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Patient: Male, 28 Final Diagnosis: Cystic chromophobe rcc Symptoms: Flank pain Medication: — Clinical Procedure: Ct scan Specialty: Radiology Objective: Rare disease Background: Chromophobe renal cell carcinoma is a rare subtype of renal cell carcinoma which accounts for 4% to 6% of renal cell carcinoma subtypes. These tumors generally have a good prognosis. Typical radiological features include a well-defined homogeneous mass and a hypo-vascular soft tissue enhancement. Cystic chromophobe renal cell carcinoma is a very rare entity in which the overall estimated cystic renal cell carcinoma of all subtypes account for 4%. We describe a rare presentation of chromophobe renal cell carcinoma presenting as a unilocular cystic mass with mural nodules. Case Report: A 28-year-old healthy male presented with a history of right upper quadrant fullness and discomfort. Hepatomegaly was expected on examination. Ultrasonographic assessment revealed a normal liver size and echo texture. However, a large cystic mass measuring 15.7×12.8 cm was found arising from the right kidney which showed no internal vascularity but turbid fluid and debris within the dependent regions of the mass. Multiphase computed tomography scan showed a large unilocular cystic mass with enhancing mural nodules. There was no lymphovascular or perirenal invasion. The patient underwent uneventful total right renal nephrectomy. Histological examination revealed a chromophobe renal cell carcinoma of grade II. Conclusions: Cystic renal neoplasm differential diagnosis varies from benign entities to malignant neoplasms. Knowledge of the variable common features for each renal cell carcinoma subtypes can aid in narrowing the differential diagnosis and prompt appropriate surgical management since a possibility of nephron sparing technique might still take place in suspected cystic chromophobe renal cell carcinoma but never for cystic clear cell renal cell carcinoma.

Vascular endothelial growth factor (VEGF), its inhibitory splice variant, VEGF165b and Endocrine Gland derived VEGF (EG-VEGF) have a controversial role in pituitary gland. We aim to study VEGF, VEGF165b and EG-VEGF expression in pituitary adenomas. A significant correlation was found between growth hormone (GH) and VEGF secretion (P=0.024). For prolactinomas, VEGF and prolactin expression, had a P-value of 0.02 for Kendall coefficient and a P-value of 0.043 for the Spearman coefficient. VEGF-mRNA amplification was detected in both tumor cells and folliculostellate cells. VEGF165b was positive in 16.66% of pituitary adenomas. EG-VEGF was significantly correlated with prolactin (P=0.025) and luteinizing hormone (P=0.028). Our data strongly support VEGF, VEGF165b and EG-VEGF as important players of pituitary adenomas tumorigenesis. Particular hormonal milieu heterogeneity, special vascular network with an unusual reactivity to tumor growth correlated with variability of VEGF, VEGF165b and EG-VEGF secretion may stratify pituitary adenomas in several molecular groups with a direct impact on therapy and prognosis.

The majority of enhancing renal masses cannot be characterized through imaging as malignant or benign; however, such characterization could save patients from unnecessary surgery and/or biopsy and associated morbidity. Herein, we review the recent literature on the emerging use ofRecent reports have shown that

The aim of the study was to assess the outcome after nephron-sparing surgery (NSS) of patients with small renal masses (SRMs) who would have been eligible for active surveillance (AS).Data were collected retrospectively for 758 patients who underwent NSS over a 5-year period. Outcomes were assessed in two groups of patients who were eligible for AS according to different criteria. Group 1 criteria were as follows: age75 years, renal mass ≤4 cm, significant comorbidities [Charlson Comorbidity Index (CCI)2]. Group 2 criteria were as follows: any SRM ≤ 4 cm regardless of age, severe comorbidities with a 10-year mortality risk50 % (CCI4). The two groups were not compared statistically because some patients were included in both.Fifty-five patients (7.3 %) were included in Group 1 and 62 (8.2 %) in Group 2. There was a significant proportion of benign tumours in Group 1 (N = 6; 11 %) and Group 2 (N = 6; 10 %). Six (11 %) positive margins were observed in Group 1 and 8 (13 %) in Group 2. The 2- and 5-year recurrence-free survival rates were 100 and 77.4 %, respectively, in Group 1, and 88.5 and 79.6 % in Group 2. The 2- and 5-year overall survival rates were 100 and 74.7 % in Group 1, and 96.7 and 78.1 % in Group 2.The majority of patients with SRMs who would have been eligible for AS had no recurrence after initial tumour removal. In these patients, a CCI4 appeared to be a pertinent criterion to identify those patients less likely to benefit from immediate surgery.

Clinical and in-vitro investigations have been performed on a corticotroph adenoma removed from a patient suffering from Cushing's disease. Prior to surgery, the patient's Cushing's disease had been successfully controlled, clinically and biochemically, by long term administration of bromocriptine. After selective adenomectomy, tumour tissue was investigated by a perfused isolated cell column technique. It was shown that the tumour cells secreted immunoreactive- (IR)- ACTH and IR-alpha-MSH and that the release of both peptides was promptly suppressed by dopamine. Chromatographic analysis of the secreted IR-alpha-MSH revealed a high proportion of acetylated alpha-MSH; smaller amounts of desacetyl alpha-MSH and diacetyl alpha-MSH were present. The relevance of these findings to the proposal that certain corticotroph adenomas are derived from the intermediate lobe of the pituitary is discussed. It is concluded that there is little direct evidence for involvement of the residual zona intermedia of the adult human pituitary in the development of Cushing's disease.

