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The authors expose the clinical, radiological and histological presentation of three cases of solitary fibrous tumors of the meninges, initially thought to be meningiomas. Actually, these three cases show typical anatomoclinical features. The authors also mention the differential diagnosis, and recall the essential contribution of immunohistochemistry., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

PEComa group is a heterogeneous group of rare mesenchymal tumors supposed to derive from perivascular cells and characterized by a coexpression of myogenic and melanocytic markers. We describe an 11-year-old female patient presenting a 2 cm ulcerated rectal polyp, exteriorized by anus, which was totally resected. Morphologically, this tumour was composed of cells arranged in nests or large cords separated by fibrous stroma, with abundant clear cytoplasms and with round regular small nuclei without atypia. There was no necrotic area and mitotic activity was very low. Immunohistochemically, the tumours cells stained for HMB45. Only 17 cases have been reported in literature and this case is the 18th. Here, we present a literature review focusing on both malignancy criteria and differential diagnosis., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Among primitive adenocarcinoma of nasal cavity and paranasal sinus, the 2005 WHO classification distinguishes two main categories: intestinal type adenocarcinoma (ITAC) and low-grade non-intestinal adenocarcinoma, entities with different clinical and epidemiological characteristics. Low-grade adenocarcinoma shows a respiratory type phenotype (CK20-/CK7+/CDX2-/villin-) and ITACs, an intestinal type profile (CK20+/CK7-/CDX2+/villin+). Because of histological, ultrastructural and phenotypical similarities between ITAC and colorectal adenocarcinomas, several studies have discussed a possible common pathway in carcinogenesis. But the review of literature shows conflicting results, suggesting different pathways of pathogenesis. Differential diagnoses of sinonasal intestinal-type adenocarcinoma are mainly respiratory epithelial adenomatoid hamartomas, inverted schneiderian papillomas, salivary glands-type carcinoma and more rarely metastasis of adenocarcinoma.

Langherans' cell histiocytosis is rare in adults. It should be considered in patients with lytic osseous lesions with sclerotic rim especially when involvement of adjacent soft tissues is present.

Head and neck biopsies usually have a low diagnostic value in Wegener's granulomatosis (WG). On the basis of 49 biopsies obtained from 21 WG patients at diagnosis from various sites, i.e. nose (29), paranasal sinus (7), oral cavity (4), larynx (4), conjunctiva (3) and external ear (2), we described the suggestive histological features and studied the diagnostic potential of the biopsy size, anaesthesia method (general (GA) or local (LA)), anatomic region of the biopsy, number of sections, and presenting macroscopic manifestations. Associated granulomatous inflammation (scattered giant cells, 28.5% of biopsies; poorly-formed granulomas, 28.5%), necrosis (neutrophilic microabscesses, 16.3%; geographic necrosis, 18.3%), angiitis (leukocytoclastic, 10%; necrotizing, 12%; and granulomatous, 6%) which confirmed the diagnosis were only present in 18.3% of the biopsies (28.5% of the patients). We think it is possible to propose a "WG-compatible" diagnosis when at least one of these histological features is present (24.5% of biopsies, 26% of patients in our study). We found that it was always better to perform biopsies targeted on macroscopic lesions. When there was no lesion, samples from paranasal sinuses obtained under GA had the highest diagnostic value in the head and neck region, whereas 90% of nasal systematic biopsies performed under LA were nonspecific. Moreover, we demonstrated that performing two further sections increased the sensitivity of histological examination by 7%.

Oncogenic osteomalacia is a rare clinicopathologic entity, linked to a mesenchymal tumor which overexpresses a hypophosphatemic factor, supposed to be the FGF-23. To date, about 100 cases have been published. We report the case of a 40-year-old man who presented an osteomalacic syndrome with no classical etiological diagnosis. The discovery of a subcutaneous tumor of the right foot and a high serum level of FGF-23 suggested the diagnosis of oncogenic osteomalacia. Surgical removal of the tumor resulted in complete reversal of the clinical and biochemical defects. Pathologic examination revealed spindle cells associated with osteoclast-like giant cells, embedded within a myxoid matrix which showed floculent calcification. These observations were compatible with the features of PMTMCT (Phosphaturic mesenchymal tumor mixed connective tissue variant).

Breast carcinomas with false negative mammogram correspond to carcinomas diagnosed with means other than mammogram when the mammogram, at the time of diagnosis, showed no significant abnormality. The rate of false mammogram is difficult to ascertain because few studies have been published on this subject. The absence of mammographic abnormality is related to histopathological characteristics of the tumor and mammographic features of the patient's breast tissue. The small size of the tumor, the lack of microcalcifications often due to tumor necrosis and the absence of any significant associated desmoplastic reaction are the main histopathological factors encountered, particularly for dense breasts.

