Cerebral solitary Langerhans cell histiocytosis: report of two cases and review of the literature.

Cerebral solitary Langerhans cell histiocytosis (LCH) is a very uncommon condition. We describe two new cases: a 30-year-old man with seizures and a tumour in the left frontal lobe, which was composed of a polymorphic infiltrate with a predominance of histiocytes and eosinophils; and a 65-year-old man with headaches and dysarthria, with a left parietal tumour, which showed a diffuse proliferation of histiocytic cells and areas of necrosis. In both cases, the histiocytes were strongly positive for S-100 and CD1a, and Birbeck's granules were demonstrated by electron microscopy in the first case. Both patients underwent a complete excision of their lesions. The second patient received additional postoperative radiotherapy. They were asymptomatic after 26 and 27 months, respectively. It seems that cerebral solitary LCH is a clinicopathological entity with a good outcome. Only 15 cases of this rare process have been previously reported in the English literature.

Intraorbital arteriovenous malformation: case report.

An intraorbital arteriovenous malformation became symptomatic without exophthalmos but with chemosis and bruit of the left eye. The patient underwent only surgical treatment. The orbital rim was removed through a fronto-orbital approach. The chemosis was corrected and postoperative magnetic resonance imaging and angiography confirmed complete removal of the malformation. Histopathological examination disclosed an arteriovenous malformation in the extrabulbar fatty tissue without invasion of the extraocular muscles or optic nerve. The differences between various types of intraorbital malformations are discussed.

[Assessment of the degree of resection of high grade supratentorial gliomas with early postoperative magnetic resonance]. / Valoración del grado de resección de los gliomas supratentoriales de alto grado con resonancia magnética postoperatoria precoz.

UNLABELLED: In this paper we report the results of a prospective study in which we evaluate the degree of tumor removal of 25 supratentorial high grade gliomas by means of an MRI performed in the early postoperative period. In all cases, there was preoperative enhancement 8 patients had been previously operated on while the others had their first operation. In all cases the postoperative early MRI was performed within the first week and in 15 within the first 3 days. In order to evaluate the degree of tumor removal the presence or not of enhancement was considered and if it existed, classified as linear or nodular. RESULTS: To avoid postsurgical artifacts it is important to perform the MRI as soon as possible after surgery, especially within the first 3 days. Generally, linear enhancement disappeared on subsequent follow-up examinations, showing that it probably does no represent residual, tumor while nodular enhancement usually does. Survival with the high-grade tumors was slightly higher in the group without postoperative enhancement or linear one than in the group with nodular enhancement but the difference was not significant.

[Prognosis value of p53 gene and cellular proliferation factors in malignant supratentorial gliomas]. / Significación pronóstica de los factores de proliferación celular y del gen p53 en gliomas malignos supratentoriales del adulto.

OBJECT: The aim of this study was to investigate prognosis value of p53 oncosuppresor gene and labeling index by MIB-1 and PCNA monoclonal antibodies in malignant gliomas. METHODS AND RESULTS: We operated 60 patient with malignant glioma between July 1994 and August 1998. Forty-five cases had the histopathological diagnosis of Glioblastoma Multiforme and fifteen cases of anaplastic astrocytoma or olygoastrocytoma. Initially we compare MIB-1/PCNA labeling index in eighteen cases. Patients < 40 years old, short period of symptoms preoperatively, only one cerebral lobe localization, total surgical exeresis, Karnofsky index > 70 postoperatively, low labeling index (MIB < 8.6%), forward radiotherapy treatment and surgical re-operation, were identified like favourable outcome factors in the uninvariable analysis. However to the multivariable analysis, only had outcome significance: patients < 40 years old, low labeling index, Karnofsky index > 70 postoperatively and surgical reoperation. CONCLUSIONS: On the basis of the results of current analysis, moreover classical factors associated with better outcome, low labeling index to Glioblastoma Multiforme have been noted in patients with longer survival, and must be included to conventional histopathological studies.