A series of 11 pituitary tumors in budgerigars were classified on the basis of their clinical, gross, microscopic, and immunohistochemical characteristics. Affected birds were young to middle-aged. Clinically, neurologic signs—including difficulties flying, ataxia, and blindness—were most commonly reported. Additional clinical signs included weight loss, abnormal feathers or molting, increased respiratory efforts, and exophthalmos. Nine birds were diagnosed with chromophobic pituitary adenomas, and 2 birds had chromophobic pituitary carcinomas. Only 1 tumor was delimited to the pituitary gland; the other 10 variably invaded the brain, skull, and retrobulbar space. Distant metastases were identified in 2 birds. All tumors were immunohistochemically strongly positive for growth hormone, consistent with the diagnosis of somatotroph tumors. The common occurrence and early onset may suggest a genetic predisposition of budgerigars to develop somatotroph pituitary tumors with a high incidence of local invasion and with metastatic potential.

Visual functions were studied pre- and post-operatively in 128 patients treated for pituitary adenoma with operations by intracranial (89 patients) or transsphenoidal approach (39 patients). Most patients were given post-operative irradiation. Follow-up was 6 months or more. Visual field defects were seen pre-operatively in 85% of the patients operated intracranially and in 36% of the patients operated transsphenoidally. Visual acuity was reduced, at least in one eye, in 64% of the patients in the former group and in 26% of the latter group. Additional diagnostic information was obtained from recordings of visually evoked cortical potentials. After intracranial operations visual acuity was normalized or improved in 65 % and visual fields in 82 % of the patients with pre-operative visual defects. After transsphenoidal operations the corresponding values were 56% and 92%. A detailed analysis of the visual acuity showed that eyes with optic atrophy pre-operatively improved to a very limited extent. Other pre-operative ophthalmological disturbances were of much less prognostic significance. Improvement of vision was often gradual and could continue for more than a year after treatment. Even if visual acuity and visual fields had been completely restored, visually evoked cortical potentials could remain impaired, indicating that evoked potentials might be a very sensitive test for the evaluation of visual recovery.

A relationship between the degree of atrophy of retinal nerve fibres and visual field defect has been described in postoperative steady-state patients (LundstrOm & FrisEn 1976). The same factors were studied prior to surgery in six patients with compression of the chiasm due to chromophobe adenoma. In five eyes the field defect was excessive in relation to the degree of atrophy. After surgery the visual field defects improved to a level corresponding to the degree of atrophy in these eyes. In the remaining seven eyes there was a close correspondence between the atrophy and the visual field defect already before surgery. The visual field defects remained unchanged in these eyes. Provided that atrophy does not increase after surgery, simultaneous preoperative evaluation of retinal nerve fibre atrophy and visual field defect allows an accurate prediction of prognosis for improvement.

An apparatus consisting of a Goldmann perimeter fitted with a laser generator is described. Sharper definition and earlier detection of defects with this technique in six cases of chromophobe adenoma are reported.

Human primary and secondary malignant, and primary benign brain tumours were short time cultured in order to detect a coating with a protein, immunologically indistinguishable from complement component Cl inactivator. The investigations were done by cytophotometry of immunoflurescence on individual cells from human brains. The cell cultures were incubated with specific rabbit antiserum against human Cl inactivator, conjugated with fluorescein isothiocyanate. The majority of the malignant brain tumours, and a few of the benign brain tumours, were shown to carry the Cl inactivator—resembling protein. The primary malignant brain tumour cells seemed to lose this coating during prolonged culturing. Metastatic brain carcinomas retained their inactivator coating.

The effect of slow intravenous infusion of luteinizing hormone-releasing hormone (LH-RH) on serum LH was studied in 58 patients with anatomically verified hypothalamo-pituitary disorders as well as in control groups. Normal responses to the LH-RH test were found in 15 out of 15 cases with intrasellar pituitary tumours and in five out of six cases after transsphenoidal surgery for an intrasellar pituitary tumour. Twenty-one out of 25 patients where a pituitary tumour with both supra- and intrasellar extension had been removed had pathological responses to the test. All eight patients with hypothalamic disorders, mainly tumours not directly influencing the pituitary, gave normal responses to the LH-RH tests. Out of four patients with hypothalamopituitary malformations a pathological response to the LH-RH infusion was found in three cases and a normal response in one case. The increase of serum LH after LH-RH infusion was pronounced and rather constant in males and post-menopausal women. It was more difficult to evaluate a “normal” test response in women of fertile ages because of the great variations during the different menstrual phases. It is concluded that the LH-RH test may be of importance in the investigation of patients with suspected tumours of the pituitary as well as for differential diagnosis between hypothalamic and pituitary disorders of gonadotropin secretion.

