Search

We report the case of a 3-year-old child who died from the consequences of a cardio-respiratory arrest despite reanimation procedures. Echocardiography and magnetic resonance imaging (MRI) revealed a mass of the free wall of the left ventricle. Autopsy confirmed the existence of a solitary myocardial tumor, well-circumscribed, firm, with a whitish and trabeculated cut surface. Histologically, the tumor consisted of bundles of spindle-shaped and regular cells mingling with collagen and elastic fibers, insinuating themselves between myocytes in periphery. Calcifications were present. After immunohistochemistry, the cells were highlighted by anti-actin smooth muscle antibody; but they were not highlighted by anti-desmin, anti-β catenin and anti-Ki67 antibodies. The diagnosis of cardiac fibroma was made. The primary cardiac tumors of child are rare and usually benign. They are essentially represented by rhabdomyoma and fibroma. Cardiac fibroma mostly occurs during the first year of life. It can be revealed by cardiac insufficiency, arrhythmia, chest pain or sudden death., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Background: In 2005, following the first cancer plan of the national health authorities, a general cancer registry was established in northern France, in a territory designated as a "zone in proximity to the city of Lille" (ZPL). The aim of the present work was to evaluate the completeness of the registry's first year of incident cancer registration (2005) and to compare the observed cancer incidence in the "ZPL" with the estimated incidence in France., Methods: Completeness was assessed using the average number of sources per case, the percentage of histological verification and a method of independent case ascertainment (mortality/incidence ratio). A direct standardization on the world population was used to calculate the ZPL/France ratios of standardized incidence rates. Analyses were conducted for 21 cancer sites., Results: In 2005, 3635 cases of invasive cancer were recorded by the registry. The average number of sources per case was 2.7 and histological proof was available for 91.4% of cases. Mortality/incidence ratios showed satisfactory completeness of the data for men for most cancer sites. For women however, for cancer sites for which the number of cases was low, data will have to be confirmed during the subsequent years of observation. A lack of completeness was found for cutaneous melanoma. In men, an overincidence was identified for cancers of lip-mouth-pharynx, larynx, esophagus, lung, liver, bladder, kidney and colon-rectum. In women, an overincidence has been identified for cancers of lip-mouth-pharynx, liver, bladder, colon-rectum, corpus uteri and ovaries., Conclusion: The first year of incidence validated at the "Registre général des cancers de Lille et de sa région" shows a completeness of records with regards to studied criteria. The comparison with national data shows an overincidence of cancers related to tobacco and alcohol consumption in the geographical area covered by the registry. The incidence of lip-mouth-pharynx cancer in men is the highest of all French registries., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

The medical treatment of renal-cell carcinoma, and of its most frequent histologic subtype, the clear cell renal-cell carcinoma, has been changed by the emergence of targeted therapies. The development of these drugs has been made possible by more precise knowledge of molecular mechanisms involved in the carcinogenesis of these tumors. Three molecular pathways may be involved in clear cell renal-cell carcinoma: VHL/HIF/VEGF, PI3K/AKT/mTOR and MAPKinases pathways. These antiangiogenic therapies are approved for the treatment of advanced and metastatic clear cell renal-cell carcinoma. In metastatic disease, pathologists have significant and different implications: 1) in tumor biopsy, only the diagnosis of clear cell renal-cell carcinoma implies the prescription of antiangiogenic drugs, and 2) after targeted therapies, pathologists must evaluate the therapeutic effects on tumor. Consensus on macroscopic and histologic reports are needed by pathologists., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

The survival of osteosarcoma and Ewing family tumours has been improved by the introduction of neoadjuvant chemotherapy. The response to preoperative chemotherapy is evaluated on the microscopic analysis of the surgical resection, by the percentage of tumour necrosis according to the Huvos and Rosen's grading. It remains the only reliable prognostic factor for patients and is used to guide the choice of post-operative chemotherapy. The macroscopic and microscopic management of the surgical resection (cf. supra) is essential and is the subject of a specific protocol. Several studies have been conducted to identify news factors able to predict the response to chemotherapy, the tumour aggressiveness and its ability to develop metastases. Inhibitors of mTOR and/or regulators of the balance RANKL/OPG are promising therapeutics. The study's expression of these new factors could be performed on the biopsy and will offer new therapeutic strategy., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

