[Characterization and outcome of renal cancers ≤ 4cm treated by surgery]. / Caractéristiques cliniques, histologiques et évolutives des cancers du rein de taille inférieure ou égale à 4cm traités chirurgicalement.

PURPOSE: In a retrospective study, we described the characteristics and the outcome of renal cancers less than or equal to 4cm treated by surgical excision. MATERIAL: Two hundred and eighty four cancers less than or equal to 4cm on preoperative CT scan (T1a) were extracted from our database. We studied, the presence of symptoms, the ECOG and ASA scores, the size, the histological type and the Fuhrman grade. The follow up was clinical, biological and radiological. RESULTS: The mean age was 60.8 years. 21% of patients were symptomatic. The renal capsule was intact in 182 cases (64.08%), the urinary tract in 267 cases (94.01%). Seven patients (2.46%) were metastatic with tumors greater than or equal to pT3a. The most common histological types were the conventional renal cell carcinoma (78.52%) and the papillary renal cell carcinoma (16.55%). 76.06%. of the tumors were low grade. With a median of 66.9 months, 33 patients died (11.61%) . For N0M0 patients, with a median of 59.3 months, three specific deaths (1.19%) and 17 deaths from other causes (6.77%) were observed. The average survival of N0M0 group was 227.2 months. CONCLUSION: The renal cell carcinoma less than or equal to 4cm was a heterogeneous group including locally advanced and aggressive or metastatic tumors. For localized forms, surgical excision provided an effective long-term treatment regardless the histological type or the tumor grade.

[Giant aneurysm of the right coronary artery]. / Anévrysme pseudotumoral de l'artère coronaire droite.

INTRODUCTION: Giant coronary aneurysms are rare and often confused with cardiac tumours. OBSERVATION: We report a new case of this type of aneurysm occurring on the right coronary artery revealed by a cardiac congestion. COMMENTS: These aneurysms can be due to inflammatory diseases or dysplasia. But in these pseudotumoral forms, atherosclerosis is the main aetiology. The diagnosis and treatment require surgery and histological examination.

p21(WAF1/CIP1) response to genotoxic agents in wild-type TP53 expressing breast primary tumours.

Functional inactivation of the wild-type p53 protein has been described in different human cancers. Since a significant proportion of breast tumours express wild-type TP53, the p53 antiproliferative activity could be inactivated in transformed mammary epithelial cells by a mechanism independent on structural alteration of the gene. To test this hypothesis, we analysed the p53 activity in primary breast tumour cells. As a preliminary study, we demonstrated in breast adenocarcinoma cell lines that the nuclear accumulation of the inhibitor of cyclin dependent kinase p21(WAFl/CIP1), in response to adriamycin treatment, specifically reflected the activity of a functional wild-type p53 protein. Then, we used this strategy to study the p53 activity in 23 primary breast tumours. p21(WAF1/CIP1 accumulation was detected in all tumours expressing wild-type TP53. In contrast, no p21(WAF1/CIP1) response was detected in cells harboring a mutant TP53 gene. This report is the first functional study of p53 in primary breast tumours. The results demonstrate that TP53 mutation represents the only common mechanism leading to an irreversible inactivation of p53 functions in this cancer type.

S100A1 and KIT gene expressions in common subtypes of renal tumours.

AIM: The aim of this study is to evaluate the S100A1 and KIT as gene markers for the differentiation of common subtypes of renal tumours. METHODS: Fifty-five tissue samples (15 clear cell RCCs, 15 papillary RCCs, 7 chromophobe RCCs, 8 oncocytomas and 10 normal renal tissues) were studied The gene expressions of S100A1 and KIT were analysed by one-step RT-PCR by using the specific primers. RESULTS: S100A1 was expressed in 2/15 clear cell RCCs, 11/15 papillary RCCs, 7/8 oncocytomas and in 0/7 chromophobe RCCs. KIT gene was expressed in 6/7 chromophobe RCCs and 7/8 oncocytomas while 0/15 clear cell RCCs and 1/15 papillary RCCs expressed kit gene. Normal tissue expressed neither S100A1 nor KIT gene. CONCLUSION: S100A1 and KIT can be used as gene markers for the differentiation of common subtypes of renal tumours.