RESUME Les shunts biventriculaire, bilatiral et unilateral-double Chez 19 malades presentant de ‘multiples obstructions de cavite’ intracrâniennes, des experiences de shunts biventriculaires, bilateraux et unilateraux-double ont ete decrits. Les tumeurs de la ligne mediane anterieure proches du trou de Monro constituent l'indication principale d'un shunt biventriculaire tandis que les obstructions repetees du catheter ventriculaire dans les hydrocephalies non communicantes constituent une indication relative. Les diffusions sous-durales importantes avec ou sans hydrocephalic ou porencephalic et/ou autres malformations kystiques constituent l'indication d'un shunt bilateral ou unilateral-double. Alors que le benefice d'un shunt biventricula:re dans les obstructions tumorales au trou de Monro ou a la partie anterieure du troisieme ventricule est bien determine, une etude plus approfondie des autres procedes apparait necessaire. Les avant-ages fonctionnels totaux de ces derivations ne peuvent etre obtenus que si les principes du moment de l'application, de l'observation reguliere et d'un controle precoce sont appliques de la meme facon qu'avec un shunt unilateral. ZUSAMMENFASSUNG Der biventrikuare, der bilaterale und der doppelt-unilaterale Shunt Die Erfahrungen mit biventrikularen, bilateralen und doppelt-unilateralen Shunt– Techniken bei 19 Patienten mit intracranialen multiplen Verschlussen der Hohlraume wurden beschrieben. Wahrend Tumoren an der vorderen Mittellinie nahe dem Monro'schen Foramen die Hauptindikation fur einen biventrikularen Shunt, und wiederholte Verschlusse eines Ventrikelkatheters beim occlusiven Hydrocephalus eine relative Indikation fur einen biventrikularen Shunt darstellen, sind grosere subdurale Ergusse mit oder ohne Hydrocephalus oder porencephalische und/oder andere cystische Misbildungen als Indikationen fur einen bilateralen oder doppelt-unilateralen Shunt zu betrachten. Wahrend die Vorteile eines biventrikularen Shunts bei einem Tumorverschlus am Monro'schen Foramen oder in H6he des vorderen dritten Ventrikels unb;stritten sind, miissen die anderen Verfahren noch weiter uberpruft werden. Den vollen funktionellen Vorteil dieser Systeme kann man nur erwarten, wenn die Grundsatze der rechtzeitigen Anwendung, der regelmausigen Beobachtung und der fruhen Revision in derselben Weise wie bei unilateralen Shunts beachtet werden. RESUMEN La comunicacidn biventricular, la bilateral y la dual-unitaria Se describe la practica de comunicacioAn biventricular, bilateral y dual-unitaria en 19 pacientes de las obstrucciones multiples con cavidad intracrCneales. Mientras que los tumores de la linea media anterior junto a los agujeros de Monro, representan la mayor indicacion para una comunicacion biventricular, y la repetida obstruccidn del cateter ventricular en la hidrocefalia oclusiva se considera como una indicacioAn relativa de comunicacidn biventricular, los grandes derrames subdurales con o sin hidrocefalia o porencefalia y/o otras malformaciones quisticas representan una indicaci6n para una comunicacidn bilateral o dual-unilateral. Mientras que es bien definido el beneficio que se obtiene con una comunicacion biventricular en la obstruccidn tumoral del agujero de Monro o de la parte anterior del tercer ventrfculo, es precisa una evaluacidn mas amplia de los otros procedimientos. Solo puede esperarse una ventaja functional total en estos casos si se adoptan los principios del momento de aplicacion, observacidn regular y revisidn precoz de la misma forma que en en las comunicaciones unilaterales.

Our better understanding of the morphologic spectrum of renal cortical tumors has resulted in a clinically more relevant classification of these tumor types. We now recognize that "granular cell" and "sarcomatoid" renal cell carcinoma are only nonspecific descriptors, and that such features are seen in a variety of types of renal tumors. The authors believe that the recently gained knowledge about molecular-driven antigen expression will play an important role in the characterization, development, and evaluation of targeted therapies in kidney cancer in the coming years.

We describe here a case of a clinically nonfunctioning pituitary adenoma, but with expression of ACTH and PRL. A 42-year-old woman was referred to our department for further evaluation of pituitary tumor. She had no acromegaloid features, and no typical Cushingoid features. She had no galactorrhea, and had regular menses. GH, IGF-I, LH, FSH, TSH, ACTH and cortisol levels in blood were all within the normal ranges, while PRL levels were mildly elevated. Both ACTH and cortisol levels were adequately increased in response to CRH, and both were suppressed by a small dose of dexamethasone. Plasma ACTH and cortisol levels were decreased at night, suggesting the circadian rhythms for plasma ACTH levels were undisturbed. Based on these findings we did not clinically suspect ACTH-producing tumor, however immunohistochemistry revealed ACTH immunoreactivity in the pituitary adenoma. Therefore, the tumor was considered a silent corticotroph adenoma. PRL was co-expressed in a significant subpopulation of ACTH-immunoreactive tumor cells. Ptx1, Neuro D1, and T pit were densely expressed and Pit-1 was sparsely expressed in the nuclei of adenoma cells. It is therefore possible that a tumor originating in an immature or uncommited adenohypophysial stem cell may later differentiate into different cell types due to a combination of certain specific transcriptional factors.