A case of primary adenocarcinoma of the renal pelvis occurring in a 57-year-old woman who had no previous history is reported. The lesions were thought to be a renal destruction by pyelolithiasis upon symptomatology and imaging study data. A nephrectomy was thus performed. Macroscopic examination revealed voluminous and exophytic lesions surrounding lithiasis. Diagnosis of intestinal-type adenocarcinoma of the renal pelvis was established on histological examination. No evidence of other tumor localization was revealed by complete exploration. Primary adenocarcinoma of renal pelvis is a rare and often mucinous intestinal-type tumour. Chronic inflammation and renal lithiasis seem to be associated with this tumour. Few cases are reported and prognosis is doubtful. The main differential diagnosis to eliminate is secondary lesions to the kidney of adenocarcinoma from another origin. A careful pathological examination in case of pyelonephritic kidney is necessary to look for an associated tumour., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Azoospermia may be obstructive (blockage of the genital ducts) or non-obstructive (a lack of testicular production). The distinction is based on an ensemble of clinical, spermiological, hormonal, ultrasound, genetic and histological data. Azoospermia is the main indication for testicular biopsy for therapeutic and diagnostic purposes. Testicular spermatozoids are processed in the reproductive biology laboratory (simultaneously with oocyte retrieval or not) for in vitro fertilization with intra-cytoplasmic sperm injection. The histological study of spermatogenesis is usually performed on a testicular biopsy sample taken at the same time and provides additional diagnostic information on infertility. Histological alterations in the testicular tissues are frequently observed in azoospermic men. In non-obstructive azoospermia, three histological situations prevail: hypospermatogenesis, Sertoli-cell-only syndrome and germ cell arrest. One can distinguish between pure forms (in which all the seminiferous tubules have the same appearance) and mixed forms (in which the tubules' aspects are heterogeneous). Hypospermatogenesis is highly prevalent in azoospermia and is characterized by a low, basal level of spermatozoid production. The prevalence of Sertoli-cell-only syndrome varies from 27 to 68% and the mean spermatozoid recovery rate is between 16 and 33%. Germ cell arrests are rare phenotypes and have a poor prognosis for spermatozoid recovery. Overall, histological examination (still the only way to fully describe spermatogenesis) must be qualitative and quantitative, with the adoption of a standardized, universally understood terminology. It is essential to compare the histological data with (i) recovery of testicular spermatozoids, (ii) clinical, ultrasound, hormonal and genetic data and (iii) the outcome of IVF/ICSI procedures., (2010 Elsevier Masson SAS. All rights reserved.)

MUC1 is a large trans-membrane highly glycosylated mucin which is expressed at the apical pole of normal cells in glandular epithelia. MUC1 is implicated in many physiological mechanisms such as adhesion, development and differentiation. Also, MUC1 is frequently deregulated and over-expressed with a membrane circumferential and/or cytoplasmic expression. The intracellular tail of MUC1 is phosphorylated and can interact with many signalling proteins and transcriptional factors. Indeed, MUC1 can interact with B-catenin competitively for E-cadherin, thus destabilizing intercellular junctions and favouring metastatic dissemination. In carcinomas, the overexpression and membrane delocalization of MUC1 is associated with a worse prognosis and a shorter survival in breast, colon, kidney, prostate or gastro-intestinal cancers. MUC1 appears to be a novel therapeutic target for immunotherapy or anti-tumour vaccines.

Medical imaging has an important role in the diagnosis and treatment options of prostate cancer (TRUS guided biopsies, MRI). The knowledge of the different types of cancers, their preferred topography, imaging features, extension pattern, and also the important items that may help the surgical procedure (or any other treatment) are as many crucial key points for optimal management of patients.

