[Cerebral hemangiopericytoma. Ultrastructural study of one case]. / Hémangiopéricytome cérébral. Etude ultrastructurale d'un cas

The authors report electron-microscopic observations upon a primitive cerebral haemangiopericytoma. The vascular appearance of the tumour is due to the presence of abundant extracellular material which has a structure like that of vascular basement membranes. The fact that the tumour cells are pericytes is confirmed by the existence of intracytoplasmic microfilaments of 60-80 A in diameter, sometimes gathered into osmiophilic aggregations and forming simple cellular junctions (zonulae adherentes). Stress is laid upon the importance of differentiating this rare tumour from an angioblastic meningioma; the haemangiopericytoma is more rapidly growing and carries a more serious prognosis.

[Atypical mesoblastic nephroma with metastases right away?]. / Néphrome mésoblastique atypique avec métastases d'emblée?

The diagnosis of a metastatic kidney tumor arising in a 2-month infant is discussed between atypical mesoblastic nephroma and clear cell sarcoma. The precocity of distant metastases, their location in bone marrow, liver and thoracic soft tissues, and their association with myelofibrosis set up an original clinical presentation which seems to have never been described elsewhere. Treatment strategy with surgery of the primary followed by a polychemotherapy combining vincristin-etoposide-ifosfamide and the short term follow-up are reported.

[Synovialo-sarcoma of the laryngopharynx: electron microscopic study (author's transl)]. / Synovialo-sarcome pharyngé. Etude ultrastructurale d'un cas.

The authors report a new case of synovialo sarcoma of the laryngo-pharynx by optic and electron microscopy. Ultrastructural features were similar to those of joint synovialo-sarcoma, i-e, showing glandular like and spindle cell componments. Both of these shared several ultrastructural characteristics, favouring the hypothesis of a common origin. The site and the fine structure of this tumor are discussed according to the literature.

[Duodenal sarcoidosis with selective IGA deficiency and lymphoid nodular hyperplasia]. / Sarcoïdose duodénale avec déficit sélectif en IgA et hyperplasie nodulaire lymphoïde.

Coexistence of IgA deficiency with sarcoidosis has been reported occasionally. Enteropathy occurs exceptionally in this association. We report the case of a 45 year-old woman experiencing disseminated sarcoidosis which involved mediastinum, chest, lymph nodes, liver, bone marrow, and skin. Immunoglobulin deficiency involving essentially IgA was found 15 years later. Digestive investigations showed gastroduodenal specific localizations associated with lymphoid nodular hyperplasia without villi atrophy.

[Major hypoproteinemia revealing enteritis caused by Yersinia enterolytica and cytomegalovirus at the onset of chronic lymphocytic leukemia]. / Hypoprotidémie majeure révélatrice d'une entérite à Yersinia enterolytica et cytomégalovirus à la phase initiale d'une leucémie lymphoïde chronique.

We report the first case of probable protein-losing enteropathy revealing a cytomegalovirus/Yersinia enterolytica infection at the onset of a chronic lymphocytic leukaemia. Severe hypoprotidaemia, digestive tract yersiniosis, ulcerative and microgranulomatous enteritis with a large number of cytomegalic inclusions in mucosal cells, and incipient lymphoid proliferation were the most characteristic findings.

[Hepatic granulomatosis associated with mononucleosis syndrome secondary to cytomegalovirus infection: apropos of 2 cases in healthy adults]. / Granulomatose hépatique associée à un syndrome mononucléosique secondaire à une infection à cytomégalovirus: à propos de deux cas chez l'adulte sain.

In this study, the authors report two observations of granulomatous hepatitis. The secondary appearance of a mononucleosis syndrome, three weeks after the onset of fever, in healthy adults, evoked the diagnosis of a cytomegalovirus infection. The authors insist on the histologic and virologic differences of the CMV infection between the healthy adults and the immunodepressed patients. They also note the difficulties of the diagnosis of the CMV infection in healthy adults.

[Extensive pharyngeal necrosis: an unusual complication of dermatomyositis]. / Nécrose pharyngée extensive: complication exceptionnelle d'une dermatomyosite.

