Pituitary apoplexy and rivaroxaban.

Pituitary apoplexy (PA), defined by the occurrence of a massive hemorrhagic necrotic rearrangement within a pituitary adenoma, is rare. Its occurrence can be associated with certain risk factors, including anticoagulation. We report the first case of PA with rivaroxaban which is one of the new oral anticoagulants: a 73 year-old patient presenting with severe headache and visual field deterioration. Surgery was performed. Radiotherapy treatment was decided three months after surgery because of tumor residue.

Genome-wide paternal uniparental disomy as a cause of Beckwith-Wiedemann syndrome associated with recurrent virilizing adrenocortical tumors.

Beckwith-Wiedemann syndrome (BWS) is an overgrowth syndrome characterized by fetal macrosomia, macroglossia, and abdominal wall defects. BWS patients are at risk to develop Wilms tumor, neuroblastoma, hepatoblastoma, and adrenal tumors. A young woman with BWS features, but with inconclusive genetic evidence for the disease, came to clinical observation for signs of virilization at the age of 16 years. An adrenocortical tumor was diagnosed and surgically resected. The tumor underwent 2 local relapses that were also surgically treated. The patient was also operated to remove a breast fibroadenoma. SNP arrays were used to analyze chromosome abnormalities in normal and tumor samples from the patient and her parents. The patient presented genome-wide mosaic paternal uniparental disomy (patUPD) both in the adrenocortical and the breast tumors, with different degrees of loss of heterozygosity (LOH). The more recent relapses of the adrenocortical tumor showed a loss of part of chromosome 17p that was absent in the first tumor. Analysis of a skin biopsy sample also showed mosaic patUPD with partial LOH, while no LOH was detected in leukocyte DNA. This case shows that virilizing adrenocortical tumors may be a clinical feature of patients with BWS. The SNP array technology is useful to diagnose genome-wide patUPD mosaicism in BWS patients with an inconclusive molecular diagnosis and underlines the tumorigenic potential of the absence of the maternal genome combined with an excess of the paternal genome.

Association of sarcoidosis and ulcerative colitis: a review of 20 cases.

Sarcoidosis and ulcerative colitis is a rare association. We report a case of this association and describe its clinical, radiological and functional characteristics based on a review of the literature. This association must be recognized by the physician and must be clearly distinguished from a systemic site of ulcerative colitis or drug-related pneumonitis.

A new prognostic clinicopathological classification of pituitary adenomas: a multicentric case-control study of 410 patients with 8 years post-operative follow-up.

Pituitary adenomas are currently classified by histological, immunocytochemical and numerous ultrastructural characteristics lacking unequivocal prognostic correlations. We investigated the prognostic value of a new clinicopathological classification with grades based on invasion and proliferation. This retrospective multicentric case-control study comprised 410 patients who had surgery for a pituitary tumour with long-term follow-up. Using pituitary magnetic resonance imaging for diagnosis of cavernous or sphenoid sinus invasion, immunocytochemistry, markers of the cell cycle (Ki-67, mitoses) and p53, tumours were classified according to size (micro, macro and giant), type (PRL, GH, FSH/LH, ACTH and TSH) and grade (grade 1a: non-invasive, 1b: non-invasive and proliferative, 2a: invasive, 2b: invasive and proliferative, and 3: metastatic). The association between patient status at 8-year follow-up and age, sex, and classification was evaluated by two multivariate analyses assessing disease- or recurrence/progression-free status. At 8 years after surgery, 195 patients were disease-free (controls) and 215 patients were not (cases). In 125 of the cases the tumours had recurred or progressed. Analyses of disease-free and recurrence/progression-free status revealed the significant prognostic value (p < 0.001; p < 0.05) of age, tumour type, and grade across all tumour types and for each tumour type. Invasive and proliferative tumours (grade 2b) had a poor prognosis with an increased probability of tumour persistence or progression of 25- or 12-fold, respectively, as compared to non-invasive tumours (grade 1a). This new, easy to use clinicopathological classification of pituitary endocrine tumours has demonstrated its prognostic worth by strongly predicting the probability of post-operative complete remission or tumour progression and so could help clinicians choose the best post-operative therapy.

[Intradiploic cavernous hemangioma of the calvaria: case report and review of the literature]. / Hémangiome caverneux intradiploïque du vertex: à propos d'un cas et revue de la littérature.

We report the case of a 31-year-old patient who had had frontal cephalalgias for several years. CT and MRI anatomical imaging objectified a frontal osteolytic tumor respecting the osseous external table but compressing the superior sagittal sinus. Total en bloc resection of the tumor associated with titan cranioplasty was performed. The postoperative course was uneventful. Three months after surgery the patient no longer reported headache. The anatomical and pathological results concluded in intradiploic cavernous hemangioma. We discuss this case and others described in the literature.

