Pituitary macroadenoma with persisting dense lymphocytic infiltration in a young male patient.

CONTEXT: Lymphocytic anterior hypophysitis in association with a pituitary adenoma was reported previously. In rare instance, inflammatory infiltration was confined to adenoma tissue itself, excluding anterior pituitary. CASE: The patient - a 27-year-old male - presented with visual field defect. Further examination revealed a pituitary mass with suprasellar extension. Hormonal evaluation indicated mild hyperprolactinemia (42 ng/ml, normal < 19). After transsphenoidal resection, a pituitary adenoma showing cytoplasmic immunoreactivity to prolactin was identified. Dense and diffuse lymphocytic infiltration was seen within the tumor. At 15th month, a second transsphenoidal operation was necessary because of rapid development of visual compromise and headache. Excised surgical specimen consistent with previously resected adenoma showed diffuse lymphocytic infiltration composed of B and T cells within the adenoma tissue again. CONCLUSION: Presence of dense, hypophysitis-like lymphocytic infiltration within pituitary adenoma tissue obtained by two consecutive operations may reflect an host-mediated immune reaction to tumor. This rare finding could be challenging in terms of differential diagnosis and follow-up course.

Do cyst wall thickness and cyst size have any effect on the intra-operative inadvertent rupture of echinococcal hydatid cyst of central nervous system?

Our primary aim was to investigate whether there is any association between thickness of the hydatid cyst's wall and size and whether it has any effect on intact removal of the cyst, independent of the surgeons' technique. Sixteen cases of echinococcal hydatid cyst of the central nervous system, operated on between 1991 and 2007 by various surgeons, were examined. Cyst diameter was directly measured on the specimen or calculated on radiological scans. Histological preparations were made identically by cutting 5-microm-thick longitudinal sections from paraffin blocks of formalin-fixed tissue and stained with HE. Wall thickness was measured using a micrometer by the same pathologist blinded to the measurements of cyst diameter. Cyst diameter and wall thickness correlated negatively (the larger the cyst the thinner the cyst wall); however this was not statistically significant. Neither the cyst size nor the wall thickness proved to be statistically significant as a factor having an effect on intact removal of the cyst. In conclusion, the cyst size and wall thickness do not have any effect on the intact removal of hydatid cyst. It seems that the use of appropriate surgical techniques play the most effective role in outcome of hydatid cyst surgery.

Microscopic local leptomeningeal invasion at diagnosis of medulloblastoma.

PURPOSE: To document the incidence and outcome of patients with microscopic local leptomeningeal invasion at presentation, children with medulloblastoma were reviewed. METHODS AND MATERIALS: Nineteen patients (1-12 years of age), who had surgical resection (14 total, 5 subtotal) and were diagnosed as medulloblastoma, were evaluated for disease extent by pre- and postoperative computerized tomography/magnetic resonance imaging, histopathologic evaluation of leptomeninges adjacent to the resected tumor tissue, myelography/magnetic resonance of the entire spine, and analysis of cerebrospinal fluid cytology. Patients were also reviewed for disease outcome. RESULTS: Staging revealed one T2, nine T3a, eight T3b, and one T4 disease. There were 10 M0, 2 M1, 2 M2, 2 M3, and 3 Mx patients according to Chang's classification. Ten out of 19 patients (52.6%) demonstrated microscopic local leptomeningeal invasion. When only the 10 patients with M0 disease were considered, three out of five patients with microscopic local leptomeningeal invasion was found to have one or more relapses. The five other M0 patients without microscopic local invasion were in complete remission at the time of analysis. CONCLUSION: The incidence of microscopic local leptomeningeal invasion in patients with medulloblastoma is high. Whereas the impact on survival remains to be determined in larger series, data suggests prognostic role for isolated microscopic local leptomeningeal invasion, thus validity for inclusion in the future staging system.

Baló's concentric sclerosis: clinical and radiologic features of five cases.

Baló's concentric sclerosis (BCS) is a rare demyelinating disease considered to be a variant of multiple sclerosis. Five BCS cases were diagnosed antemortem based on their typical concentric mass patterns on MR images and based on clinical and CSF findings. Histopathologic investigation was also performed in one case. Our case report supports the concept that BCS may be a self-limited disease that is not always fatal. Characteristic MR imaging findings may allow antemortem diagnosis of BCS when performed at the onset of the disease.

Skull base amyloidoma. Case report.

A mass lesion of amyloid involving the central nervous system is a rare finding. A 64-year-old woman presented with a large amyloidoma at the skull base causing neural tissue compression. The only accompanying disease was an asymptomatic renal cyst. The mass had caused destruction of the bone elements and pathological calcification as seen on x-ray films, computerized tomography (CT) scans, and magnetic resonance (MR) images, and was enhanced after injection of contrast medium on both CT scans and MR imaging.

