Epstein-Barr virus--positive undifferentiated thymic carcinoma in a 12-year-old white girl.

Thymic epithelial malignant diseases are extremely rare in children. The authors report a 12-year-old white girl admitted for a polymetastatic tumor of the anterior mediastinum. Tumor proliferation was typical of an undifferentiated thymic carcinoma. A close link between Epstein-Barr virus (EBV) and the tumor was established by a high titer of anti-VCA IgA and the presence of EBV RNA and DNA in the tumor. In addition, monoclonal viral episomes were present in tumor cells, indicating that EBV infection was an early event in the oncogenic process. The patient died despite resection, irradiation, and chemotherapy.

[Solitary fibrous tumor of the meninges]. / Tumeur fibreuse solitaire des méninges.

We report a case of solitary fibrous tumor (SFT) of the meninges. SFTs have been initially described in the pleura. SFTs show similar histological findings as in other locations. SFTs show a diffuse positive staining for vimentin and CD34. Meningeal SFTs have usually a favourable outcome. These tumors have to be essentially distinguished from hemangiopericytomas and fibrous meningiomas. Immunostaining for CD34 is of value for this purpose. CD34 expression is often patchy and weaker in hemangiopericytomas whereas it is rarely observed in fibrous meningiomas. It is of great interest to isolate SFTs from hemangiopericytomas because of their favourable outcome.

Pyothorax-associated lymphoma: relationship with Epstein-Barr virus, human herpes virus-8 and body cavity-based high grade lymphomas.

Pyothorax-associated lymphoma (PAL) is a newly-described entity developing several decades after artificial pneumothorax treatment for pulmonary or pleural tuberculosis. It is known to be associated with Epstein-Barr virus (EBV) with constant expression of the two latent membrane proteins: latent membrane protein (LMP)-1 and EBV-associated nuclear antigen (EBNA)-2. We are reporting three new cases of PAL. All of the tumours were of B-cell lineage and classified as large-cell diffuse lymphomas according to the International Working Formulation for the Classification of Lymphomas. The EBV genome was detected in two of the cases with LMP-1 and EBNA-2 expression. No EBV could be detected in the third case suggesting that different mechanisms may be involved in the pathogenesis of the disease. Body cavity-based high grade lymphomas (BCBL) represent a new disease, developing mainly in human immunodeficiency virus (HIV) infected patients: the tumoural cells often contain both human herpes virus (HHV)-8 (or Kaposi's sarcoma herpes virus) and EBV genomes, suggesting that these viruses might co-operate in the pathogenesis of the disease. The pleural location and the association of EBV have led to speculation that PAL could also be related to HHV-8 infection. However, no HHV-8 genome could be detected in any of the 14 tested cases already reported in the literature nor in the two cases we studied (one EBV-positive and one EBV-negative), suggesting that PAL and BCBL are two different entities.

The relationship between MIB-1 proliferation index and outcome in pancreatic neuroendocrine tumors.

The expression of the cell-cycle-associated Ki-67 antigen by MIB-1 monoclonal antibody was retrospectively assessed in 35 surgically resected neuroendocrine tumor specimens of the pancreas embedded in paraffin. The MIB-1 proliferation index was correlated with the classification of the neuroendocrine tumors of the pancreas proposed by Klöppel et al. Four prognostic factors showed a significant correlation with MIB-1: local invasion, metastases, tumor differentiation, and production of insulin. However, analysis by the Cox Proportional Hazards Regression Model showed that only local invasion was an independent predictor of outcome. Finally, our study showed a statistically significant increase in the number of deaths and a statistically significant decrease in survival time when the MIB-1 proliferation index was higher than 4%. We conclude that MIB-1 proliferation index is a simple and reliable tool to predict the clinical outcome of the neuroendocrine tumors of the pancreas. The index might be useful for determining the prognosis for an individual because of the significant decrease in survival when the index is higher than 4%.

[Anatomical and clinical effects of neoadjuvant hormonal treatment before radical prostatectomy]. / Effets anatomo-cliniques du traitement hormonal néo-adjuvant avant prostatectomie radicale.