Valoración del grado de resección de los gliomas supratentoriales de alto grado con resonancia magnética postoperatoria precoz / Assessment of the degree of tumor removal in supratentorial high grade gliomas by early postoperative magnetic resonance imaging

En este trabajo se presentan los resultados de un estudio prospectivo consistente en valorar el grado de resección de 25 gliomas supratentoriales de alto grado mediante RM realizada en el postoperatorio inmediato. En todos los casos existía captación de contraste preoperatoria. 8 pacientes habían sido ya previamente intervenidos mientras que el resto fue una primera intervención. En todos los casos la RM postoperatoria fue practicada en la primera semana tras la intervención y en 15 casos en los 3 primeros días. Para valorar el grado de resección conseguido, se tuvo en cuenta la presencia o no de captación de contraste y en el caso de existir ésta, se clasificó en lineal y nodular. Resultados: para conseguir imágenes libres de artefactos y vaIorables, es importante realizar la RM postoperatoria de forma lo más precoz posible, preferentemente en los 3 primeros días. En general, la captación lineal desapareció a lo largo del seguimiento, demostrando no estar probablemente en relación con el resto tumoral, mientras que la captación nodular casi siempre demostró revelar un resto tumoral. La supervivencia para los tumores de alto grado fue ligeramente mayor en el caso de que en la RM postoperatoria precoz no haya captación o bien ésta sea lineal que en el caso de que sea nodular, pero sin que existan diferencias significativas (AU)

[Low-grade oncocytic adrenal carcinoma]. / Carcinoma suprarrenal oncocítico de bajo grado.

OBJECTIVE: To report a case of left adrenocortical neoplasm. METHODS: A case of left adrenocortical neoplasm measuring 16 x 10 x 7 cm in a 47-year-old male is presented. After puncture biopsy, a left adrenalectomy through the abdominal approach was performed. RESULTS: Immunohistochemical studies showed reactivity to the anti-Melan-A A103 antibody, suggesting an adrenal origin of the tumor cells. Strong reactivity to the mES-13 mitochondrial antibody, which is characteristic of oncocytes, made ultrastructural studies unnecessary. CONCLUSIONS: In this type of tumor, an increased mitotic activity and necrosis are suggestive of malignancy. Despite the foregoing, most of this type of lesions are clinically benign and nonfunctioning.

[Intravesical hemangiopericytoma]. / Hemangiopericitoma intravesical.

OBJECTIVE: To report on a case of hemangiopericytoma of the urinary bladder mimicking a urothelial tumor. METHODS: A case of hemangiopericytoma arising from the urinary bladder in a 78-year-old man is described. The initial symptoms included gross hematuria, and it was confused with a urothelial tumor. The difficulty in making the diagnosis in regard to the site of origin and histological findings are discussed. The literature is briefly reviewed. RESULTS/CONCLUSIONS: Ultrasound and urographic evaluation showed an intravesical mass which was suspected to be a urothelial tumor. The patient was submitted to transurethral surgery. Histological analysis and immunohistochemical techniques demonstrated a hemangiopericytoma.

Myofibroblastoma of the breast with diverse histological features.

We report two cases of myofibroblastoma with unusual pathological features, in a 66-year-old woman and a 49-year-old man. Both tumours were unilateral, grossly nodular and well circumscribed, but not encapsulated. The lesions were made up of bipolar spindle cells arranged in fascicular clusters separated by bands of hialinized collagen; one included several islands of mature cartilage next to fat cells. The other contained atypical mononucleated and multinucleated giant cells. No mitotic figures were observed. Immunohistochemically, both tumours showed strong and diffuse cytoplasmic staining for vimentin and CD 34 and focal positivity for alpha-smooth muscle actin, and both were negative for cytokeratins, CD 68, Ham 5, 6, Mac 387, and S-100 protein. Desmin was positive in one case. Ultrastructural study revealed populations composed of fibroblastic cells without signs of myofibroblastic differentiation in one case; the second featured abundant undifferentiated mesenchymal cells with myofibroblastic differentiation. Both patients remain disease-free 38 and 36 months after lumpectomy.