The expression of G250/mn/CA9 antigen by flow cytometry: its possible implication for detection of micrometastatic renal cancer cells.

Monoclonal antibody (mAb) G250 is a well characterized and specific mAb to renal cell carcinoma (RCC). The gene G250 was recently cloned and was proved to be homologous to MN/CA9. The G250/MN/CA9 antigen was recently explored as a potential marker for RCC. Flow cytometry (FCM) allows quantitative analysis of cells. The present study describes a flow cytometric method to detect this antigen in human cell lines and in malignant and normal renal tissues. Twelve human carcinoma cell lines (HeLa, Colo205, HT29, BxPC3, OVCAR3, SKOV3, ACHN, A704, CAKI-2, SKRC-59, SKRC-10, and SKRC-52), 10 specimens of normal peripheral blood mononuclear cells, and 38 malignant and 36 adjacent normal renal tissues were studied. The malignant and normal renal tissues were disaggregated mechanically into a single-cell suspension, stained by mAb G250, and analyzed by FCM. All 22 of the clear cell carcinomas, 6 of 8 mixed cell carcinomas, and 3 of 6 granular cell carcinomas were positive for G250/MN/CA9 antigen. SKRC-52 and SKRC-10 were strongly positive for G250/ MN/CA9. The G250/MN/CA9 antigen could also be detected in HeLa, SKOV3, HT29, and A704 cells. One chromophobic, one chromophilic cell carcinoma, the normal renal tissues, and normal peripheral blood mononuclear cells were considered as negative. Our results further confirmed that the G250/MN/CA9 antigen was an ideal marker for RCC, especially for clear cell carcinomas, and that this antigen was present in several types of malignant cells. FCM may serve as a fast tool of immunocytochemical detection of renal cancer cells. Flow cytometric detection of renal cancer cells by using mAb G250 should be further explored.

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%.

Flow cytometric analysis of antigen expression in malignant and normal renal cells.

UNLABELLED: Flow cytometry allows quantitative analysis of cancer cells. The aim of this study was to make a quantitative study of antigen expression in malignant and normal renal cells in order to find the efficient monoclonal antibodies (mAbs) for labelling renal cancer cells. MATERIAL AND METHODS: 15 malignant and adjacent normal renal tissues and three renal carcinoma cell lines (ACHN, A704 and CAKI-2) were analyzed. The malignant and normal renal tissues were dissociated mechanically into cell suspension. The mAbs and isotype controls were used for immunochemical labelling. The stained cells were analyzed by flow cytometry. RESULTS: Renal tumor associated antigen G 250 was frequently detected in malignant renal cells but not in normal renal cells. Renal tumor associated antigen gp200 recognized by 66.4.C2 and PN-15 was frequently detected in malignant cells, normal renal cells and also in all three carcinoma cell lines. Epithelial antigens were strongly positive in normal renal cells. Compared with MOC 31, Ber-EP4 and E 29, W-lD9 was mostly reactive to malignant renal cells. VU-1D9 was strongly positive on ACHN and A704. The carbohydrate carcinoma antigens CA 125, DF3 and Sialyl Lewis(a) were detectable in some of the malignant and normal renal cells. Sialyl Lewis(a) could be weakly detected on ACHN and A 704. Pan-cytokeratins and cytokeratin (CK) 8 were strongly expressed in malignant and normal renal cells and in all three cell lines. CONCLUSION: Our results indicated that G 250, 66.4.Ca, PN-15, VU-1D9, MNF116 and anti-ckg were efficient mAbs for labelling renal cancer cells. Their potential clinical application by flow cytometry should be explored.