Neoadjuvant endocrine treatment before radical prostatectomy is designed to reduce the positive margin rate and therefore the risk of recurrence. It usually consists of complete androgen blockade for 3 months, but no consensus has been reached concerning the type of blockade and the optimal duration. The main histological changes consist of glandular atrophy with pycnosis and vacuolization of tumour cells. These changes could reflect increased apoptosis. The residual tumour is usually small. PIN become rare, raising the problem of possible chemoprevention. The overall result is a markedly increased frequency of intracapsular tumours and a reduction of at least 50% of positive margins. However, some authors consider that this is simply due to a more detailed histological examination. The frequency of seminal vesicle and lymph node involvement does not appear to be modified. Endocrine therapy could act on the apoptosis-proliferation balance and also decrease the number of circulating cells. However, the intermediate results of randomized studies are contradictory. As improvement in overall survival remains the main objective, only a longer follow-up will be able to clearly determine the value or uselessness of neoadjuvant endocrine treatment before radical prostatectomy.

The role of technetium-99m sestamibi single photon emission tomography in the follow-up of malignant melanoma and the detection of lymph node metastases.

In the follow-up of patients with malignant melanoma treated by surgical resection of the cutaneous tumour, it is important to achieve early detection of possible lymph node metastasis. In many cases, clinical examination alone will not be sufficient. In our study, single-photon emission tomography (SPET) with technetium-99m sestamibi (MIBI) was used in the assessment of 30 patients with previously resected malignant melanoma when the clinical examination raised the suspicion of lymph node metastasis. Using MIBI, 16 out of 17 lymph node metastases were detected and confirmed by histology. No false-positive results were obtained during this prospective study. It is concluded that MIBI scintigraphy may be useful in the early detection of lymph node metastases of malignant melanomas. If our preliminary results are confirmed, early detection of lymph node metastasis of previously resected malignant melanoma by 99mTc-MIBI scintigraphy may have a significant impact on the management of these patients.

Role of oral contraceptives in the growth of a multilobular adenoma associated with a hepatocellular carcinoma in a young woman.

BACKGROUND: The occurrence of hepatocellular neoplasms is frequently reported in young females. The role of oral contraceptives in the development of these tumors is considered. METHODS: A 14-cm tumor was diagnosed in a 24-year-old female who had been taking the contraceptive pill for the last 3 years. Numerous blocks of this lesion were histologically and immunohistochemically analyzed. Some fragments of the lesion were snap-frozen in order to search for sex hormonal receptors and hepatitis B and C virus. RESULTS: On histological examination, the tumor was found to be a hepatocellular, multilobular adenoma with small foci of hepatocellular carcinoma. Neither hepatitis B virus nor hepatitis C virus was found in serum or in the tumor. Nuclear estrogen receptors and progesterone receptors were detected by immunohistochemical analysis in both the adenoma and the carcinoma, but only progesterone receptors were detected by a radio-ligand binding assay in the tumor. CONCLUSION: This finding suggests that the contraceptive pill may stimulate the growth of hepatocellular, multilobular adenomas through the binding of hormonal compounds to their specific receptors within tumoral cells.

Expression of the bcl-2 oncoprotein in meningiomas.

The purpose of this study was to assess the expression of the bcl-2 oncoprotein in meningiomas and to compare it with the phenotype, the Ki-67 proliferative index and the sex hormone receptor status of the tumors. The expression of the bcl-2 oncoprotein was studied by Western blotting and immunohistochemistry. A quantitative study of the Ki-67 proliferative index and the expression of estrogen and progesterone receptors was performed. Western blot detected the bcl-2 oncoprotein in nearly all meningiomas. Immunohistochemistry detected the oncogene in only 43.5% of the cases. Expression of bcl-2 was essentially by spindle cells of transitional and fibrous meningiomas expressing neural cell adhesion molecule. There was neither correlation between the expression of bcl-2 and Ki-67 proliferative index of meningiomas nor statistical concordance between the expression of bcl-2 oncoprotein by meningiomas and their sex hormone receptor protein status. Inhibition of apoptosis could be involved in the growth of meningiomas with a mesenchymal differentiation.

Salivary gland anlage tumor of the nasopharynx: a clinicopathologic and immunohistochemical study of three cases.