Histological pleomorphism of testicular germ cell tumors complicates diagnosis. These tumors share highly specific differentiation properties ranging from intra-tubular neoplasia to classical infiltrating lesions. This general review presents the histopathological features and diagnostic criteria of typical forms and atypical variants. In current practice, distinction between seminoma and non-seminomatous germ cell tumors is fundamental for treatment. Histological typing of non-seminomatous tumors may be problematical, especially in "mixed" forms. Vitelline tumor variants and atypical embryonic carcinoma are described. Unlike scattered isolated syncytiotrophoblastic cells, true chorio-carcinoma is rare and must be carefully identified. Such cells are frequently associated with seminoma, embryonic carcinoma and vitelline tumors. Intra-tubular germ-cell neoplasia and gonadoblastoma, are pre-invasive lesions associated with cryptorchidism and gonadal dysgenesis. Teratomatous elements usually occur in mixed germ cell tumors. Rare pure teratoma are benign in children. On the contrary, post-pubertal forms have an unpredictable evolution.

Amyloidosis of the genito-urinary tract is uncommon. We report 8 cases, often misdiagnosed as a neoplastic process (6/8). Amyloidosis was localized in the bladder (3 cases), in the ureter (1 case) and in the prostate and/or seminal vesicles (4 cases). The amyloid protein was characterized in 7 cases by immunohistochemistry. Among the bladder and ureter amyloidosis, 2 cases were classified as AL lambda amyloidosis and one case as AA amyloidosis in a patient with long history of chronic arthritis. In the fourth case, the deposits could not be identified. Nevertheless an AL amyloidosis might be suggested. Two cases of prostate and/or seminal vesicles amyloidosis were stained with an anti-B2M antibody, in hemodialyzed patients. The 2 others, positive with the anti-Transthyretina antibody, were classified as senile amyloidosis. This small series illustrated the heterogeneous pathogenic types of amyloidosis in the urogenital tract and emphasized the interest of immunohistochemistry to identify the chemical composition of these deposits.

We report a case of congenital cervical rhabdoid tumor with association of a medulloblastoma in a brother. The immunohistochemical features of this tumor are compatible with a neuroectodermal differentiation (MIC 2+, Leu 7+). Extrarenal rhabdoid tumors share a common morphology but do not represent a single entity with only one histogenesis. Most of them are now considered to be of neuroectodermal origin. In our case, the association with a medulloblastoma in a brother seems to confirm this concept.

An angiosarcoma arising at the site of an arteriovenous fistula in a renal transplant recipient is reported. Only two similar cases have yet been described. This report is documented with an immunohistochemical study and etiopathogenesis is discussed. Immunosuppressive therapy and the arterio-venous fistula might both be involved in the occurrence of this neoplasm. We focus attention on this uncommon complication which should always be entertained when a renal transplant recipient complains about a painful arterio-venous fistula.

We report a case concerning a 65 year old man who suffered from a drug resistant asthma and radiographically visible mass in the right lower lobe of the lung. Pathological studies demonstrated it to be "Bronchopathia Osteoplastica", involving segmental bronchi, with completed hematopoietic metaplasia. This rare dystrophic lesion was associated with localized lambda light chain amyloidosis as was proved by the immunohistochemistry. This association suggests a possible relation between amyloidosis and abnormal osteogenesis.

The subcutaneous calcifications in a case of CRST syndrome were studied by electron microscopy and by several physico-chemical tests in order to establish their structure and development. Samples of powder, obtained by crushing and homogenisation of the deposits, were subjected to the following analyses: - radiocrystallography by X-ray of the raw and heated material - infra-red spectroscopy before and after heating - quantitative chemical analysis and differential thermal micro-analysis. Connective tissue fibres seemed to play an important part in the development of the calcifications. The initial deposits of calcium first appeared in the central part of elastic fibres whose general morphology remained nevertheless undisturbed. In the older deposits disorganised elastic (and possibly collagen) fibres and areas of bone formation were found. Evidence of an inflammatory reaction was always minimal or nil. There was considerable variation in the calibre of collagen fibres in the non-calcified zones, the smaller fibres predominating. These appearances, sometimes noted in scleroderma, seem to be associated with increased collagen synthesis. X-ray diffraction and infra-red spectroscopy studies show that the deposits consist essentially of type B hydroxyapatite carbonate, confirmed by differential thermic analysis. On electron microscopy the calcifications were shown to comprise straight, needle-like crystals, 100-600 nm in length and 30-100 nm broad, characteristic of the apatites. Very occasionally, target-pattern deposits conforming to whitlockite were encountered. Chemical analysis revealed a high proportion of calcium with a Ca/P ratio greater than 1-666 as is typical of synthetic hydroxyapatite carbonates. The deposits also contained up to 20% by weight of organic material.

Hemangiopericytoma is a rare vascular tumor of pericyte origin and of variable but always preoccupying potential since falling within the category of sarcomas. A thoracic localization is rare and primary pulmonary parenchyma lesions even more so, only 87 cases being reported in the literature. Radiological diagnostic criteria are of weak specificity, diagnosis being provided by thin section CT scan imaging with contrast. The differential diagnosis of these vascular tumors includes arteriovenous fistula, pulmonary varices, other vascular tumors or even organized bronchomucoceles. The value of CT scan puncture is obviously limited. The course is unpredictable since even histopathology findings fail to provide definitive data.