Purpose: We compared histological subtype, pathological features and outcome of patients with solid renal masses who were 18 to 40 years old vs patients who were 60 to 70 years old. Materials and Methods: We conducted a retrospective review of the Mayo Clinic Nephrectomy Registry from 1970 to 2000, and identified 124 patients 18 to 40 years old and 1,067 patients 60 to 70 years old available for analysis. Results: There was no significant difference in the incidence of benign solid renal masses between patients 18 to 40 years old and those 60 to 70 years old (13.7% vs 10.2%). Among patients with renal cell carcinoma (RCC), younger patients were more likely to have chromophobe RCC (13.1% vs 3.6%) and less likely to have clear cell RCC (70.1% vs 81.5%) than older patients. Among patients with clear cell RCC, younger patients were more likely to have stage pT2b or lower tumors (82.7% vs 69.9%) and a higher incidence of cystic clear cell RCC (10.7% vs 2.2%) than older patients. Younger patients had an improved cancer specific survival compared with older patients but this difference was not statistically significant (risk ratio 0.71, p=0.127). Conclusions: We found that patients 18 to 40 years old were more likely to have chromophobe and less likely to have clear cell RCC compared with patients 60 to 70 years old. We did not identify a higher incidence of papillary RCC in younger patients. Patients with clear cell RCC 18 to 40 years old had a higher incidence of low stage and cystic tumors compared with patients 60 to 70 years old, features which have been shown to have a favorable prognosis. These factors likely contributed to improved cancer specific survival for younger patients.

Objectives: The common subtypes of renal tumors are conventional or clear cell carcinoma, papillary carcinoma, chromophobe carcinoma and oncocytoma. Each subtype has its distinct histogenesis and clinical evolution. DNA ploidy is viewed as a marker of gross genomic aberrations. The aim of this study is to evaluate the DNA ploidy in the common subtypes of renal tumors to increase our understanding of renal tumor biology and to broaden clinical application of DNA ploidy. Methods: 38 renal tumor samples (13 clear cell RCCs, 12 papillary RCCs, 7 chromophobe RCCs, and 6 oncocytomas) were studied. Five biopsies of different parts of each fresh tumor were subjected to a flow cytometric analysis of DNA ploidy. Results: All tumors except one papillary RCC generated interpretable DNA histograms. Flow cytometric analysis of oncocytomas showed the diploid pattern (29/30 frequencies) while the chromophobe RCC never showed the diploid pattern (0/55 frequencies) (p < 0.01). 3/7 chromopbobe RCCs possessed the hypodiploid stemline. The hypodiploid stemline appeared neither in conventional RCCs (0/63 frequencies) nor in papillary RCCs (0/50 frequencies). The diploid pattern was dominant in conventional and papillary RCCs. 10/13 (76.9%) of clear cell RCCs and 9/11 (81.8%) of papillary RCCs possessed a homogeneous DNA ploidy pattern while only 1/7 (14.3%) has a homogeneous DNA ploidy pattern. 6/7 chromophobe RCCs had multiple aneuploid stemlines. Conclusions: Flow cytometric analysis reveals that conventional and papillary RCCs are more homogeneous than chromophobe RCC. Each subtype of renal tumors possesses a specific DNA ploidy pattern. The analysis of DNA ploidy is useful for the differentiation of common subtypes of renal tumors in morphologically difficult cases.

Although the human central nervous system used to be considered relatively resistant to the carcinogenic action of ionizing radiation, several lines of evidence now document a high incidence of secondary tumors in irradiated patients. The numerous reports of radiation-induced cerebral meningiomas generally distinguish those induced by high-dose radiation from those induced by low-dose radiation. We describe the case of patient who underwent subtotal resection of a chromophobe pituitary adenoma at the age of 18 years, who was successively treated by conventional fractionated radiotherapy with gamma rays emitted by a source of 60Co until a total dose of 41 Gy. Over the next 30 years the patient experienced all the known late effects of radiation, including panhypopituitarism, cranial-nerve deficits (II, III and VI), massive radiation necrosis involving the left cerebral hemisphere and causing right hemiparesis and aphasia and, ultimately, an atypical tentorial meningioma with early recurrence after total resection.

The Dextrostix Reflectance Meter (DRM) system has been used for monitoring of the blood glucose level during diagnostic hypoglycemia in 16 patients with suspected pituitary insufficiency. In the range 15–40 mg glucose/100 ml blood the reproducibility of the DRM system, + 1.7 mg/100 ml, was as good as that of the reference method. The agreement between values obtained with the DRM and the reference method showed a straight line relationship, the equation for the regression line being y = 1.02x- 3.1 and the coefficient of correlation 0.94. The access to immediate and continuous information about the blood glucose level was of value for avoidance of complications and methodological misinterpretations, and permitted the test to be performed in open care.

Two rare cases of pituitary adenoma with neuronal choristoma are described. Both patients were female and presented with features of acromegaly with elevated growth hormone and prolactin levels. Radiologically, both lesions were predominantly intrasellar in location with extension into suprasellar region, but hypothalamus was not involved. Histopathological examination revealed a mixture of chromophobe pituitary adenoma cells and neuronal cells. In both cases, the adenoma component was positive for growth hormone and prolactin. Interestingly, immunopositivity for alpha-subunit, cytokeratin and prolactin was seen in the adenoma and neuronal cells in one case. Our findings support the hypothesis that the neuronal cells possibly arise from adenoma cells as a result of metaplasia.