The histologic and immunohistochemical features of three congenital pedunculated nasopharyngeal midline masses are reported. The follow-up in all cases was uneventful. The tumors were characterized by solid and cystic squamous nests and ductlike structures focally continuous with the surface squamous mucosa of the tumor. Most of epithelial structures coalesced with densely cellular stroma-like nodules. Immunoperoxidase staining showed the presence of epithelial markers in both spindle cells and epithelial structures. Spindle cells were also reactive to vimentin and smooth muscle actin, revealing their myoepithelial phenotype. Based on these observations, a diagnosis of salivary gland anlage tumor, also called congenital pleomorphic adenoma of the nasopharynx, was made. The similarity of these tumors' architecture and cellular composition to the normally developing salivary gland has led to the hypothesis of a tumorlike, hamartomatous lesion developing in a site in which minor salivary gland tissue occurs. This report reviews 12 identified cases of this tumor, of which all but one (in which the patient died of sepsis) had a favorable outcome. In an infant with respiratory distress and/or feeding difficulties, these tumors must be differentiated from other midline masses such as encephaloceles and teratomas. They appear curable by surgical exeresis only.

[Scrotal panniculitis due to cold: a pseudo-tumoral lesion in the prepubertal child. Report of a case]. / Panniculite scrotale au froid: une lésion pseudo-tumorale chez l'enfant prépubère. A propos d'une observation.

We reported a case of scrotal panniculitis in a 7 year-old boy after exposure to cold by swimming in cold sea water. Scrotal cold panniculitis is an unusual, confined to prepuberal patients. This entity must be known to avoid surgical exploration because injuries subside spontaneously within few weeks.

An immunohistochemical study of the simultaneous expression of bcl-2 and p53 oncoproteins in epithelial tumors of the colon and rectum.

OBJECTIVE: Bcl-2 and p53 genes may participate in a common pathway for regulation of apoptosis. The aims of this study were (1) to study the immunohistochemical expression of the bcl-2 oncoprotein in colorectal tumors, (2) to correlate it with that of p53 protein overexpression, and (3) to compare it with histopathologic prognostic factors, such as TNM classification and grade. DESIGN: Prospective study of expression of bcl-2 and p53 oncogenes in colorectal tumors. We examined 6 colorectal hyperplastic polyps, 33 adenomas, and 61 carcinomas. SETTING: Regional academic medical center. METHODS: An immunohistochemical study with bcl-2 and p53 antibodies was performed on frozen sections of colorectal tumors. The levels of bcl-2 and p53 expression were evaluated using a semiquantitative grading system. Two-color immunohistochemistry was performed to examine the intracellular colocalization of bcl-2 and p53 in all tumors with a strong positivity for both antigens. RESULTS: Bcl-2 was expressed in 28 (85%) of the 33 adenomas, whereas p53 was expressed in only one adenoma, which had areas of in situ carcinoma. Bcl-2 and p53 were each expressed in 43 (70.4%) of the 61 carcinomas. Thirty-one (50%) of the colorectal carcinomas coexpressed the two oncoproteins. There was no correlation between the number of cells expressing bcl-2 and the number expressing p53 in a given carcinoma. No correlation was observed between the expression of bcl-2 or p53 and the established prognostic factor. CONCLUSION: Abnormal bcl-2 oncoprotein expression appears earlier than p53 accumulation in colorectal carcinogenesis. This study suggests that there is more than one sequence and mechanism of bcl-2 and p53 gene deregulation in colorectal carcinomas.

Bronchial carcinoids. A review of 22 cases.

This study investigated 22 patients with histologically proven primary bronchial carcinoids and in particular the relationship between assessment parameters and survival. In each patient, age, sex, smoking habits, mode of presentation, results of diagnostic investigations, methods of treatment and pathological examination of resected specimens were recorded. Follow-up was possible in all patients for at least 5 years. Patients with atypical carcinoids were 10 years older than patients with typical ones. Eleven patients were smokers, all the patients with atypical and 4 patients with typical carcinoid. The most common presenting mode was pneumonia. Fiberoptic bronchoscopy was diagnostic in 11 cases. The survival rate for patients with typical carcinoid was 100%, while it was 57% in patients with atypical carcinoids. Patients with limited disease had a survival of 100%, while in stage III/IV patients showed a lower survival rate (50%). In conclusion, carcinoid tumors of the lung are tumors showing a good prognosis. Five-year survival indicated a significant difference between typical and atypical carcinoids and between limited and extensive disease. A precise staging is recommended for a better treatment and follow-up.

[Colonic pseudo-obstruction caused by digestive amyloidosis in a chronic hemodialyzed patient. Apropos of a case]. / Pseudo-obstruction colique par amylose digestive chez l'hémodialysé chronique. A propos d'un cas.