[Solid pseudopapillary tumors of the pancreas. Pathology report of 13 cases]. / Tumeurs pseudo-papillaires et solides du pancréas. Etude anatomoclinique de 13 cas.

OBJECTIVES: Solid pseudopapillary tumors of the pancreas are exceptional. The aims of our study were to reevaluate the mode of presentation of these tumors and to analyze the role of pathological examination in diagnostic assessment and prognostic evaluation. PATIENTS: We report the clinical, radiological and pathological findings in a retrospective series of 13 patients in whom a diagnosis of solid pseudopapillary tumor of the pancreas was made between 1983 and 1997. There were 12 females (median age: 22.5 years) and one male, aged 73. RESULTS: The tumor was discovered incidentally (3 cases) or because of nonspecific digestive symptoms (10 cases). Biological data were uninformative. The tumor was pancreatic in 12 cases and duodenal in 1. In all cases, imaging techniques showed an heterogeneous lesion with no or poor vascularization. A cystic component was identified in 4 cases. Surgical resection was performed in all cases. Pathological examination showed an encapsulated tumor in 8 cases, a non-encapsulated but well-limited lesion in 3 cases and an infiltrative tumor in 2 cases. At the time of diagnosis, multiple liver metastases were present in 1 case. Mean duration of follow-up was 24 months (range: 3-168). At last follow-up, all patients, including the patient with synchronous metastatic disease, were alive, without local recurrence. CONCLUSION: Our study confirms that most cases of solid pseudopapillary tumors of the pancreas present with a suggestive clinical picture, including their occurrence in young women and their good prognosis after surgical resection. However, our results also underline the occurrence of cases presenting with unusual features, including old age, male sex, extra-pancreatic localization and malignant evolution. Histopathological examination is essential for the establishment of the diagnosis but morphological data are of little prognostic value.

[Microcystic meningiomas: comparison of histology and computed tomography]. / Aspects histologiques et tomodensitométriques des méningiomes microkystiques.

INTRODUCTION: Microcystic meningiomas are defined by large vacuolated and stellate shaped cells. We recently examined a microcystic meningioma mimicking a malignant tumor on computed tomography (CT). The aim of the current study was to compare the radiological features of microcystic meningiomas with their histological patterns. METHODS: We have diagnosed 7 intracranial microcystic meningiomas among 204 meningiomas registered in the files of our Department of Pathology from 1994 to 2001. All CT scans performed before surgery were reviewed. RESULTS: Three of the microcystic meningiomas appeared as entirely microcystic tumors. Two of them were homogeneously hypodense or isodense on CT scan. The third mening was heterogeneous, containing some blood. The histologic pattern of the 4 other meningiomas showed microcystic tumor cells associated with meningothelial or fibrous tumor cells. These meningiomas were heterogeneous on CT scan. All meningiomas seemed to be connected to the dura mater. Three tumors were strongly and homogeneously enhanced after contrast media injection while 3 others were heterogeneously enhanced. No enhanced CT scan was available for 1 case. Astrocytomas were incorrectly diagnosed by CT scan in the 3 heterogeneously enhanced tumors. Meningiomas were correctly diagnosed in the 3 strongly enhanced tumors. CONCLUSION: The presence of microcystic tumour cells in meningiomas often results in erroneous diagnosis on CT scan, particularly for those which are heterogeneously enhanced. In these cases, a diagnosis of astrocytoma is often made.

[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.

[Congenital pigmented nevus: prognosis and therapeutic possibilities]. / Le naevus pigmentaire congénital: pronostic, possibilités thérapeutiques.

Congenital pigmented nevi are found in approximately 1% of newborn infants. Two main factors determine their management: 1) the risk of malignancy (melanoma); 2) the aesthetic consequences, these factors being themselves dependent upon the size and the localization of the nevi. Thus, if the systematic resection of small nevi is not required, early treatment of giant nevi which carry a high risk of malignancy before the age of ten years, is highly recommended. Main methods of treatment are surgical resection (with the help of skin grafts, or cutaneous expansion or mobilization, in giant nevi), dermabrasion and curettage.