This report describes the presentation, pre-operative findings, surgical management and follow-up results of 20 pediatric patients with pituitary adenomas. The most common presenting complaints encountered were, headache and decrease in visual acuity. Among the patients with endocrine problems, majority had frank Cushing's disease (30%). Nine patients (45%) were endocrinally normal. The most common, histological type of pituitary tumour encountered in this series was the Chromophobe adenoma. One patient died following surgery. Radiation therapy was given in all post operative cases. Response to radiation therapy has been satisfactory on follow up. The generally encouraging results we have observed following the multi-disciplinary treatment, confirms the findings reported by other workers.

In a man with a histologically verified non-functioning pituitary adenoma with suprasellar extension, Doppler signals resembled those associated with bruits (ultrasonic bruits). These signals were detected in the anterior circulation of Willis both preoperatively and intraoperatively. The large tumor was resected subtotally via a right orbitozygomatic approach. The use of microvascular sonography for intraoperative monitoring can provide information on the potential cerebrovascular complications of surgery. No previous studies on the presence of ultrasonic bruits associated with pituitary adenomas have been reported. The clinical implications for the surgical treatment of pituitary adenomas are discussed.

We report two cases of acromegaly due to pituitary adenoma without any other endocrinopathy in a family. The patients had high plasma GH and were improved by transsphenoidal adenomectomy. Acromegaly is usually a clinical syndrome of sporadic nonfamilial occurrence. The familial occurrence of acromegaly not associated with multiple endocrine neoplasia is very rare. Our patients are unlikely to be associated with the multiple endocrine neoplasia type 1 syndrome. Here we describe two patients with acromegaly, a father and his daughter, and review familial cases reported.

CASE REPORT We have experienced a case in which surgical removal of a pituitary tumor from a male patient was followed by the occurrence of hyponatremia, which in turn was later associated with central pontine myelinolysis (CPM). A 4 X 3 X 3 cm pituitary tumor with hypothalamic extension was removed via a transsphenoidal approach. The post-operative course was uneventful until severe hyponatremia developed. To avoid drastic correction of electrolyte levels, reestablishment of normal serum levels was spread over 1 week. Following this, however, various neurologic symptoms such as pseudobulbar palsy, quadriplegia, extrapyramidal symptoms, and mental symptoms appeared. The case was diagnosed as CPM and extrapontine myelinolysis (EPM) on the basis of the clinical course and symptoms, and high-dosage steroid therapy was commenced. RESULTS There was consequent gradual improvement in symptoms. One month later, MRI revealed typical demyelination lesions in the central pons and striatum. Abnormal electrolyte conditions easily occur in pituitary tumors associated with hypothalamic extension in an altered hormone environment. It is known that CPM and EPM result from drastic correction of hyponatremia. CONCLUSIONS The frequent measurement of electrolytes and cautious correction of sodium imbalance are important for the prevention of CPM and EPM in the postoperative management of patients who undergo surgery for a pituitary tumor and whose high-dosage steroid therapy are effective.

We describe a very rare case of nonfunctional pituitary adenoma (NFPA) that exhibited corticotrophic activity after resection and radiotherapy. The possible mechanisms of the transformation from NFPA to Cushing disease (CD) are discussed. A 43-year-old man presented with impaired vision, bilateral frontal headaches, and hyposexuality. He had no symptoms suggestive of hypercortisolism, and 8 am plasma cortisol concentration was 67.88 ng/mL. Brain imaging revealed a 15 × 15 × 21-mm sellar mass suggestive of a macroadenoma. The tumor was resected by transsphenoidal surgery and identified by immunohistochemical analysis as a chromophobic adenoma that did not stain for pituitary hormones. The patient was treated with prednisone and levothyroxine replacement therapy. After a third recurrence, the patient presented with clinical features and physical signs of Cushing syndrome. Plasma adrenocorticotropic hormone (ACTH) and cortisol concentrations were elevated, and there was a loss of circadian rhythms. Inferior petrosal sinus sampling after desmopressin showed the central–peripheral ACTH ratio was greater than 3:1. A repeat transsphenoidal resection was undertaken. Immunohistochemistry revealed ACTH positivity. Three months following surgery, imaging showed little residual tumor, but plasma ACTH remained elevated. He was referred for postoperative Gamma Knife radiotherapy. The immunological activity and biological features of the hormones secreted from a pituitary adenoma vary with time. Because long-term outcomes are unpredictable, postoperative follow-up is essential to detect postoperative transformation from NFPA to CD.

Leptomeningeal dissemination of pituitary adenoma is a very rare occurrence. The present report describes the case of a 28 year old man with a nonfunctioning pituitary adenoma which was operated on and irradiated. Eight years later, the patient developed Cushing's syndrome and multiple leptomeningeal masses were revealed by brain CT and MNR. The diagnosis was ACTH-cell adenoma, without malignant histological signs. The growth fraction of the tumor, detected by means of the immunohistochemical demonstration of proliferating cell nuclear antigen (PCNA), was 5.45% of cells; this figure is higher than that reported for non-recurrent pituitary adenomas. From a review of the reported cases, the possibility of predicting late malignant behaviour is discussed. The microscopic aspect has no prognostic value, since metastasizing cases are not overtly malignant in a histological or cytological sense. The application of methods aimed at detecting the growth fraction of the tumor may prove useful in the early identification of aggressive pituitary tumors.