Bromocriptine-induced cerebrospinal fluid fistula in patients with macroprolactinomas: report of three cases and a review of the literature.

Bromocriptine therapy for macroprolactinoma induced cerebrospinal fluid (CSF) rhinorrhea in three patients. The tumor had extended well beyond the sella turcica and caused bony erosion in all the cases. All three patients responded to bromocriptine therapy rapidly. CSF fistula occurred concomitantly with the reduction of tumor size and caused meningitis in two of the patients. Withdrawal of bromocriptine resulted in cessation of the leakage. One of the patients underwent transsphenoidal repair. Two patients refused surgery. This potentially lethal complication encountered in these three cases demonstrates the need for close supervision of macroprolactinoma patients with skull base erosion placed under bromocriptine therapy.

Stereotactic surgery in the management of brain abscess.

BACKGROUND: Bacterial brain abscesses can be diagnosed and treated with stereotactic aspiration. METHODS: From 1991 to 1997 we have used computed tomography-guided stereotactic aspiration to diagnose and treat 21 patients with a total of 58 bacterial brain abscesses. The ages of the patients ranged from 4 to 72 years (median 25 years); 11 of these 21 patients had multiple abscesses. The number of abscesses per patient with multiple abscesses ranged from 2 to 9, all located deep in subcortical white matter. RESULTS: All patients underwent stereotactic surgical drainage and an 8-week intravenous antibiotic medical treatment. Of the 58 abscesses, 23 were aspirated. Of these 23 abscesses, 19 were radiologically stage III or IV and four were stage I or II. Pathological examination confirmed radiological staging in 19 patients (83%). Except for the three patients who have mild residual hemiparesis and one patient recovering from ataxia, all patients had complete neurological recovery. CONCLUSIONS: Computed tomography-guided stereotaxy achieved all the objectives of management; namely, ascertaining the diagnosis, draining the content of the mass, and obtaining pus for accurate bacteriological diagnosis without morbidity. Stereotactic aspiration combined with an 8-week intravenous antibiotic regimen has yielded an effective therapeutic result in all of our abscesses, small or large, solitary or multiple, superficial or deep-seated. A high radiological-pathological correlation was also deduced from this study.

[Intracerebral solitary plasmacytoma. Apropos of a case possibly induced by head injury]. / Plasmocytome solitaire intracérébral. A propos d'un cas peut-être induit par un trauma crânien.

We report a rare occurrence of an intraparenchymal plasmocytoma. On the past history of a severe head injury twenty years ago and the presence of a porencephalic area on the computerized tomography scan and magnetic resonance imaging we suspected a possible trauma-tumor relation. Accuracy of the histological diagnosis of plasmocytoma was confirmed. The treatment was a surgical excision only, and after a 4.5 years follow-up period, no clinical nor radiographic recurrence was noted.

Gliomatosis cerebri with neurofibromatosis: an autopsy-proven case.

Gliomatosis cerebri is a glial neoplastic process that is diffusely distributed through neural structures, whose anatomical configuration remains intact. Among the more than 19,000 cases hospitalized in Istanbul University Istanbul School of Medicine Department of Neurosurgery throughout the past 45 years, only 2 cases were diagnosed as gliomatosis cerebri, 1 by stereotactic ante-mortem diagnosis and the other after autopsy. In this paper, the autopsy-proven case of this rare disease with coexistent neurofibromatosis--the sixth case reported in the literature--is presented.

Micro-invasive squamous cell carcinoma arising in a pre-existing intraventricular epidermoid cyst. Case report and literature review.

The authors report the case of a 67-year-old woman with primary micro-invasive squamous cell carcinoma located in the lateral ventricle and originating from an epidermoid cyst. Radiological and histological features of the neoplasm are described and the relevant literature is studied briefly. Two consecutive surgical specimens were studied by light microscopy. In the histological sections of the subtotally removed material of the first operation, the cyst wall was layered by epithelium-the same as expected in an epidermoid cyst- and showed foci with mild to moderate dysplastic changes. Theses changes did not reach up to the degree of a carcinoma. However, because the cyst could not be resected totally, the possibility of a squamous cell carcinoma was considered in the differential diagnosis. In the present case, the diagnosis was firmly established in a second surgical specimen obtained ten months after the first operation. Now, the tumour proved to be a primary squamous cell carcinoma, which exhibited severe dysplastic changes. Primary squamous cell carcinoma of brain is extremely rare. To our knowledge, only 20 such tumours with pre-existing epidermoid cyst have been reported in the central nervous system. The intriguing observation in this case is a three year recurrence free survival following the subtotal removal of the malignant neoplasm at the second surgery, without postoperative radiotherapy.