A case of intestinal pseudo-obstruction by amyloidosis, occurring after 20 years of dialysis in a 72-year-old woman is reported. Although acute intestinal complications of intestinal amyloidosis, such as ischemia, colonic obstruction or haemorrhage are well known, colonic pseudo-obstruction is more unusual. It gradually results in, it carries out an intestinal obstruction with colonic and gastric distension. The diffuse topography of amyloid deposits throughout the gastrointestinal tract carries a poor prognosis and surgery cannot be curative.

[Mediastinal adenopathies metastatic from a basocellular skin carcinoma]. / Adénopathies médiastinales métastatiques d'un carcinome basocellulaire de la peau.

Skin basocellular carcinoma is very frequent, and does not usually cause distant metastasis. However, a few cases of distal lymph node, pulmonary, bone metastasis have been described in the literature. In the present case, we report a basocellular mediastinal lymph node metastasis. Some precise histological characteristics described by Lattses must be present to admit authenticity of these metastasis. The treatment of such cases is based on surgery, radiotherapy and chemotherapy containing cisplatinum. However, the overall prognosis of these metastases remains poor.

[Diabetic mastopathy with epithelioid fibroblasts :differential diagnosis from an infiltrating lobular carcinoma of the breast. Report of two cases]. / Mastopathie diabétique avec fibroblastes épithélioïdes: piège diagnostique avec un carcinome lobulaire infiltrant du sein. A propos de deux cas.

Two cases of diabetic mastopathy are reported. The patients presented with a rapidly increasing mammary mass. The pathologic findings of diabetic mastopathy usually consist of a lymphocytic lobulitis, a dense stromal fibrosis and prominent epithelioid fibroblasts. In our two cases, the latter findings were initially misinterpreted as malignant cells of an invasive lobular carcinoma. These lesions were first described in longstanding insulin dependent diabetics but recent reports showed that they could also occur in patients with other auto-immune disorders (systemic lupus erythematous, hypothyroidism). Diabetic mastopathy may reoccur locally and the treatment remains to be determined.

[Metastatic epidermoid carcinoma in idiopathic CD4+ T lymphocytopenia syndrome]. / Carcinome épidermoïde métastatique au cours d'un syndrome de lymphocytopénie T CD4+ idiopathique.

INTRODUCTION: Skin cancers are more frequent and more aggressive in immunosuppressed patients. CASE REPORT: A 58-year-old man was seen in January 1994 for squamous cell carcinoma of the right shoulder which had grown to 7 x 7 cm in the last 5 months. The patient had a past history of surgical exeresis of 5 squamous cell carcinomas and 3 basal cell carcinomas. Despite complete exeresis, the disease spread to localized then diffuse skin metastases. Acitretine and alpha-interferon were uneffective. Polychemotherapy stabilized the situation but several bronchopulmonary infections with atypical germs led to death (in February 1995). The patient had CD4 lymphocytopenia with a count < 100/mm3 in January 1994 of unknown origin (viral infection was eliminated). DISCUSSION: Our patient had idiopathic CD4 lymphocytopenia. In 40 p. 100 of the cases CD4 lymphocytopenia is caused by AIDS and in 53 p. 100 by other diseases, especially skin diseases including Kaposi syndrome, mycosis fungoides, squamous cell or basal cell carcinoma. The cutaneous carcinomas which often develop in AIDS patients with low CD4 counts are usually less aggressive than in our patient. Certain neoplasia could be the cause of the lymphopenia. CD4 counts are usually more variable and lymphopenia of shorter duration in other etiologies.

[Intravascular malignant lymphoma (ex-malignant angioendotheliosis). 3 cases]. / Lymphome malin intravasculaire (ex-angioendothéliose maligne). 3 observations.

Malignant angioendotheliomatosis is an intravascular proliferation of tumour cells in the small arteries, veins and capillaries. Its lymphomatous origin has been proved recently by immunohistochemistry. It is a rare, generally rapidly fatal condition. Less than 150 cases have been reported in the literature. We report three cases. Long course fever with delayed neurologic disturbances were observed in the first case and long course fever, hyponatraemia, corticotropic and gonadotropic insufficiency in the second. The third case began with febrile meningo-encephalitis. Diagnosis was made at autopsy in the two first cases and on evidence from kidney needle-biopsy in the third. Clinical manifestations are polymorphic and frequently simulate vasculitis. Neurologic signs are the most common. Histologically, kidneys, skin, endocrine glands, lungs, muscles and most of the organs may be involved. Surprisingly lymph nodes, bone marrow and spleen are often free from disease. Current treatment is based on polychemotherapy. Earlier diagnosis of the disease should improve the prognosis as observed in our third patient.