Fifteen cases of the rare association of pituitary adenoma and neuronal choristoma (PANCH) were investigated by histology, immunohistochemistry, and electron microscopy. Acromegaly was apparent clinically in 11 patients and was equivocal in 1, and 3 lesions appeared to be nonfunctioning. Histology revealed various proportions of chromophobic PA and nervous tissue consisting of neuronlike cells and neuropil. Immunohistochemistry documented growth hormone (GH) in every PA, including those unassociated with clinical acromegaly. In contrast, the NCH component showed no consistent immunohistochemical profile. Most frequent reactivities were for the pituitary hormone alpha subunit, thyroid-stimulating hormone, and GH, whereas only a few cases displayed scattered positivity for GH-releasing hormone. Low-molecular weight keratin tested positive in PAs and in a few cells and processes of an NCH. A few fibrous bodies were immunoreactive for neurofilament protein. Electron microscopy revealed sparsely granulated GH cell adenoma, neurons, and neuropil. Cells intermediate between PA and neurons were numerous in 1 lesion. The present morphologic findings as well as lack of GH cell hyperplasia and the consistent association of NCH with but one type of PA do not support the causative role of NCH in the initiation of PA, as proposed previously. It appears that NCH is the result of neuronal differentiation within sparsely granulated GH cell adenomas.

Both chromophobe carcinoma and sarcomatoid carcinoma of the kidney are rare. The former is characterized by a relatively good prognosis, while the latter is a highly aggressive tumor. Coexistence of the two components in one renal tumor, which has been reported only rarely, is therefore paradoxical. Both sarcomatoid and chromophobe renal carcinoma were diagnosed in a 52-year-old woman following nephrectomy and resection of metastases in the right lobe of the liver. She died of the disease two months after the first operation; only the sarcomatoid component of her tumor was seen in the liver metastasis and the recurrent carcinoma. Differences in phenotype, immunophenotype and DNA-ploidy patterns of the two components are reported. The intensive p53 staining observed only in the sarcomatoid area supports the role of the TP53 gene in the transformation of chromophobe renal carcinoma to sarcomatoid carcinoma.

Acromegaly is a rare disease that, in the majority of cases, is due to the presence of a benign growth hormone (GH)- producing tumor of the pituitary. Growth hormone has profound effects on linear bone growth, bone metabolism, and bone mass. In acromegaly, the skeletal effects of chronic GH excess have been mainly addressed by evaluating bone mineral density (BMD). Most data were obtained in patients with active acromegaly, and apparently high or normal BMD was observed in the absence of hypogonadism. The Autors describe a case of patient affected by acromegaly without hypogonadism with serious osteoporosis and biological signs of osteomalacia.

Secreting pituitary adenomas have marked prevalence in pediatric adenomas. We described a case of an 11-year-old boy with pituitary null cell macroadenoma with silent macroscopic pituitary apoplexy. The patient was admitted with bitemporal hemianopsia and a gradual deterioration of visual acuity. An endocrinological study revealed a low concentration of testosterone and in the plasma a decreased response of luteinizing hormone and follicle-stimulating hormone to luteinizing hormone-releasing hormone. This patient may be the first case of prepubescent null cell macroadenoma confirmed with immunohistochemical study in addition to the findings of classical histopathology.

In this study, we compared the clinical and endocrinological characteristics, neuroimaging findings, surgical outcome, and conventional histological findings (including immunohistochemistry) with the electron microscopic appearance of 31 growth hormone (GH)-producing adenomas. By electron microscopy, these 31 tumors were divided into 23 densely granulated somatotroph adenomas (DG adenomas) and 8 sparsely granulated somatotroph adenomas (SG adenomas). SG adenomas more frequently affected younger women, but no significant correlation was found between the adenoma type and the characteristic signs and symptoms of acromegaly, the incidence of diabetes mellitus or hypertension, or the basal serum GH and insulin-like growth factor I levels. A distinct response of GH to thyrotropin-releasing hormone, bromocriptine, or GH-releasing hormone was significantly more common in patients with DG adenomas than in those with SG adenomas, whereas the incidence of a response to gonadotropin-releasing hormone or oral glucose was not significantly different between the two groups. An analysis of neuroimaging findings and surgical results indicated that SG adenomas were more likely to be macroadenomas with suprasellar extension or invasive tumors and had a lower surgical cure rate. However, postoperative radiotherapy seemed to be similarly effective in both types of adenoma to prevent a tumor recurrence and to reduce postoperative GH basal level in serum. Light microscopy showed that DG adenomas were mainly acidophilic and were immunopositive not only for GH but also for prolactin (43%), the beta subunit of thyroid-stimulating hormone (26%), and the alpha subunit of glycoprotein hormone (87%), whereas SG adenomas were almost all chromophobic and only revealed immunopositivity for GH.(ABSTRACT TRUNCATED AT 250 WORDS)