We report two cases of giant-cell tumour of soft tissue (TCG-TM). The first case occurred in a 26-year-old woman presenting with a subcutaneous tumour of the left leg. Pathological study revealed a tumour comparable to benign giant cell tumour of bone. The patient is well without recurrence 10 months after the diagnosis. The second case concerned a 49-year-old man with a huge mass of the thigh. Microscopically, the tumour was composed of sheets of mononuclear and multinucleated cells. Mononuclear cells presented severe atypia and a high mitotic activity. Eighteen months later, the patient died with diffuse pulmonary metastases. TCG-TMs are uncommon and represent a distinct entity whose clinical behaviour and histological features are similar to giant-cell tumour of bone. The malignant variant is debatable. The differential diagnosis includes other tumours rich in osteoclast-like cells.

Adrenocortical carcinomas are rare tumors. In the absence of the gold standard that constitutes metastases, local invasion or recurrence, the diagnosis of malignancy may represent a great challenge for both clinicians and pathologists. Several multiparametric systems have been developed to assess this malignancy. Among them, the Weiss system, established in 1984 and based on nine microscopic criteria, appears to be the most employed because of its simplicity and reliability. The presence of three and more criteria is in favor of malignancy. However, the application of such system is not devoid of difficulties and subjectivity. A more precise definition of the different microscopic criteria and the proposition of a modified Weiss system based on the most reliable criteria could help. Besides, the Weiss system is of limited value for the diagnosis of malignancy in oxyphil tumors and children tumors of the adrenal cortex.

Lipomatous tumors rarely occur in the salivary glands. We report an unusual case of lipomatous pleomorphic adenoma in a 47-year-old man. The patient had no significant medical history and presented with a well circumscribed nodule measuring 3 cm in the right parotid. Histologically, the tumour was predominantly composed of sheets of mature fat cells. Rare myoepithelial cells and exceptional tubules were intermingled with the mature adipose tissue. One year after surgery the patient was alive without recurrence. In conclusion, it is a rare neoplasm who needs to be recognized and discussed with true fatty tumors and lipomatosis.

Solitary fibrous tumor is a rare circumscribed neoplasm involving usually the pleura and now described in many organs and in the soft tissues with a favorable outcome when completely excised. We report herein a malignant solitary fibrous tumor of the pleura occurring in a 59-year-old woman. The tumor cells strongly expressed vimentin and CD 34. In addition a weak positive staining was obtained using CD 99 and bcl 2. A disconcerting focal immunoreactivity with cytokeratin was observed. Solitary fibrous tumor is composed of loosely arranged spindle cells of variable density and may occasionally mimic malignant mesothelioma, an hemangiopericytoma or synovial sarcoma. Immunohistochemical study is helpful demonstrating a strong positivity for vimentin and CD 34 and absence of staining for epithelial markers and S-100 protein.

Primary neuroendocrine carcinomas of the kidney are infrequent. The large cell neuroendocrine carcinoma is a subtype with an aggressive course and a worse prognosis. We report two cases, a 35-year-old man and a 75-year-old woman who died within 6 and 5-months after surgery despite radical nephrectomy and chemotherapy. Histological and immunohistochemical features are presented.

We describe a juvenile granulosa cell tumor expressed by an early pseudopuberty occurring in a 3-year-old black child. Clinically, an endocrine syndrome was associated with a pelvic mass and ascites. Hyperoestrogenia and serum alphafetoprotein level were biologically detected. Histological examination showed typical microscopic features of a granulosa cell tumor. The patient is well four years after surgery. Growth and mental development are normal.

We report a case of malignant mesenchymoma developed in the mesentery in a 62-year-old man with no past history. It was an huge mass in the mesentery of the ileum. The tumor was composed of areas of chondrosarcoma and rhabdomyosarcoma in addition to an undifferentiated fusiform component. Malignant mesenchymoma is a rare sarcoma occurring preferentially in the retroperitoneum or the thigh. The location in the mesentery is exceptional. Malignant mesenchymoma is discussed more particularly with dedifferentiated liposarcoma with heterologous elements. The patient is alive without recurrence three years after surgery.

Juvenile xanthogranuloma of parotid gland is reported in a 9-year-old boy. This kind of tumor is thought to be very rare in salivary glands. Histological, immunohistochemical and ultrastructural data showed characteristic features and excluded a Langerhans cell histiocytosis. Follow-up was uneventful after 18 months.