[Alveolar hemorrhage associated with sarcoidosis]. / Sarcoïdose médiastinopulmonaire associée à une hémorragie intra-alvéolaire.

INTRODUCTION: Sarcoidosis is a systemic granulomatous disease of unknown origin. We report an unusual case associated with alveolar haemorrhage. CASE REPORT: An 18-year-old caucasian man was admitted for recent dyspnea. He reported regular tobacco- and occasional cannabis smoking. Lung CT scan revealed mediastinal lymphadenopathy and lung nodules in both fields. Bronchoalveolar lavage recovered bloody alveolar fluid containing many siderophages. Because of rapid deterioration leading to acute respiratory failure, intravenous corticosteroid treatment was started. Improvement was obtained in few days and a diagnosis of sarcoidosis was established based on lymph node biopsy performed under mediastinoscopy. CONCLUSION: This unusual case and those reported in the literature give us the opportunity to describe the characteristics of alveolar haemorrhage associated with sarcoidosis.

Fatal Epstein-Barr virus primo infection in a 25-year-old man treated with azathioprine for Crohn's disease.

We report a case of Epstein-Barr virus (EBV) primo infection with the development of successive infectious mononucleosis, hemophagocytic lymphohistiocytosis, and B-cell lymphoproliferative disorder in a patient treated with azathioprine for Crohn's disease. This case report suggests that specific EBV-related clinical and virological management should be considered when treating a patient with inflammatory bowel disease with azathioprine.

[Follicular bronchiolitis]. / Bronchiolite folliculaire.

INTRODUCTION: Follicular bronchiolitis (FB) is a rare type of cellular bronchiolitis characterised by the presence of hyperplastic lymphoid follicles with reactive germinal centres, distributed along the bronchovascular bundles. OBSERVATION: A non-smoking 36 year old woman was seen because of progressive exertional dyspnoea for 2 years. Chest x-ray and lung CT scan were normal. Pulmonary function tests revealed irreversible airflow obstruction. Exercise testing showed a ventilatory limitation of aerobic capacity with limitation of volume recruitment. The diagnosis of FB was obtained by surgical biopsy. A deficiency of immunoglobulins G4 and M was found. CONCLUSION: The main clinical manifestation of FB is exertional dyspnoea in a young patient. The lung CT scan frequently shows peripheral micronodules but a normal scan does not exclude the diagnosis. The main causes of FB are collagen vascular diseases (especially rheumatoid arthritis) and immunodeficiency syndromes. The treatment of FB is not well defined.

[How to interprete hypercalcitoninemia?]. / Comment interpréter une hypercalcitoninémie?

PURPOSE: Today, calcitonin assay is used for the diagnosis of thyroid medullary cancer in the context of nodular thyroid disease. Calcitonin is an excellent marker of thyroid medullary cancer but some hypercalcitoninemia can also be related to other diseases, such as renal failure, endocrine tumors other than thyroid medullary cancer and sometimes to C cell hyperplasia, which is a not well-defined situation. Recent studies contributed to define calcitoninemia thresholds, which guide decision and avoid excessive invasive treatment. CURRENT KNOWLEDGE AND KEY POINTS: After a brief reminder of physiological role of calcitonin and assays, the difficulties encountered in interpreting hypercalcitoninemia and its potential causes other than thyroid medullary cancer are addressed. Recent studies, on large series, now allow a better knowledge of specificity and sensitivity of calcitonin measurement in patients with nodular thyroid disease and a well-argued management. FUTURE PROSPECTS AND PROJECTS: In the future, calcitonin dosage will be ordered even more frequently, as some authors recommend it for the diagnosis of thyroid nodule. It is up to us to know how to use this remarkable marker, by considering all possible situations of benign hypercalcitoninemia and reserving aggressive treatments for patients who really need them.

[Paraneoplastic acquired ichthyosis revealing non-Hodgkin's lymphoma]. / Ichtyose acquise paranéoplasique révélatrice d'un lymphome non Hodgkinien.

BACKGROUND: Paraneoplastic ichthyosis is a rare affection generally associated with malignant hemopathy. CASE REPORT: A 61 year-old man was seen with a generalized ichthyosis associated with a palmoplantar hyperkeratosis developed over the last 2 years, and an axillary lymph node. Histology of the lymph node revealed a large cell anaplastic lymphoma. Both ichthyosis and palmoplantar keratosis totally disappeared three weeks after adenectomy, without any other treatment. DISCUSSION: This rapid and complete regression of ichthyosis, after the sole ablation of the involved lymph node confirmed the paraneoplastic nature of the disorders of the keratinization (ichtyosis, palmoplantar keratodermia). The review of the literature showed only one case characterized by a rapid disappearance of ichthyosis after surgical treatment of the malignant lymphoma.