trans-4-Acetylaminostilbene (AAS) is a complete carcinogen in rats and produces quite selectively tumors in Zymbal's glands. On the basis of DNA adduct formation, it has been proposed that this model arylamine initiates neoplastic transformation of cells in many tissues, particularly liver and kidney, which, in the classical sense are considered to be non-target tissues for this chemical. In the present study an initiating treatment with AAS was followed by unilateral nephrectomy and the application of two nephrotoxic substances, gentamycin or beta-cyclodextrin which, among other activities, stimulate cell proliferation specifically in kidney. The initiating dose of AAS, given alone, gave rise to Zymbal's gland and mammary tumors in female Wistar rats within 88 weeks but not to liver or kidney tumors. When the initiation treatment was followed by unilateral nephrectomy, alone or in combination with gentamycin, or by beta-cyclodextrin, four tumors in two out of ten animals, eight tumors in three/ten, and seven tumors in three/ten, respectively, were observed in the kidney. The administered dose of gentamycin was not sufficient to induce tumors on its own. The results support the view that the genotoxic effects of AAS produce promotable lesions in rat kidney. None of the animals that had been treated with AAS, with or without other treatments, developed tumors or the predominant types of preneoplastic lesions in the liver within 88 weeks; this supports the notion that liver, like kidney, is not a target for complete carcinogenesis for this chemical.

There is general agreement that postoperative radiation therapy is beneficial for patients with subtotally resected pituitary adenomas. We have identified 41 such patients treated during a 20-year period who received postoperative irradiation for a pituitary adenoma. The usual dose was 5040 cGy in 28 fractions. The mean follow-up time was 10.3 years. On routine hematoxylin and eosin (H&E) staining, there were thirty-three chromophobe, seven eosinophilic, and one basophilic adenoma. Tissue blocks were stained for growth hormone (GH), luteinizing hormone (LH), thyroid-stimulating hormone (TSH), prolactin (PRL), and/or adrenocorticotropin (ACTH) using the peroxidase-antiperoxidase immunohistochemistry (IHC) method. Routine H&E staining was a poor predictor of the IHC stain. While most patients with a known clinical endocrine syndrome stained positive on IHC for the suspected offending hormone, many patients without a clinical syndrome also stained positive indicating the presence of hormonally occult adenomas in this locally invasive group. The IHC stain results were compared to clinical outcome. The presence of positive GH IHC staining decreased the 15-year progression-free survival (PFS) from 100% to 64% compared to GH negative adenomas (p = 0.06). There was a trend toward decreased 15-year PFS in patients who did not stain for LH. Positive staining for prolactin, ACTH, or TSH had no influence on the progression-free survival. We conclude that additional prognostic information can be obtained in this subset of patients (by performing IHC analysis) that is not known by the clinical presentation or appearance on H&E stain.

Laparoscopic adrenalectomy has become the standard procedure for treating patents with adrenal masses. The purpose of this study was to evaluate the use of the LigaSure vessel closure system during laparoscopic adrenalectomy.The LigaSure device was used in 32 patients undergoing laparoscopic adrenalectomy for adrenal masses. Adrenalectomy was carried out without the use of clips or sutures for vessel closure. In 1 patient the adrenal tumor had invaded the ipsilateral kidney, so laparoscopic nephrectomy was also carried out during the same operation. In another patient, a renal cell carcinoma in the left kidney had metastasized to the right adrenal gland. Both the kidney and the contralateral adrenal gland were removed laparoscopically during the same operation.Adrenal masses had a mean greatest diameter of 3.48 cm (range 2 to 11 cm). Mean operative time was 83.2 minutes (range 30 to 190 min). Mean blood loss was 36.2 mL (range 10 to 140 mL). No conversions to open surgery were necessary. No patients experienced major bleeding intraoperatively or postoperatively. Adrenal tumor types included adrenocortical adenoma (16 patients), pheochromocytoma (13 patients), malignant pheochromocytoma (1 patient), chromophobic carcinoma (1 patient), and metastasis from a renal cell carcinoma (1 patient).For vessel closure during laparoscopic adrenalectomy, the LigaSure device seems to be safe and effective. For patients with conditions such as renal cell carcinoma combined with metastasis to the contralateral adrenal gland, nephrectomy, and contralateral adrenalectomy can be carried out during the same laparoscopic operation.

Cranial irradiation may lead to accelerated atherosclerosis over several years. Stroke has been described after cranial irradiation administered for a number of conditions. However, pituitary irradiation has only rarely been associated with stroke. Two patients, 39 and 46 years of age, suffered strokes 13 and 20 years, respectively, after irradiation for pituitary tumors. Strokes were in the territories of small perforating arteries, but large vessels such as the carotid siphon and anterior cerebral arteries were also abnormal. Other risk factors for stroke were absent. It is suggested that pituitary irradiation increases the risk of subsequent stroke due to the known effects of ionizing radiation on vascular walls.

A case of interposed nerve graft repair of an oculomotor nerve damaged during surgery for a laterally growing retrosellar pituitary adenoma is reported. The patient showed partial but functionally useful recovery of nerve function and was able to resume professional work. Only a few reports of end-to-end repair have been published, and they indicate that partial return of nerve function can be expected in such cases.