[Granuloma annulare in the child]. / Granulome annulaire de l'enfant.

Granuloma annulare is a benign, common, inflammatory skin lesion of unknown etiology that is seen in both adults and children. The typical lesions are single or multiple small cutaneous papules with an annular distribution. The histology is consistent with an area of fibrinoid degeneration of collagen, surrounded by palisading histiocytes and inflammatory cells. There are four clinically distinct subtypes: localized, generalized, subcutaneous and perforating. Usually a spontaneous resolution is expected. Many medical treatments have been proposed but without evidence of efficacy. The association with insulin dependent diabetes is still being discussed.

[Psoriasis in childhood]. / Psoriasis chez l'enfant.

Psoriasis is a common chronic disease in childhood of yet unclear etiology. Thirty per cent of psoriatic patients experience onset of their disease before 15 years of age. Psoriasis is exceptionally congenital but may present in infancy as a napkin dermatitis. There are specific pediatric clinical forms, but at the beginning the eruption is usually discrete, and the diagnosis can be difficult. Pustular, erythrodermic and arthropathic forms are rare. Treatment must weigh the advantages and disadvantages of the possible therapies and be kept to the minimum compatible with asymptomatic control.

[Solitary congenital histiocytoma: two cases]. / Histiocytomes congénitaux : 2 cas.

BACKGROUND: Solitary histiocytoma is an uncommon form of Hashimoto-Pritzker syndrome and an exceptional type of histiocytosis with cells of undetermined origin. A solitary often ulcerated congenital nodule is generally observed. We report two cases, one of each form. CASE REPORTS: Both cases presented an ulcerative budding congenital tumefaction of the plantar aspect of the right foot for the first child and the parieto-axillary region in the second. Histology disclosed a granulomatous infiltrate of histiocytes positive for specific immunolabels (protein S100 and CD1a). In the first case, electron microscopy revealed histiocytes devoid of Birbeck granules and myelinoid bodies leading to the diagnosis of Langerhans histiocytosis with cells of unknown origin. In the second case, 18 p. 100 of the cells contained Birbeck granules. There has been no recurrence after a 5-year follow-up in a case. DISCUSSION: These cases recall the congenital nature of some types of solitary histiocytomas. Indeed, congenital Langerhans histiocytoma can occur as a unique nodule. The tumefaction may lie in any localization. Histological diagnosis is required. The benign nature of these lesions is confirmed by the absence of distant lesions and the lack of recurrence after complete excision. About a dozen cases have been reported. Most have been Hashimoto-Pritzker syndromes. Only one case has been reported with cells of undetermined origin. The diagnosis of histiocytosis with cells of undetermined origin is made when the ultrastructure study demonstrates the vacuity of the histiocyte cytoplasm. This condition is similar to Hashimoto-Pritzker syndrome by the absence of recurrence and systemic diffusion. It can however be observed in adults. The undetermined cell types would correspond different phases of Langerhans cell maturation or involution.

[Focal familial palmoplantar keratoderma with punctate hyperkeratosis of the palmar creases]. / Kératodermie palmo-plantaire focale familiale avec hyperkératose ponctuée des plis palmaires.

BACKGROUND: Keratosis punctata of the palmar creases is a variant of punctata palmoplantar keratoderma. CASE REPORT: Keratosis punctata of the palmar creases was observed in a 53 year-old North African male. The lesions presented as small keratotic papules confined exclusively to the flexion creases of the palms and digits, with a bilateral localization on the soles. The lesions were sensitive to pressure. The proband's 19 year-old son had similar lesions. DISCUSSION: Keratosis punctata of the palmar creases is characterized by the development of small round keratotic papules, electively and exclusively found in the palmar, digital, and uncommonly soles creases. The etiology is unknown. The frequency is higher in the black population. Although considered as an hereditary palmoplantar keratoderma with autosomal dominant transmission, familial cases with localizations on the soles is uncommon.