To our knowledge, the occurrence of extensive pharyngeal necrosis in patients with dermatomyositis has not yet been reported. We report such a case characterized by a vast ulceration of the posterior laryngeal wall, extending from the velum palati to the larynx and responsible for both dysphagia and dysphonia. Treatment with an immunosuppressant had no effect on the lesions.

[Severe upper airway obstruction in infectious mononucleosis: a life emergency]. / L'obstruction sévère des voies aériennes supérieures au cours de la mononucléose infectieuse: une urgence vitale.

BACKGROUND: Upper airway obstruction can represent a severe, life-threatening complication of infectious mononucleosis. We report a rare case of airway obstruction in a child with infectious mononucleosis associated with herpes virus infection, and we discuss management strategy that can be proposed in such cases. CASE REPORT: A 9-year-old girl was hospitalised in intensive care unit for obstructive dyspnea during infectious mononucleosis. Despite five days of corticosteroids and tracheal intubation, persistent pharyngo-tonsillar tumefaction led us to perform a surgical adenotonsillectomy. This latter treatment allowed immediate tracheal extubation and a rapid recovery. Histology showed a herpes virus infection associated with infectious mononucleosis. CONCLUSION: Maintaining airway opening in infectious mononucleosis needs sometimes to use instrumental interventions: nasal trumpet, endotracheal intubation, even tracheostomy. Early tonsilloadenoidectomy may relieve airway obstruction and allow a rapid recovery in the most severe cases. Airway obstruction in infectious mononucleosis may be aggravated by concomitant herpes virus infection that should be searched for in this situation, in order to adapt the treatment.

[Pleomorphic adenoma of the parotid: histopathological study]. / Adénome pléomorphe parotidien. Etude histopathologique.

OBJECTIVES: Capsular ruptures play a major role in recurrences of parotid pleomorphic adenomas. The aim of this retrospective study was to define histomorphological characteristics of pleomorphic adenoma in order to define possible recurrence mechanisms and to set a clear surgical management. MATERIAL AND METHOD: Histological study was performed after reviewing of slides originating from 100 patients with pleomorphic adenomas of the parotid gland. These patients were treated in our institution during the period May 1992 - November 2002. RESULTS: The studied population was distributed into 3 histological subtypes for better analysis. Hypocellular (stroma rich) pleomorphic adenoma was reported in 56%, hypercellular pleomorphic adenoma was reported in 29% and "classic" in 15%. Thinness of capsule was significantly related to hypocellularity. Pseudopodias and satellite nodules were reported in 72% of this series. CONCLUSION: Due to capsular characteristics, surgical excision should avoid dissection in the vicinity of the tumor in order to avoid capsular bare area, so minimizing the risk of surgical induced recurrence. According to these findings, enucleation surgery for pleomorphic adenoma should not be performed anymore. Parotidectomy techniques (total or lateral) constitute the surgical procedures of choice.

[Pleomorphic adenoma of the nasal cavity: a case arising from the wall of the maxillary sinus]. / Adénome pléomorphe des fosses nasales: une localisation à la cloison inter sinuso nasale.

INTRODUCTION: Pleomorphic adenoma is a common and benign tumour, originating from sero-mucous glands. Location in the nasal cavity and particularly on the medial wall of the maxillary sinus is very rare. PATIENTS AND METHODS: One patient with a pleomorphic adenoma of the nasal cavity and a review of literature are reported in order to describe the diagnostic and therapeutic management of this tumour. RESULTS: A 63-year-old woman was referred to our institution for evaluation of an unilateral nasal tumour. Clinical and radiological studies revealed an intra-nasal tumour, slightly lytic in nature, arising from the mucosa of the medial wall of the maxillary sinus. More than 2 years after complete excision of the tumour using an endoscopic procedure, the nasal cavity was free of tumour. DISCUSSION: Pleomorphic adenoma of the nasal cavity is a rare and benign tumour, usually presenting as a unilateral nasal tumour. Prognosis of this tumour is mainly linked to recurrences in cases of incomplete resection and to possible metastasis, such as an eventual malignant evolution. The gold standard treatment is complete surgical resection. After a complete imaging study for patient selection (CT scan and MRI), endonasal endoscopic surgery seems to be a reliable therapeutic treatment. CONCLUSION: Intra-nasal pleomorphic adenoma originates from nasal seromucous glands. Radiological study reveals a slightly lytic tumour developed from the septum of the medial wall of the maxillary sinus. Resecton can be performed with endoscopic endonasal surgery as long as the orbital bony framework and the ethmoidal roof are free of tumour. Whatever technique is employed, long term follow-up has to be used to detect any recurrence.