Surgical anatomy and embryology of the adrenal glands.

Gross anatomy explains the different surgical approaches to adrenalectomy and the difficulties encountered by surgeons during this procedure. Development of the adrenal glands explains the location of the ectopic sites and excess hormone production by adrenal tumors. The choice of a surgical approach is sometimes difficult and is dependent on (1) the morphology of the body; (2) the volume of the tumor, which necessitates immediate vascular control; and (3) the type of disease, which may necessitate a complete exploration of the abdominal cavity.

Medullary thyroid microcarcinoma: a clinicopathologic retrospective study of 38 patients with no prior familial disease.

Thirty-eight patients (25 women, 13 men; mean age, 57.8 [32 to 91]) showing one or more medullary thyroid microcarcinomas (ie, < 1 cm), with no prior MEN II or medullary thyroid carcinoma history in their family, were reviewed. Follow-up was available for 29 patients (mean, 53.6 months [1 to 147]). 21 patients (72.4%) are alive and free of disease, four patients (13.8%) died during follow-up without disease, 2 patients are alive with disease (local recurrence and persistent hypercalcitoninemia) after 80 and 99 months, respectively, and 2 patients died of disease after 24 and 46 months. Most tumors were incidental pathological findings (19 of 38) or were discovered by systematic blood calcitonin measurement for a nodular thyroid disease (15 of 38). Only the four patients who had an unfavorable outcome were symptomatic cases (palpable micro-MTC, diarrhea, cervical lymph node metastasis and pulmonary metastatic disease). The two patients with metastatic disease at diagnosis died during follow-up. In univariate analysis, a symptomatic medullary thyroid carcinoma was a strong predictor of an unfavourable outcome (p < .00008), as were the preoperative calcitonin level (P = .007) and an elevated postoperative calcitonin level (P = .004). Among 30 histopathological criteria, only the presence of amyloid correlated with an unfavorable outcome (P = .018).

[How to optimize lymph node dissection in colorectal cancers. A technique for simple and efficacious clarification]. / Comment optimiser la dissection des ganglions lymphatiques dans les carcinomes colorectaux. Une technique de clarification simple et efficace.

Studies relating to the prognosis of colorectal carcinomas insist on the quality of mesocolic examination and on the frequency of metastases in minute lymph nodes. The aim of our study was to evaluate an easy, rapid and new clearance method recently described by Koren et al. Twenty-one surgical specimens for colorectal carcinomas have been investigated. After lymph node examination with the traditional method, the mesocolic fat was immersed in a clearing solution. After 6 hours, lymph nodes stood out as white chalky nodules, and were dissected. By the traditional method 182 lymph nodes were detected and 89 additional lymph nodes after clearing. In one case, the detection of a metastasis in lymph node after clearing allowed to upstage the tumour from Dukes B (N0) to Dukes C (N+). This easy and inexpensive method allows the detection of minute lymph nodes and helps to establish a more accurate staging.

[112 cases of sporadic and genetically determined pheochromocytoma: a comparative pathologic study]. / 112 cas de phéochromocytomes sporadiques et génétiquement déterminés: Etude anatomo-pathologique comparative.

The aim of this study was to compare 64 genetically determined pheochromocytomas (PH) (49 MEN IIa, 3 MEN IIb, 6 Von Recklinghausen diseases, 1 von Hippel-Lindau disease, 5 familial pheochromocytomas) and 48 sporadic PH. Genetically determined PH were more often observed among men and more frequently bilateral and multicentric than sporadic PH. Sporadic tumors had more often adrenal capsular invasion, necrosis and pseudocysts. Genetically determined PH were more differentiated with an insular pattern, hyaline globules and a higher percentage of polyhedric cells. Sporadic tumors were less differentiated with more frequently a diffuse pattern and small cells. Adrenal medullar hyperplasia was significantly associated with genetically determined PH. Adrenal cortical hyperplasia was not associated with a particular type of PH. The PS100 and chromogranin immunodetection was equivalent in both groups.

Immunohistochemical study of thrombospondin and its receptors alpha root of beta 3 and CD36 in normal thyroid and in thyroid tumours.