S100A1: a powerful marker to differentiate chromophobe renal cell carcinoma from renal oncocytoma.

AIMS: The common subtypes of renal tumours are conventional, papillary, chromophobe carcinoma and oncocytoma. The morphological differentiation between chromophobe carcinoma and oncocytoma may be difficult. The aim was to evaluate S100A1 as a new marker for the differentiation of the two subtypes. METHODS AND RESULTS: Thirty-nine tumour samples [nine clear cell renal cell carcinomas (RCCs), six papillary RCCs, nine chromophobe RCCs and 15 oncocytomas] were studied. The protein expression of S100A1 was evaluated by immunohistochemistry. The gene expression of S100A1 was analysed by reverse transcriptase-polymerase chain reaction. Nine oncocytomas showed strong immunoreactivity for S100A1. Four oncocytomas were scored as moderate and one as weak reactivity. In total, 14/15 (93%) of oncocytomas were considered to be immunopositive. In contrast, all nine chromophobe RCCs were considered to be immunonegative. There was a significant difference in the positive percentages of staining of S100A1 between these two subtypes (P < 0.01). S100A1 immunoreactivity was observed in 6/9 clear cell and 4/6 papillary carcinomas. The results of S100A1 gene expression corresponded well with the results of immunohistochemistry. CONCLUSION: S100A1 may be a potentially powerful marker to differentiate the chromophobe RCC from renal oncocytoma.

MN/CA9: a potential gene marker for detection of malignant cells in effusions.

Many cancers cause malignant effusions. The presence of malignant cells in effusions has implications in diagnosis, tumour staging and prognosis. The detection of malignant cells currently presents a challenge for cytopathologists. New adjunctive methods are needed. Although the effusions provide excellent materials for molecular assay, the available molecular markers are extremely limited, which hinders its clinical application. MN/CA9 has proved to be a valuable marker in many cancers such as lung, breast, colon, kidney, etc. The present study was to evaluate MN/CA9 as a new molecular marker for the detection of cancer cells in pleural effusions. Seventy-one pleural effusions including 59 malignant effusions from patients with cancer, and 12 patients with benign diseases as a control, were subjected to RT-PCR for detection of MN/CA9 gene expression. MN/CA9 gene expression was detected in 53/59 (89.8%) pleural effusions from cancer patients (15/16 for breast cancers, 10/11 for lung cancers, 4/4 for ovary cancers, 2/3 for colon-rectal cancers, 5/6 for cancers of unknown site, 7/8 for mesothelioma and 10/11 for other cancers). Furthermore, MN/CA9 was positive in 13/18 (72.2%) of cytologically negative effusions of cancer patients. MN/CA9 was detected in only 1/12 (8.3%) effusions from the control patients (p < 0.01). The sensitivity and specificity of MN/CA9 gene expression were, respectively, 89.8% and 91.7%. Our preliminary results suggest that MN/CA9 could be a potential marker for the detection of malignant cells in effusions. A large-scale study is needed to confirm these results.

Successful treatment via chemotherapy and surgical resection of a femoral hemangiopericytoma with pulmonary metastasis.

Hemangiopericytoma (HPC) is a soft-tissue neoplasm composed of proliferating capillary pericytes. It has variable and unpredictable malignancy and most commonly occurs in the fifth or sixth decade of life. Diagnosis is based on the histological aspect. HPC is exceedingly rare in childhood. In both adults and children, curative surgery is the most important predictor of survival. The place of chemotherapy in the treatment of HPC is not well established. We describe a case of adult-type metastatic HPC of the thigh in a 13-year-old boy. The response to neoadjuvant chemotherapy was excellent, and local control of this initially unresectable tumor was achieved without radiation therapy or mutilating surgery. The child is alive and well and has had 8 years of follow-up after treatment.