[Diagnosis management of Warthin tumour: clinical presentation, fine needle cytology and MRI]. / Approche diagnostique des tumeurs de Warthin: clinique, cytoponction et IRM.

OBJECTIVES: Warthin tumour is the second most frequent benign tumour of the parotid gland after pleomorphic adenoma. The aim of this retrospective study was to define clinical, cytological and MRI characteristics in order to work out a therapeutic strategy. MATERIAL AND METHOD: During the period May 1991-January 2003, 53 patients with Warthin tumors were treated in our institution. Clinical data were reported from medical records reviewing. Histological diagnoses were compared with FNAB and MRI results. RESULTS: Warthin tumours represented 13% of all parotid lesions of our series. Mean age of patients was 58 year-old. Sex ratio was 3.8 men and 1 woman. Sensitivity of FNAB for Warthin tumour diagnosis was 75% while positive predictive value was 71%. Six cases of false positive patients were reported: 2 acinic cells carcinomas, 2 pleomorphic adenomas, 1 dermoid cyst, 1 branchial cyst. CONCLUSIONS: Surgical management is based on partial parotidectomy that may be adapted to the tumour location. Because of elderly patients, benign nature and low risk of malignant transformation of Warthin tumours, one might prompt to adopt a conservative strategy, avoiding surgery in some cases. Despite diagnosis of Whartin tumour on FNAB and MRI, one must be cautious in recommending conservative treatment in order to avoid ignoring a surgical tumour.

[Recurrences of pleomorphic adenomas of the parotid: development of concepts]. / Récidives des adénomes pléiomorphes parotidiens. Evolution des concepts.

OBJECTIVES: The aim of this study was to examine the surgical and pathological factors that led to recurrent parotid pleomorphic adenomas. The study also tried to determine best practice for the treatment of recurrence. In addition the study looked at the correlation between fine needle cytology and definitive histology. MATERIAL AND METHOD: Histological study was undertaken on 100 pleomorphic adenomas surgically removed from patients in our institution between 1992 and 2002. Study of diagnostic value of fine needle cytology aspiration and MRI for pleomorphic adenoma diagnosis was performed in 181 patients operated on for parotid tumors. RESULTS: Hypocellular pleomorphic adenomas often have a thin capsule and constitute the most frequently encountered histological type in recurrence. Pseudopodias are considered as an additional factor in recurrence. In our series, cytological study had an excellent diagnostic value with a sensitivity of 92% and a PPV of 96%. MRI study had a sensitivity of 83% and a PPV of 89%. CONCLUSIONS: According to these findings, enucleation surgery on a pleomorphic adenoma should not be performed anymore. Parotidectomy techniques (total or lateral) constitute the surgical treatment of choice. Fine needle aspirate cystology in a useful diagnostic procedure. Management of recurrences is based on surgery with total parotidectomy and facial nerve preservation. Role of radiotherapy is still indeterminate.

[Recurrences of pleomorphic adenomas of the parotid: changing attitudes]. / Récidives des adénomes pléiomorphes parotidiens: évolution des concepts.

OBJECTIVE: The real issue of parotid surgery for pleomorphic adenoma is its risk of recurrence. The aim of this study was to compare the changing attitudes towards the pathology and surgical treatment in order to better define prevention and management of pleomorphic adenoma recurrences. MATERIAL AND METHOD: Histological study was performed after reviewing of slides originating from 100 patients with pleomorphic adenomas of the parotid gland, treated in our institution during the period May 1992 - November 2002. Study of the diagnostic value of fine needle cytology aspiration and MRI for pleomorphic adenoma diagnosis was performed for 181 patients operated on for parotid tumors. RESULTS: Hypocellular pleomorphic adenomas often have a thin capsule and constitute the most frequently encountered subtypes of recurrence. Pseudopodia are considered as an additional risk of recurrence. In our series, cytological study gave excellent diagnostic value with a sensitivity of 92% and a PPV of 96%. MRI study had a sensitivity of 83% and a PPV of 89%. CONCLUSIONS: According to these findings, enucleation surgery on a pleomorphic adenoma should not be performed any more. Parotidectomy techniques (total or superficial) constitute the surgical treatment of choice. The management of recurrences is based on surgery, with total parotidectomy and facial nerve preservation. The rôle of radiotherapy is still indeterminate.

