[Place of the microscopic examination in the diagnosis of costal fibrous dysplasia]. / Place de l'examen anatomopathologique dans le diagnostic de la dysplasie fibreuse costale.

BACKGROUND: Fibrous dysplasia of bone is a rare benign lesion characterized by the coexistence of a fibrous tissue and an immature osteogenesis. Costal localization is rare and may be monostotic or polyostotic. The diagnosis may be suspected based on clinical and radiological findings. Facing the development of radiological investigations, we tried to highlight the diagnostic role of the microscopic examination through the experience of our department. METHODS: We describe a retrospective study about 12 costal fibrous dysplasias diagnosed over a 17-year-period. Clinical records were retrieved from the department of thoracic surgery of the same hospital. RESULTS: Costal fibrous dysplasia is equally observed in men and women with predominance in the third and fourth decades. Clinical symptoms consist mainly in chest pain. Physical examination was normal in almost all cases. Based on the radiological findings, the diagnosis was suspected in 33% of the cases. Microscopic examination highlighted the diagnosis in all cases but it was challenging in one case and necessitated a multi-disciplinary approach. The difficulties encountered were due to artifact decalcification. CONCLUSION: Costal fibrous dysplasia is a benign lesion which diagnosis is based on microscopic features. Radiologic investigations show nonspecific features but allow to rule out a malignant tumor. The outcome of the patients is generally good except in rare cases with a malignant transformation.

[Bronchopulmonary carcinoid tumors: Tunisian experience with 115 surgically treated cases]. / Tumeurs carcinoïdes bronchopulmonaires : une série tunisienne de 115 cas opérés.

INTRODUCTION: Bronchial carcinoid tumors are rare well-differentiated, neuro-endocrine tumors, with low-grade malignancy. They are distinguished in two different groups: typical and atypical carcinoid. The purpose of this study was to review clinical, surgical and pathological characteristics in patients with primary bronchopulmonary carcinoid tumors. METHODS: This retrospective study included 115 bronchial carcinoid tumors surgically treated at a single institution during a twenty-one-year period, between 1992 and 2012. RESULTS: There were 56 men and 59 women, with a mean age of 43.73 years. Hundred patients had typical carcinoids and 15 atypical carcinoids. The majority of patients were symptomatic (96.52%). CT revealed a proximal mass in 51% which was obstructive leading to ventilator disorders in 80%. Bronchoscopy showed an endo-bronchial tumor in 83.48% of the cases. Anatomical resection had been achieved among 99 patients, and a conservative resection among 16 patients with typical carcinoid tumor. Lymph node metastases were present in 12.17% of the cases. Follow-up revealed recurrence in one patient and distant metastasis in 4 others. The 5-year survival rate was worse for atypical carcinoid with 45% than typical carcinoid with 95%. CONCLUSIONS: Carcinoids are rare malignant tumors, and their outcome is usually favorable after surgery. However, local recurrence and/or metastases can occur with both typical and atypical carcinoid tumors, justifying the need of prompt diagnosis and long-term follow-up. The factors influencing the survival included the pathological type, distant metastasis and mediastinal lymph node involvement.

[Pulmonary zygomycosis in a diabetic child complicated with thrombus of the left atrial auricle]. / Mucormycose pulmonaire chez une enfant diabétique, compliquée d'un thrombus intra-auriculaire gauche.

Mucormycosis is a rare but fatal, opportunistic fungal infection caused by fungi of the order of mucorales in the class of Zygomycetes. Isolated pulmonary mucormycosis is rare and occurs principally in particular conditions, especially in patients with uncontrolled diabetes. The fungi invades the blood vessels and causes distal ischemic necrosis. We report a case in a 13-year-old girl with diabetes mellitus who developed pulmonary zygomycosis complicated with thrombus of the left atrial auricle. The diagnosis of mucormycosis was confirmed by histologic examination of endobronchial biopsies. Combined treatment with systemic amphotericin B and surgery resulted in a favorable outcome. This difficult diagnosis must be raised in diabetic patients with unresponsive lung infections in order to apply early aggressive therapy. Successful management continues to be early diagnosis, followed by systemic antifungal therapy and surgical resection combined with control of the underlying disease.

[Tracheal tumor treated as asthma]. / Une tumeur trachéale traitée comme un asthme.

Primary tumors of the trachea are very rare. In adults, the majority of them are malignant. Schwannomas are exceedingly rare benign tumors in the tracheobronchial tree. We report a case of a 37-year-old man who was hospitalized for increasing dyspnea. He had been treated for bronchial asthma for the last 4 years with no benefit. The CT scan of the chest and bronchoscopy identified a tracheal mass that was prolapsed in the left stem bronchus. The patient did not remain free of disease after endoscopic laser resection. So, surgical resection was made. The tumor was excised at its base. A segment of the left stem bronchus was removed and primary anastomosis was performed. The histopathologic diagnosis was of a benign schwannoma without malignant elements. There was no recurrence during the follow-up period. This case demonstrates that intratracheal masses should be considered in patients with dyspnea or in patients with asthma refractory to conventional therapy.

