[Multiple bilateral pulmonary nodules revealing a congenital cystic adenomatoid malformation]. / Lâcher de ballon révélateur d'une malformation adénomatoïde kystique congénitale du poumon.

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare congenital developmental abnormality, representing about 25% of all congenital lung lesions. It is very rare that presentation is delayed until adulthood. We report a case of 63-year-old woman without notable pathological antecedents in whom a systematic chest X-ray revealed multiple bilateral pulmonary nodules. The patient was asymptomatic and her physical examination was normal. CT scan showed bilateral liquid rounded thin-walled densities of various size, with a homogeneous non calcified content. The diagnosis was based on radiological findings and surgery. The result of histopathological examination obtained by thoracic surgery confirmed CCAM without malignancy. The postoperative follow up showed an excellent recovery.

[Cerebral actinomycosis pseudotumor: a case report]. / Actinomycose cérébrale d'aspect pseudotumoral: à propos d'un cas.

INTRODUCTION: Cerebral actinomycosis is rare and difficult to diagnose. CASE REPORT: We report a case of a 45-year-old man hospitalized for seizures associated with fever and left hemiparesis. The white cell count and C-reactive protein were elevated. HIV serology was negative. Blood cultures remained sterile. The CT scan revealed hyperdense nodular lesions in the occipital area, with annular contrast uptake and peripheral edema causing a mass effect, suggestive of brain metastasis. The pathology examination of a surgical specimen disclosed cerebral actinomycosis. A dental origin of the infection was suspected. Hemiparesis remained after a 12-month antibiotic regimen associated with dental care and short-term corticosteroid therapy. CONCLUSION: Actinomycosis should be discussed as a possible diagnosis for all cerebral lesions, particularly in patients with a potential dental infection. Histology is required for positive diagnosis. Antibiotic therapy alone is generally sufficient; surgery is often performed for diagnostic purposes.

Amyloid goiter: first manifestation of systemic amyloidosis.

Amyloidosis is an abnormal extracellular deposit, which can occur in several tissues. The mechanism is not clearly defined. In systemic amyloidosis, all the organs can be infiltrated, but amyloid goiter as the initial manifestation of systemic amyloidosis is an exceedingly rare condition. We report a rare case of a patient who presented an amyloid goiter as the first manifestation of systemic amyloidosis. This patient had a known Crohn's disease. He developed a goiter without compressive complications. Histologic examination revealed a diffuse amyloid deposition surrounding thyroid follicles. The gland was enlarged with an eosinophilic and amorphic deposit. Confirmation of amyloid was made by the presence of congophilia and apple-green birefringence under polarized-light microscopy. An immunoreactivity was seen with AA protein. Amyloid goiter is a rare manifestation of amyloidosis. About 250 cases of amyloid goiter have been reported in the literature. The goiter enlarges rapidly and progressively, often becoming compressive like thyroid cancer. The prognosis depends on the treatment of the amyloidosis and the underlying chronic disease.

[An exceptional carotid tumor]. / Une tumeur rare du glomus carotidien.

Carotid paraganglioma (CP) is a relatively rare neoplasm, most commonly located in the head and neck. It is a slow-growing tumor and there has been some debate on the optimal techniques of excision. We report herein the case of a 35-year-old woman who presented with an asymptomatic mass in the right side of the neck. The diagnosis of CP was confirmed by computed tomography and carotid angiography. This tumor was successfully removed surgically without complication. Throughout the description of this case, we analyze the clinical, radiological, pathological and therapeutic particularities of this entity.

[Intracerebral schwannoma: case report]. / Schwannome intracérébral: étude anatomoclinique d'une observation.

BACKGROUND AND PURPOSE: We report a well-documented case of intracerebral schwannoma, presenting as a cystic and solid parietal mass, arising in a 20-year-old woman. Possible mechanisms underlying the histogenesis of this rare lesion are discussed. METHODS: The clinical, radiological and pathological features are described. The relevant literature is reviewed. RESULTS: The patient presented seizures and elevated intracranial pressure. Neuroradiologic findings showed a right parietal lesion with cystic and tissular components, intensely enhanced after injecting intravenous gadolinium. The tumor was removed through a right parietal craniotomy. Histological and immunochemical features confirmed the diagnostic of intracerebral schwannoma. The patient is alive without progressive local disease or metastasis. The origin of intracerebral schwannomas has been the source of much controversy. The most popular hypothesis argues that these tumors arise from the proliferation of Schwann cells in the perivascular nerve plexii. CONCLUSIONS: Intracerebral schwannoma is an extremely rare benign tumor. About fifty cases have been reported. The importance of recognizing this tumor is stressed, particularly in younger patients, given its benign nature, radiological resemblance to other tumors and favorable response to resection without toxic treatment.

[Epithelial-myoepithelial carcinoma of the nasal cavity]. / Carcinome épithélial-myoépithélial de la cavité nasale.