A patient with an FSH secreting pituitary adenoma is reported. Elevated FSH and serum free alpha-subunit (SU) with low levels of LH and testosterone (T) were found. Immunostaining showed the presence of alpha-SU, FSH-beta and LH-beta subunits. LHRH analogue (buserelin) was administered in a pulsatile manner, by portable computerized infusion pump sc for ten days. During the first 24 h of treatment FSH, LH (p less than 0.001) and T (p less than 0.01) rose significantly. Ten days later, the expected desensitization phenomenon did not occur, but further increases of T (8.4 +/- 2.6, mean +/- SD, vs 17.4 +/- 4.1 nmol/l, p less than 0.001) and FSH (58.9 +/- 9.6 vs 70.7 +/- 3.8 mlU/ml, p less than 0.001) were registered. LH decreased (12.5 +/- 2.4 vs 7.1 +/- 0.6 mlU/ml, p less than 0.001) at day 10, but remained higher than basal level (5.0 +/- 0.6, p less than 0.001). Free alpha-SU also rose (2.8 +/- 0.4 vs 4.4 +/- 1.7 mlU/ml, p less than 0.001) after ten days of treatment. The chronic stimulatory effect of analogue on LH with a lack of desensitization suggests tumorous secretion despite a partially preserved negative feedback of testosterone. Low basal LH levels, in some patients with FSH secreting tumors may not be due to tumor mass effect, but rather may be the consequence of altered LH production and/or secretion by the tumor. Although buserelin may not have a therapeutic effect, it is of use in differential diagnosis of hypergonadotropinemia.

Between 1967 and 1985 95 patients with pituitary adenoma received radiation therapy at the University of California, Los Angeles (UCLA), and the West Los Angeles Veterans' Administration (VA) Medical Center. Seventy of these patients received radiation therapy immediately as adjuvant therapy following incomplete resection, and 25 were irradiated as primary treatment or after surgical failure. The majority of cases (87%) presented clinically with macroadenomas. Two-thirds of the patients were treated with parallel opposed lateral portals to a midline total dose of 50 Gy. The mean follow-up was 7 years (range: 30-250 months). In our series, there is a difference in response by disease subtype. The control rates were 83% (30 of 36) for nonfunctioning adenoma, 60% (nine of 15) for growth-hormone secreting adenoma, 44% (16 of 36) for prolactin secreting adenoma, three of five for Nelson's syndrome, and three of three for Cushing's disease. There was a tendency for a better response rate with a total dose of greater than or equal to 50 Gy for prolactinoma. None of the patients were known to develop brain necrosis or radiation-induced sarcoma. An analysis of these results and a review of the literature is presented here.

[Abstract] Cancer is a heritable disorder of somatic cells: environment and heredity are both important in the carcinogenic process. The primal force is the “two hits” of Knudson’s hypothesis, which has proved true for many tumours, including renal cell carcinoma. Knudson et al. [1, 2] recognised that familial forms of cancer might hold the key to the identification of important regulatory elements known as tumour-suppressor genes. Their observations (i.e., that retinoblastoma tend to be multifocal in familial cases and unifocal in sporadic presentation) led them to propose a two-hit theory of carcinogenesis. Furthermore, Knudson postulated that patients with the familial form of the cancer would be born with one mutant allele and that all cells in that organ or tissue would be at risk, accounting for early onset and the multifocal nature of the disease. In contrast, sporadic tumours would develop only if a mutation occurred in both alleles within the same cell, and, as each event would be expected to occur with low frequency, most tumours would develop late in life and in a unifocal manner [3, 4]. The kidney is affected in a variety of inherited cancer syndromes. For most of them, both the oncogene/tumour-suppressor gene involved and the respective germline mutations have been identified. Each of the inherited syndromes predisposes to distinct types of renal carcinoma. Families with hereditary predisposition to cancer continue to provide a unique opportunity for the identification and characterisation of genes involved in carcinogenesis. A surprising number of genetic syndromes predispose to the development of renal cell carcinoma, and genes associated with five of these syndromes have been already identified: VHL, MET, FH, BHD and HRPT2. Few cancers have as many different types of genetic predisposition as renal cancer, although to date only a small proportion of renal cell cancers can be explained by genetic predisposition.

Contemporary radiologic imaging has resulted in an increasing number of smaller renal cortical tumors being identified. The ability of imaging to classify these tumors is limited, although certain features may help classify the renal cortical neoplasm. The important role of radiologic imaging in tumor detection, characterization, staging, and follow-up of patients who have renal cortical tumors is reviewed in this article.

In both cases optic disc neuropathy with perimetry defects and loss of vision is caused by a cerebral tumour. The progression of optic damage was stopped by resection of the tumor in both patients. We recommend the performance of a radiological examination in patients with visual field defects if the intraocular pressure is normal and thus glaucoma may not be the cause of the defects.

Three patients who developed unilateral ptosis followed by partial third nerve palsy were found to have a pituitary tumour. The visual field defects were minimal and asymptomatic. Two patients had a chromophobe adenoma and one patient had a prolactinoma. The importance of recognising a pituitary tumour as the cause of acquired unilateral ptosis is emphasised.

Pituitary tumours originating primarily from sites other than the sella turcica are rare. A case of an ectopic pituitary tumour in the region of the lesser wing of the sphenoid is reported. The patient presented with signs and symptoms of a progressive increase in intracranial pressure. CT scan appearances resembled those of a sphenoid wing meningioma. The vascular lesion was partially excised. Histology showed it to be a pituitary tumour.