Fibroadenoma in an ectopic vulvar breast gland: a common neoplasm in an uncommon site.

Ectopic breast tissue is defined as glands located outside of the breast. It can be found anywhere along the milk line extending from the axilla to the groin, and can occur in the vulva. Ectopic breast tissue should be excised because it may develop benign or malignant pathologic processes. Less than 40 cases of fibroadenoma in the vulva have been reported in the literature. We report a case of a 37-year-old woman presenting a solitary vulvar mass. The mass was excised completely, and histology demonstrated an ectopic breast fibroadenoma. This is one of the few reports on the benign pathologies of vulvar mammary glands.

[Case report of pulmonary biphasic tumor with unusual follow-up]. / À propos d'une tumeur biphasique du poumon d'évolution inhabituelle.

BACKGROUND: Pulmonary blastoma, a rare primary lung malignancy is subdivided in 3 categories: well-differentiated fetal adenocarcinoma (WDFA), classic biphasic pulmonary blastoma (CBPB) and pleuropulmonary blastoma (PPB). Classic pulmonary blastoma is composed of a mixture of immature epithelial and mesenchymal tissue resembling fetal lung tissue. CASE REPORT: We described a case of a 48-year-old male, cigarette smoker, who presented with left thoracic pain and hemoptysis for 2 months. Chest radiography showed a well-delimited, homogeneous 4cm mass in the left lung periphery. Bronchoscopic examination revealed left endobronchial bleeding. Computed tomography of the chest revealed a tumor shadow measuring 7cm in the left upper lobe and bilateral nodules with no lymphadenopathy. A systemic evaluation demonstrated no metastatic lesion. Patient underwent a left upper lobectomy. The diagnosis of CBPB was affirmed on anatomopathology of the tumor resection. Immunohistochemical studies showed that tumor cells were positive for vimentin, desmin, actin, Pan Cytokeratin and TTF-1. The final diagnosis was BPB classified as pathological T3N0M0 and no adjuvant treatment was associated. The patient showed good objective response with no evidence of disease recurrence still in 5 years surgery resection. CONCLUSION: This case reiterates the importance of pathomorphological or immunohistochemical features in diagnosis of BPB.

[Thoracic chest wall fistula formation]. / Une suppuration thoracique fistulisante Actinomycose thoracique avec atteinte pulmonaire et pariétale.

Thoracic actinomycosis is a suppurative infection which can be difficult to diagnose as its presentation may mimic cancer or tuberculosis. We report a new case of thoracic actinomycosis in a 35-year-old man who presented with thoracic symptoms associated to a productive parietal fistula. Imaging exploration revealed an opacity of the right ventroapical segment with parietal infiltration. A bilobectomy and a parietectomy were performed. The anatomopathologic diagnosis actinomycosis was confirmed. The patient was first put on a treatment of azathioprine 1g daily during two weeks, then switched to a combination with Vibramycin 100 mg twice a day during 17 months, The evolution was marked by the persistence of productive fistulae, which were treated surgically, and resistance to the initial treatment leading to a switch to Augmentin 3 g daily during 25 days. The patient experienced clinical improvement with a follow up of 18 months than was lost to follow-up.

[Orbital metastasis of liposarcoma]. / Métastase orbitaire d'un liposarcome.

INTRODUCTION: Liposarcoma, the most common soft tissue sarcoma in adults, rarely involves the orbit. Primary orbital liposarcomas are extremely rare, with less than 30 cases previously reported. Metastatic orbital liposarcomas are also extremely rare, with only a few cases documented in the literature. We report a new case of an orbital metastatic liposarcoma and review the literature. OBSERVATION: A 57-year-old man with a history 5 years before of myxoid liposarcoma on the right calf, completely surgically resected, was admitted for recent proptosis of the left eye. A computed tomographic scan revealed a heterogeneously enhancing, left orbital mass measuring 30x23x20 mm. The mass adhered to the internal medial muscle without extension to the optic nerve. A biopsy was taken, which showed typical aspects of myxoid liposarcoma. The patient underwent an orbital left exenteration after first refusing any proposed treatment. CONCLUSION: Liposarcoma metastatic to the orbit is exceptional. It should be suspected in a patient with exophthalmia caused by a space-occupying lesion and a history of liposarcoma.

Primary central nervous system malignant melanoma: report of 5 cases.

Primary melanocytic neoplasms of the central nervous system consist of a spectrum of lesions ranging from well-differentiated melanocytoma to its overtly malignant counterpart, melanoma. Clinicopathologic features of 5 cases of primary central nervous system malignant melanoma were studied to define histologic appearances, diagnostic criteria, and the clinical behavior of lesions along this spectrum. There were three women and two men (mean age was 35 years) with symptoms of increased intracranial pressure. Radiological findings showed a solitary intra parenchymal lesion with variable enhancement after gadolinium administration. All patients underwent surgical treatment. Histologic examination revealed primary melanoma.