Two cases of renal benign renin-secreting tumours (juxta-glomerular cells tumors) have been compared by optical and electron microscopy. The first is the simplest type of tumoral form which can be observed. This contains secretory cells similar to epitheloid cells of normal juxta-glomerular apparatus, which multiply in well developed arteriolar and capillary network. As in afferent arterioles of glomeruli, transformation of parietal smooth cells in secretory cells can be observed. This results in the presence of intermediate cells, containing both contractile filaments and secretory granules. This tumor does not contain nerves. The second tumor has a more complex structure. Beside usual secretory cells, tubular formations and adrenergic amyelinic nerves are observed. Tubes and nerves have been described separated in many juxta glomerular cells tumors but had never been observed in association. Tubules with small lumen are made of highly dystrophic cells. High concentration of "kallikrein" in tumoral tissue, strongly suggests that they proceed from distal tubule. Unmyelinated nerves from varicosities containing densely cored vesicles characteristics of adrenergic nerves, and synaptic terminal endings on secretory cells. The presence of nerve bundles suggest a nervous regulation of tumoral secretions. This hypothesis is confirmed by dynamic explorations of sympathetic system.

A 24-year old female patient developed extramembranous glomerulonephritis 8 months after acute hepatitis-B with virus antigen persisting in the serum. Two years later, the glomerular lesions were found to be clinically and histologically cured, with parallel disappearance of the antigen from the serum. Although the presence of HBsAg in the glomeruli could not be demonstrated, it is likely from the sequence of events that hepatitis-B virus was responsible for the renal disease.

The authors describe a case of cirrhogenic hepatitis due to Pexid which was given for 8 months at 400 mg/day for a severe angina pectoris. We find here the anatomo-clinical profile of perhexiline maleate hepatiits already described in approximately 20 cases. There was a cirrhogenic evolution in our case as in 5 others : but here cirrhosis was revealing and seems stabilized since the treatment was stopped. The cirrhogenic evolution could be due to a cumulative effect of the drug and/or to an immuno-allergic mechanism as in alcoholic cirrhosis which is very similar, especially from an anatomical point of view. However cirrhogenic hepatitis differs by a characteristic lysosomal overload : brown pigment under microscopic observation and lipolysosomes with in some cases a lamellar structure under electron microscopic observation. The prescription of such a drug should be limited to cases of refractory angina pectoris and needed a regular clinical and biological survey.

A new case of an association between phaeochromocytoma and a brown fat pseudo-tumour is presented, with arteriographic and histological documents. The case serves to emphasize the difficulties in diagnosis related to the confusing coexistence of the two entities and to discuss the role of catecholamines in the growth of brown adipose tissue under normal and pathological circumstances.

A case of pneumoblastoma concerning a 33 year old man, incidentally discovered on a systematic chest X Ray, is reported. The radiologic findings include a voluminous and peripherical mass with necrotic zones. The pathologic findings in optic microscopy and the special technics consist in the three cellular contingents which form the pneumoblastoma: clusters of indifferentiated cells, epithelial cells and spindle cells. All the features show clearly a blastomatic character very similar to a foetal lung which allows to differentiate the pulmonary blastoma from carcinosarcoma. The authors, through a review of the literature, demonstrate the rarity (less than 100 cases reported), the malignant evolution which metastasis and the age (strong majority of adults) which characterize this pathology.

Flat wart-like lesions of two patients with epidermodysplasia verruciformis (EDV) were associated with pink, tan or depigmented pityriasis-like macules. There was no familial history nor mental retardation. Human papilloma virus (HPV) type 5 was demonstrated in the two patients and was responsible for the pityriasis-like lesions. Malignancies seem to be closely related to HPV-5 infection since bowenoid transformation occurred in the 2 patients. Immunological studies showed an increase of serum IgE in both patients and an important decrease of IgM in one of them. Most of delayed hypersensitivity skin tests were negative. T-cell percentages (E rosettes) were decreased in one patient, normal for the other one. The mitogenic response to PHA and ConA was markedly depressed in the two patients but returned to normal values in one of them after 3 months of aromatic retinoid (Ro 10-9359) treatment. Beside the viral type, the defect of cell mediated immunity could play an important role in the disease and in the malignant conversion of the lesions. A long-term preventive treatment by retinoic acid derivatives could be of interest for patients with HPV type 5 EDV.

The authors present first correlations between histology, anatomy and serial radiography of the breast, in order to establish a new understanding of the mammographic picture, with respect to the tridimensional histological structures. Several essential points are considered: - Mammographic pictures only show fibrous, connecting and fatty tissues. The epithelium of the galactophores is not shown, because of its low radio-opacity. Mammographic densities are due above all to fibrous connecting tissue, which has a high percentage of water. Benign or malignant breast tumors present with a proliferation of epithelium and connecting tissue, but only connecting tissue, more or less hydrated is shown on the mammographic picture. Many mammographic patterns are related to various superimposed fibrous strands, appearing as pseudotumoral opacities. The benign or malignant tumoral opacities are hidden or modified by multiple superimposed adjacent structures. - The mammographic patterns is related to the degree of hydratation and components of the connecting tissue.