Interstitial irradiation for craniopharyngioma.

The results of interstitial irradiation treatment for craniopharyngioma in two patients with six year follow-ups are presented. Stereotactic interstitial irradiation with Iodine-125 sources as sole therapy was employed in two adult patients who refused surgical resection. The diagnoses were confirmed by stereotactic biopsy. The first tumour which underwent interstitial irradiation was solid and 4 cm in diameter, and the second, 2.7 cm in diameter, had both cystic and solid components. The implanted Iodine-125 seeds delivered 67 Gy and 60 Gy to tumour periphery at the rate of 12 and 14 cGy/h, respectively, were removed at the end of designated radiation periods. Tumour shrinkage and central hypodensity, first observed 3 months after irradiation, continued until one tumour shrank to less than 1 cm at 12 months, and the other disappeared completely at 24 months. In both cases functional integrity was restored, and neither radiation induced toxicity nor recurrence has occurred six years after treatment. The results in these two cases suggest that solid craniopharyngiomas are sensitive to interstitial irradiation.

Prolactin-secreting carcinoma of the pituitary: clinicopathological and immunohistochemical study of a case with intracranial and intraspinal dissemination.

A 32-year-old female patient with a primary adenohypophyseal neoplasm that rapidly progressed to a fatal outcome is presented. The time interval between her admission to the hospital and her death was 3 months. Despite dopamine agonist therapy, local invasion as well as frontal and spinal cord metastases at Th 10-12 region developed, and four surgical resections were performed. The serum prolactin levels were high. Both the primary pituitary tumour and all the metastatic tumours had the same histological findings and immunohistochemical reactions. Each was composed of pleomorphic chromophobic cells with enlarged nuclei. Mitoses and necroses were frequent. Immunostains revealed prolactin in the tumour cells. A literature review revealed that in most of the pituitary carcinomas as in our case hyperprolactinaemia did not respond to medical therapy and the histopathological appearance of the tumour has not correlated with the aggressive behaviour of the tumour. It may therefore be considered that at least some of the cases with metastases in prolactin secreting pituitary carcinomas could be the result of hyperprolactinaemia itself.

Nucleolar organizer regions in pituitary gland adenomas.

Nucleolar organizer regions are DNA segments concerned with the synthesis of ribosomal RNA (rRNA). Some specific proteins related to NORs can be demonstrated by a silver technique and are then referred to as Ag-NORs. The mean number of Ag-NORs per cell has been used as a marker of cell proliferation in recent years and there is some evidence to suggest that Ag-NOR counts can be used to differentiate between normal and neoplastic cells. Paraffin block sections of 42 pituitary gland adenomas and 24 normal adult pituitary glands have been examined using the Ag-NOR staining technique. Touch imprints of nine adenomas and 12 normal adult pituitary glands were also examined using the same Ag-NOR staining technique. Using light microscopy at x2000 magnification, the Ag-NORs were located as black dots in the cell nuclei and the mean number of Ag-NORs per cell was determined. No significant difference was observed between blocked sections and imprints of adenomas or between imprints and paraffin sections of normal glands. However, a significant difference was observed between the mean number of Ag-NORs in pituitary gland adenomas and normal glands in paraffin block sections and touch preparations (P less than 0.001). The modified Ag-NOR technique can be a useful aid in the differential diagnosis between normal pituitary gland tissue and pituitary adenomas, especially during surgical operations.

Anaplastic pleomorphic xanthoastrocytoma.

A case of anaplastic pleomorphic xanthoastrocytoma (PXA) in a 9-year-old girl is reported. Histological features of PXAs are cellular pleomorphism of GFAP-positive cells, with intracytoplasmic lipidic vacuoles and a reticulin network, bizarre giant cells, low mitotic activity, and lack of necrosis and of endothelial vascular proliferations. These tumors are generally reported to have a favorable postoperative course. In our case, a poor clinical prognosis and spread of the illness through the CSF was observed. Immunohistochemical features of the tumor, which were histologically anaplastic in nature, were analyzed. There were small foci of necrosis in the sections of the material obtained at the first operation and extensive necrosis in that from the second operation, although the patient had not received radiotherapy between the operations. The presence of necrosis in PXA is an uncommon and significant feature. It predicts the poor prognosis seen in this case, and therefore this report strongly supports the notion that necrosis should automatically exclude a tumor from the PXA category. The histological grade was evaluated as grade 3 (according to the WHO classification).

Non-resective management of pineoblastoma.