AIM: To describe the pattern of distribution of thrombospondin (TSP1) and its receptors, alpha root of beta 3 integrin and CD36, in normal human thyroid tissue and to compare their expression in different benign and malignant thyroid conditions. METHODS: Immunohistochemistry was used to study TSP1 and its receptors in 40 surgical thyroidectomy specimens (normal parenchyma, 7; follicular adenoma, 4; multinodular goitre, 13; papillary carcinoma, 6; follicular carcinoma, 8; anaplastic carcinoma, 2). RESULTS: In the normal thyroid parenchyma, there was weak expression of TSP1 limited to the vessels with no staining of the extracellular matrix. In goitres, the expression of TSP1 was more pronounced in areas of fibrosis, with staining of alpha root of beta 3 on thyrocytes located in the vicinity. In thyroid adenomas, expression of TSP1 was slightly enhanced compared with normal tissue, located in the basement membrane of vessels. In papillary carcinomas, TSP1 was abundant in the desmoplastic stroma with a cytoplasmic distribution of alpha root of beta 3 integrin in thyrocytes. In follicular carcinomas, TSP1 was less abundant in the extracellular matrix, limited to the vessels of the stroma with a weaker expression of alpha root of beta 3 on thyrocytes than in papillary carcinomas. In anaplastic carcinomas, TSP1 was only present in the numerous capillaries of the stroma, with a marked positivity for alpha root of beta 3 in one case. No immunostaining of thyrocytes is observed with CD36. CONCLUSIONS: These results suggest the importance of the interaction between alpha root of beta 3 integrin and TSP1 during remodelling of the matrix in fibrous goitres with areas of early sclerosis comparable with wound healing. In papillary carcinomas, the overexpression of TSP1 restricted to the stroma suggests protective effects against tumour progression.

[Oxyphilic cell and clear cell carcinoma of the thyroid]. / Les carcinomes à cellules oxyphiles et les carcinomes à cellules claires de la thyroïde.

Oxyphilic cell (Hürthle cell) carcinomas of the thyroid gland, variant of follicular carcinoma, are more malignant than follicular non oxyphilic cell carcinomas with a similar size and degree of invasiveness. Gross features, microscopic features of oxyphilic, clear and bicolored cells carcinomas and diagnostic techniques are related with a differential diagnosis with papillary oxyphilic cell carcinoma.

Medullary thyroid carcinoma: search for histological predictors of survival (109 proband cases analysis).

A group of 13 pathologists belonging to the French Calcitonin Tumor Study Group (GETC: Groupe d'Etude des Tumeurs à Calcitonine) examined the histological slides and medical records of 109 proband cases of medullary thyroid carcinoma (MTC) diagnosed on clinical features. The cases belonged to the various forms of the disease (80 sporadic and 29 familial MTC). The aim of the study was to detect histological predictors for survival by comparing morphological data from patients killed by the disease versus the others. Twenty-seven histological parameters were considered, including cellular heterogeneity, shape of the cells, and cytoplasmic characteristics. Other parameters such as sex, age, and phenotype of the disease were also studied. First, predictive parameters of interest on survival function were selected by univariate analysis (Mantel-Cox test). Then, the extracted parameters were tested in a multifactorial analysis using the Cox's forward stepping proportional hazard model. Five parameters were significantly associated with a lower survival function: presence of necrosis in the tumor (P = .001), squamous pattern (P = .002), age over 45 years (P = .004), presence of oxyphil cells in the tumor and absence of cells with intermediate cytoplasm (P = .025), less than 50% of calcitonin immunoreactive cells in the tumor (P = .04).

[Anatomo-clinical prognostic factors of papillary carcinoma of the thyroid. Multivariate analysis: report of a series of 52 cases]. / Facteurs pronostiques anatomo-cliniques des carcinomes papillaires de la thyroïde. Analyse multivariée: à propos d'une série de 52 cas.

A retrospective study about 52 cases of papillary thyroid carcinomas was carried out with emphasis on histopathological features. The mean follow up period was 10 years. The survival curves were estimated using the Kaplan-Meier method and compared using the log rank test. The multivariate analysis was performed using the Cox's regression model. In univariate analysis, age, Tp (histopathological extension of the tumor), histological differentiation, VAN score (Vascular invasion nuclear Atypia tumor Necrosis) of Akslen and the LeuM1 expression were significant prognostic factors. In multivariate analysis, the Tp and histological differentiation were associated with high risks of poor outcome.

Evaluation of the monoclonal antibody antithyroperoxidase MoAb47 in the diagnostic decision of cold thyroid nodules by fine-needle aspiration.

Fine needle aspiration (FNA) of cold thyroid nodules has become the first line diagnostic decision for electing which patients need surgery. In order to improve FNA accuracy, the monoclonal antithyroperoxidase (TPO) antibody (MoAb47) was tested. A total of 554 patients were included in this study and among them, 208 were referred to surgery. The results of FNA compared to the final histological diagnosis revealed a sensitivity and a specificity of 94 and 55% respectively, while the sensitivity and specificity of TPO immunodetection on the same cases reached 98 and 83% respectively. By combining the two methods, the 3 false-negative of FNA and 60% of suspicious cytology corresponding to histological benign lesions were correctly identified by immunocytochemistry. With better results than FNA alone, TPO immunodetection with MoAb47 represents a useful adjunct to conventional cytology for selecting patients for surgery.