Methods The case of a 32-year-old man with fatal progressive massive osteolysis of the cervical spine despite multiple attempts to achieve surgical stabilization was reported. Results Massive osteolysis was characterized by complete destruction of all or part of a bone by angiomatous tissue and may have represented a local disturbance of osteoclastic activity. No successful therapy was proposed, and the prognosis of spine involvement was very poor. Conclusions Massive osteolysis is a rare condition with no successful therapy. The hypothesis of involvement of circulating preosteoclasts in the osteolytic process may suggest treatment attempts with diphosphonates because of the futility of standard bone grafting techniques in spine involvement.

We report the clinical, radiological, and histological features of an 8-year-old boy with an unusual presentation of fronto-orbital sphenoidal fibrous dysplasia. The various forms of fibrous dysplasia are outlined and the differential diagnosis discussed. An approach to surgical management is proposed.

A 25-year-old man was hospitalized after suffering a subarachnoid hemorrhage. Arteriograms disclosed two arteriovenous malformations, one of which was asymptomatic. Rendu-Osler-Weber disease was suspected because of the concomitant existence of cutaneous telangiectases, Review of the literature shows that in 12 previously published cases involving multiple arteriovenous malformations, this diagnosis was established five times. The indications for surgical treatment are discussed.

A case of an ectopic prolactin-producing tumor located within the sphenoid sinus is reported. It was discovered in a girl who experienced secondary amenorrhea without galactorrhea. We discuss the different diagnoses proposed before immunocytochemical staining results were available. This case presented unusual radiological, surgical, and histopathological aspects.

A clinicopathological study with electro-oculographic recording of a patient with periodic alternating gaze (PAG) is presented. There have been five previously reported anatomical observations of patients with this disorder. Bilateral cerebral disease with a mesencephalic lesion is observed. A study of oculovestibular reactions suggests that the generator of PAG is independent of the vestibular system

The therapeutic efficacy of intravenous nimodipine to treat the syndrome of delayed ischemic deterioration or vasospasm after subarachnoid hemorrhage caused by a ruptured aneurysm was investigated in a randomized, double-blind, placebo-controlled multicenter study. A total of 188 patients (nimodipine (N) = 102, placebo (P) = 86) were enrolled in the study, both pre- and postoperatively, within 24 hours of clinical deterioration connected with vasospasm or within 24 hours of arteriography that identified vasospasm. After 61 patients were excluded for not meeting study criteria or for protocol violation, the results were supported by 127 validated case reports: 73 patients received intravenous treatment with nimodipine, and 54 were given placebo. Analysis of the main criterion of efficacy, the number of deaths and of patients with severe deficit related to vasospasm alone, showed a significant statistical difference (N = 8 (19%), P = 17 (49%), P = 0.01). The risk of death or disability was reduced by 66% in the treated group. Analysis of this criterion by type of inclusion (clinical or angiographic) also showed a significant reduction in the clinical group (P = 0,05), but there was no difference in the angiographic group. The risk of mortality connected with vasospasm was reduced by 82%, but analysis by group showed that there was no significant difference for those patients included on clinical criteria, whereas mortality was reduced to a much greater extent in the angiographic group. These results demonstrate the therapeutic efficacy of nimodipine in reducing secondary ischemic brain damage in patients already suffering from angiographic vasospasm or delayed ischemic deterioration.