The results of a non-resective treatment approach for pineoblastoma comprising stereotactic biopsy, cerebrospinal fluid diversion, and fractionated radiotherapy in six patients over a period of six years are presented. There were three male and three female patients, with a median age at diagnosis of 20 years. Magnetic resonance imaging of the spine, ventricular cerebrospinal fluid cytology, and tumour markers in cerebrospinal fluid were negative. Tumour response to initial radiotherapy was complete in three patients and partial in three patients. Recurrences were treated with interstitial irradiation with iodine-125 seeds in four instances, repeat radiotherapy when time elapsed was more than five years in one instance, with surgical resection in two instances, and chemotherapy in two instances. The diagnostic and therapeutic effectiveness of this management strategy is assessed. There were no complications related to surgical procedures. The median follow-up time was 48 months (range 14-70 months). Five patients were alive at 14, 45, 51, 57, and 70 months of follow-up. One patient died of disease at 28 months following diagnosis. The overall survival rate was 80 % +/- 17.89 % at 28 months. The results of this study suggest that this non-resective treatment approach is acceptable as an initial treatment alternative to radical surgical resection of pineoblastomas.

Gliofibroma: immunohistochemical analysis.

A case of gliofibroma occurring in an adult patient as a large circumscribed supratentorial tumor is reported. The bimorphic pattern was substantiated and further analyzed by immunohistochemistry. Some evidence in favor of collagen production by mesenchymal and/or inflammatory cells leading to a progressive fibrous replacement of the glial cells in this particular tumor type is presented.

Optimal therapy in medulloblastoma.

Twenty-eight children with medulloblastoma (19 boys and 9 girls) were treated at the Departments of Neurosurgery and Radiotherapy in Essen between 1969 and 1977, and were followed prospectively after surgical treatment and postoperative irradiation. Because different radiation techniques had been used, these patients were divided into two groups, one given a limited irradiation volume and the other irradiation of the entire central nervous system. The results obtained in the former group were inferior to those in the latter. All living patients in the seond group are in good condition. Special attention is paid to the technique of irradiation and the factors which may influence prognosis. Even after the relatively short follow up period of one to five years, it may be concluded that high-dose irradiation of the entire central nervous system considerably improved the prognosis of medulloblastoma.

A serial CT scan and MRI verification of diffuse cerebrospinal gliomatosis: a case report with stereotactic diagnosis and radiological confirmation.

Gliomatosis cerebri is an infrequent tumor of neuroepithelial origin presenting with deterioration of cognitive functions, behavioral and mental changes, motor weakness, headache, and seizures. Laboratory data are unconclusive. MRI appears to be the imaging modality of choice and mainly reveals a bilateral and diffuse infiltration of midline adjacent brain structures whose anatomical configuration remain intact. MRI- or CT-guided stereotactic biopsy is advised as the diagnostic procedure in suspected cases. A 9-year-old girl with diffuse cerebrospinal gliomatosis, investigated with sequential cranial CT scans, and MRI-verified spinal cord involvement is reported, and the corresponding literature is reviewed.

Emery-Dreifuss muscular dystrophy with unusual features.

Two families with Emery-Dreifuss muscular dystrophy (EMD) are described. Several unusual features for EMD are emphasized. One of the patients had severe neuromuscular disability with inability to walk during early childhood. This patient also had mild bifacial paresis. His brothers had the typical slow progression of EMD. In some of the patients, muscle weakness distribution was more widespread than has usually been reported, with prominent involvement of finger extensors. It is suggested that there is a wide phenotypic spectrum in EMD. In both families, the disease segregated with markers spanning the EMD locus in Xq28.

Evaluation of response to postradiation eight in one chemotherapy in childhood brain tumors.

Ten children, 3 to 15 years of age with high risk primary brain tumors were treated with postradiation 'eight in one' chemotherapy; vincristine, lomustine, procarbazine, hydroxyurea, cisplatin, cytosine arabinoside, cyclophosphamide and methylprednisolone. The tumors comprised of three medulloblastomas, two primitive neuroectodermal tumors, one ependymoblastoma and four anaplastic ependymomas. Treatment involved surgery (two total resection, six subtotal and two biopsy only) followed by conventional radiotherapy (primary tumor: 50-54 Gy, whole brain: 30-45 Gy, and spinal axis: 25-36 Gy). Objective tumor response with radiotherapy was achieved in 7 of 9 patients (78%) (6/8 patients with residual tumor and one patient with complete resection but positive cerebrospinal fluid cytology). Complete response was attained in 4 of 9 patients (44%). 'Eight in one' chemotherapy was initiated four weeks after radiation and repeated at 4 weekly intervals for 5-8 courses. Postradiation 'eight in one' failed to show any additional effect on tumor responses. Median survival was 34 months (range 9-48 months) with five of ten patients alive: four in complete and one in partial remission. All the five survivors were among the patients who had achieved response to initial treatment. This result suggested that degree of response to initial treatment might determine subsequent outcome and thus the choice of modality for initial therapy might be important.