[Primary intestinal T lymphoma with massive eosinophilia]. / Linfoma T primario de intestino asociado a intensa eosinofilia.

Primary intestinal T lymphoma with eosinophilia is an infrequent tumour. Its rarity account for the paucity of similar cases recorded in the literature. It affects predominantly middle-aged men and may be associated with ulceration, fistula formation and intestinal perforation with an abdominal mass. Lesions of the intestine with massive tissue eosinophilia may be a difficult diagnostic problem, but this entity displays distinctive histological features. The presence of intense tissue eosinophilia is a T-cell dependent lymphomas response. We describe a case of T lymphoma involving small intestine with massive eosinophilia.

[Villous adenoma of urinary bladder. Report of a new case]. / Adenoma velloso de vejiga urinaria. A propósito de un nuevo caso.

We report on an 80-year-old female patient with a villous polyp of the bladder. We believe this lesion may correspond to an intermediate stage in the well known sequence of injury-metaplasia-dysplasia-carcinoma of the large intestine. Intestinal metaplasia in the bladder is a potentially malignant lesion. If villous adenomas are diagnosed early, before malignant transformation, simple excision can achieve cure.

[Nonamyloid light-chain deposit disease. A report of 2 cases]. / Enfermedad por depósito de cadenas ligeras no-amiloides. Presentación de dos casos.

Two cases of non-amyloid light chain deposit disease (EDCL) which were diagnosed by liver biopsy are presented, and the recent literature is reviewed. This disease is a dysproteinemia which shares numerous clinical and pathological features with amyloidosis. A commentary is made on the differential diagnosis between both diseases. This disease must be suspected when the conventional histochemical and immunohistochemical stains for amyloid are negative. Immunohistochemical assays for kappa and lambda chains should then be performed.

[Presentation and variants of heterotopic salivary tissue in the neck]. / Presentación y variantes del tejido salivar heterotópico en el cuello.

Dealing with a case of salivary tissue in the lower neck in a 5 year-old boy. The AA. consider the anatomoclinical and embryologic features which should be emphasized on this type of heterotopia, as well when malignancy supervenes.

[Anaplastic tumor of the bladder with neurosecretory granules and benign courses]. / Tumor vesical "anaplásico" con gránulos neurosecretorios y evolución benigna.

We report on a 59-year-old female patient with a bladder tumor that was initially classified as anaplastic (undifferentiated) tumor. Posteriorly, electron microscopic and immunohistochemical analyses of the specimen revealed a neuroendocrine tumor distinct from a paraganglioma. The origin and prognosis of bladder carcinoids and small cell undifferentiated carcinomas are discussed. The authors indicate that the level of bladder wall infiltration is the most important prognostic parameter in these type of tumors.

Prognostic implications of proliferative activity and DNA aneuploidy in colonic adenocarcinomas.

Paraffin-embedded surgical specimens from 120 patients who underwent resections for primary untreated colonic adenocarcinoma were examined for proliferative activity, DNA aneuploidy, DNA index, and the proportion of aneuploid cells by flow cytometry. The results were correlated with survival times and clinical characteristics of the patients. The presence of metastases, both distant and restricted to local lymph nodes, was found to be a more potent adverse prognostic indicator than any DNA flow cytometry-derived parameter. Additional analyses were performed following stratification of patients into two groups on the basis of presence or absence of metastases. Analysis of 60 patients without metastatic involvement revealed a significant correlation between high proliferative activity, defined as more than 20% of cells in S-phase, and DNA aneuploidy. In fact, high proliferative activity was shown to be a more powerful adverse prognostic indicator in relation to survival than DNA aneuploidy in these cases after multivariate analysis. These results suggest that differences in proliferative activity may be an important biologic factor operative in the variable prognosis seen in colonic adenocarcinoma. In addition, they re-emphasize the importance of stratifying patients into groups based on metastatic involvement when evaluating other possible prognostic features in this disease.