OBJECTIVES: Epithelial-myoepithelial carcinoma is a rare tumor of the salivary glands with an incidence of less than 1%. Most cases arise in the parotid gland. Extraoral location is exceptional. The purpose of this study was to describe a case with nasal location, which is exceptionally reported in the literature. MATERIALS AND METHODS: We report a case of epithelial-myoepithelial carcinoma arising in the nasal cavity of a 54-year-old woman. RESULTS: The woman presented with right recurrent epistaxis and on nasal endoscopic examination was found to have a polypoid tumor in the right nasal cavity. The CT-scan demonstrated a soft tissue mass without extension to the rhinopharynx or bony destruction. Histopathologic examination revealed the tumor to consist of a mixture of a solid, tubular, and trabecular structures with a double-layered arrangement of inner eosinophilic cells and outer clear cells. Dual differentiation toward myoepithelial and epithelial cells was confirmed immunohistochemically. CONCLUSION: The occurrence of epithelial-myoepithelial carcinoma in the nasal cavity is possible. In published cases, no recurrence or metastasis has been reported in this location.

[Sclerosing hemangioma of the lung: a rare lesion with a difficult diagnosis]. / L'hémangiome sclérosant du poumon: une lésion rare et de diagnostic difficile.

Sclerosing hemangioma of the lung is a rare lesion described for the first time in 1956 by Liebow. We report a case in a 45 year-old woman who was admitted for exploration of chronic cough. The chest x-ray revealed a round opacity, well delimited in the left pulmonary parenchyma. Surgical resection enabled the histopathological diagnosis of sclerosing hemangioma. We review progress in our knowledge of the histogenesis and diagnosis of this tumor. Immunohistochemistry has been highly contributory although numerous points remain controversial.

[Aorticopulmonary paraganglioma. A case report]. / Paragangliome aortico-pulmonaire. A propos d'un cas.

Paraganglioma is a tumor which develops from the paraganglion system, generally in the adrenal medulla (90%). Thoracic localisations are exceptional and arise essentially for the aortic and subaortic bodies, leading, in this case, to an anterior and posterior localisation. We report a case of non-chromaffin non-secreting aorticopulmonary paraganglioma, discovered fortuitously in a 64-year-old man.

[Primary digestive tract Kaposi sarcoma with idiopathic CD4+ lymphocytopenia, HIV negative, HHV8 positive]. / Sarcome de Kaposi digestif primitif avec lymphocytopénie CD4 idiopathique, VIH négatif, HHV8 positif.

A 52-year-old Tunisian patient had fever, impaired health and several opportunistic infections (Campylobacter jejuni, Mycobacterium hominis, Herpes virus, Giardia intestinalis, Vibrio metschnikovii). Lymphocytopenia was noted (348/mm3; CD4+: 2.2%; CD4+/CD8+: 0.1). Polymerase chain rection search for HIV was negative in serum and in tumor tissue. Diagnosis of primary digestive Kaposi sarcoma was established at autopsy due to the deep location of the lesions. There was an ulcerofungating tumor spreading over 1.3 m of the duodenojejunum. This is the fourth reported case of CD4+ lymphocytopenia, a new and very rare immunodeficiency syndrome recently defined by the Centers for Disease Control. We detected human herpes virus 8 by immunohistochemistry of tumor tissue. Human herpes virus 8 is implicated in the pathogenesis of Kaposi sarcoma.

[Infantile fibrosarcoma: a clinicopathological and molecular study of five cases]. / Fibrosarcome infantile: étude anatomo-clinique et moléculaire de cinq cas.

We report 5 cases of infantile fibrosarcoma (4 boys and 1 girl) whose average age was 5, 7 months (range 0 days to 14 months). The tumor was congenital in 4 cases. All tumors presented in the extremities (forearm, hand, thigh: 1 case, lower leg: 2 cases). Treatment was based on surgery (3 cases: amputation, 2 cases: local excision) with a favorable course in all cases, even those with marginal excision (follow-up ranging from 5 to 21 years). The lesions were characterized by dense monotonous cells growing in a fascicular pattern, with small necrotic areas and scattered lymphocytes. The mitotic index was high (average 8/10 high-power fields). ETV6-NK3 chimeric RNA was detected by reverse transcriptase polymerase chain reaction in two cases out four cases from paraffin-embedded tissue blocks. The infantile fibrosarcoma is a good prognosis tumor characterized by particular histological features and ETV6-NK3 gene fusion.

[Peritoneal pseudomyxoma associated with an ovarian and appendicular mucinous tumor]. / Pseudomyxome péritonéal associé à une tumeur mucineuse ovarienne et appendiculaire.

The authors report a case of pseudomyxoma peritonei associated with an appendicular and ovarian mucinous tumor. They emphasize the rarity of bilateral ovarian and appendicular involvement and they discuss the problem of the origin of the primary tumor and evaluation of the prognosis.