[Nodules and paranodular lesions: a trial of anatomo-clinical correlation]. / Nodules et formations paranodulaires: essai de correlation anatomo-clinique.

The use of increasingly powerful diagnostic tools has enabled the laryngologist to refine the diagnosis of nodular lesions, and, within this group, to isolate clinical variations, paranodular lesions, mucosal thickening, mucosal pseudo-cysts. Systematic comparison between the clinical and anatomopathological diagnosis of the lesions operated upon does not make it possible to establish a strict correlation between the clinical and the histological aspects. Nevertheless, histology appears to throw light on the physiopathology of such lesions within the field of dysfunctional laryngopathies.

[Extramedullary plasmocytoma of the nasal cavity: a case report]. / Plasmocytome solitaire extra-médullaire de la fosse nasale: à propos d'un cas.

PURPOSE OF STUDY: To establish a diagnosis and therapeutic management in patients with extramedullar plasmocytomas. METHOD: There is no consensus concerning extramedullar plasmocytoma treatment. One patient with a nasal cavity tumor location and a review of literature are reported. RESULTS: Extramedullar plasmocytoma is a rare tumor that occurs most frequently in the upper respiratory tract. The most common location is the nasal cavity. Diagnosis can only be made after histological and immunohistochemical examinations. Localized tumors are treated by radiation therapy, combined or not with surgery. Disseminated diseases are treated by chemotherapy. CONCLUSION: Diagnosis can only be confirmed after the exclusion of a systemic disease (multiple myeloma).

Fine-needle aspiration cytology in the management of parotid masses: evaluation of 249 patients.

INTRODUCTION: The role of fine-needle aspiration cytology (FNAC) in the management of parotid tumours is still the subject of controversy. The purpose of this study was to determine the diagnostic value of FNAC in our institution in order to define its place in the diagnostic strategy. PATIENTS AND METHODS: This retrospective study was based on 249 patients who had undergone preoperative FNAC before being operated in our institution between 2001 and 2008. All examinations were performed and interpreted by the same experienced pathologist. RESULTS: Among the 249 patients included in this study, 187 (75%) had a benign tumour and 62 (25%) had a malignant tumour. No complications of FNAC were observed. Cytological findings were non-contributory in 47 patients (18%). The sensitivity of FNAC for the diagnosis of malignancy was 80% with a specificity of 89.5%. Among the 11 false-negative results, lymphomas and low-grade mucoepidermoid carcinomas were the most common histological types. Among the 16 false-positive results, Warthin's tumours, pleomorphic adenomas and lymphoepithelial lesions were the most common histological types. Accurate histological classification of the tumour was reported in 79.5% of cases (86% for benign tumours and 44% for malignant tumours). CONCLUSION: FNAC is a reliable examination providing important information to the surgeon in the preoperative diagnostic assessment.

[Neonatal botryomycosis]. / Tuméfactions multiples chez un nouveau-né révélant une botryomycose.

Botryomycosis is an uncommon bacterial infection. It occurs in two forms: cutaneous and visceral. Fewer than 30 pediatric cases have been reported. We present the first case of a 14-day-old newborn with botryomycosis revealed by four tumefactions located in the inguinal and popliteal hollows, without a biological inflammatory syndrome. Pathological examination of the sample demonstrated botryomycosis. The culture collection found Staphylococcus aureus. Progression was favorable with appropriate prolonged antibiotic therapy. Predicting factors such as immunodeficiency or cystic fibrosis were excluded.

Association of three different congenital malformations in a same pulmonary lobe in a 5-year-old girl.

We report the case of a 5-year-old girl with persistent chest X-ray abnormalities following an episode of pneumonia who has a complex congenital pulmonary malformation comprising of a congenital pulmonary airway malformation, an intralobar sequestration and two bronchogenic cysts, all present within the same lobe. The observation suggests a common embryological origin of these malformations.