Desmoplastic small round cell tumor of the abdomen. Report of two cases.

Desmoplastic small round cell tumor (DSRCT) is a rare clinicopathological entity individualized in 1989. Its etiopathogenesis is still unknown, and diagnosis can be achieved only by immunohistochemistry and cytogenetic studies. The objective of this work is to report two new cases of DSRCT and to review the literature to clarify its epidemiological, clinical and pathological aspects.

Intracranial medulloepithelioma. A case report and review of the literature.

Medulloepithelioma is a rare primitive neuroectodermal tumour of the central nervous system usually developing in childhood, displaying highly malignant behaviour, with early progression or recurrence. The Authors report a new case of intracranial medulloepithelioma removed from the right cerebellar hemisphere of a 13-year-old boy who showed unspecific signs of intracranial hypertension. Based on a review of the thirty seven cases reported in the literature, they analyse the clinical, histological, immunohistochemical, ultrastructural and therapeutic features of this rare nervous system tumour.

[Benign sacrococcygeal teratoma in an adult]. / Tératome sacrococcygien bénin de l'adulte.

Sacrococcygeal teratomas are rare congenital tumors generally discovered at birth. Exceptionally these tumors are observed in adults. Radical resection must be performed to avoid potentially malignant recurrence even if the primary lesion was benign. We report a case of a benign sacroccygeal teratoma diagnosed in a 25-year-old male and discuss clinical features, pathological considerations and treatment.

[Subcutaneous phaeomycotic cyst of the ankle]. / Kyste phaeomycotique sous-cutané de la cheville.

Phaeohyphomycosis is a rare mycotic opportunist disease witch usually affects immunodepressed patients. Infection follows a traumatic inoculation of fungi into the skin with formation of a subcutaneous cyst. We report the case of a 20-year-old woman presenting with a subcutaneous, painless, and recurrent cystic lesion on the right ankle for 9 years. A surgical excision was performed. Histopathologic examination showed a granuloma with central abscess surrounded by a thick fibrous tissue. After staining with PAS, septate hyphae and spores were found in the granuloma, consistent with a phaeomycotic cyst.

[Hydatid cyst of the posterior fossa]. / Kyste hydatique de la fosse postérieure.

Hydatidosis is an endemic affection in Tunisia. Cerebral echinococcosis is a relatively rare entity accounting for only 1-2% of all hydatid cysts in humans. Extradural hydatid cyst of the posterior fossa is a very uncommon site for the disease. We report the case of a four-year-old child admitted for high intracranial pressure. Brain CT scan showed an extradural posterior fossa cyst without enhancement after contrast medium injection. Operative finding revealed a hydatid cyst. The histological examination of the tissue sample confirmed the diagnosis. The patient was given albendazole post operatively. She feels well six months later.

[Chordoid meningioma. Two cases]. / Le méningiome chordoïde. A propos de deux cas.

Chordoid meningioma is an uncommon histopathological variant of meningioma frequently confused with chordoma. Recurrence is not uncommon. The aim of our study is to present the clinical and pathological characteristics of this entity and a review of the literature. We report two cases of chordoid meningioma occurring in two women aged 47 and 63 years. Both women presented neurological signs without systemic disease. Radiological findings were temporoparietal and temporofrontal lesions with intense enhancement after contrast injection. Complete surgical resection was performed. Histopathologic findings were consistent with chordoid meningioma.

[Costal chondromyxoid fibroma]. / Fibrome chondromyxoïde costal.

Chondromyxoid fibroma is a rare benign bone tumor observed in less than 1% of all bone tumors. Pathological diagnosis remains difficult. All bone sites may be involved with a predominance of the metaphysis of long tubular bones, particularly the proximal tibia. The tumor has been rarely reported in ribs. We present of case of chondromyxoid fibroma observed in this unusual location in a 31-year-old woman. Routine chest x-ray and computed tomography revealed an expansive destructive mass with a lobulated contour which arose in the fourth left rib. Local resection was performed and the histological examination showed benign chondromyxoid fibroma. The clinical outcome was favorable without recurrence after 15 months follow-up.

[Pulmonary mucormycosis. Two cases]. / Mucormycose pulmonaire. A propos de deux cas.

Mucormycosis is a rare, devastating, opportunistic fungal infection, which occurs principally in some particular conditions, specially in non-controlled diabetic patients, notably during keratoacidosis. We report two cases in 62 and 72 year-old diabetic women. In both cases, histologic examination of endobronchial biopsies showed tissue invasion by hyphae with characteristic morphology leading to the diagnosis of pulmonary zygomycosis. This difficult diagnosis must be evoked in diabetic patients with non-responsive lung infections in order to apply early aggressive therapy.

[Clear cell tumor of the lung. A case report with review of the literature]. / La tumeur à cellules claires du poumon. A propos d'un cas avec revue de la littérature.

Clear-cell tumor of the lung is a rare entity of unknown etiology and histogenesis. This neoplasm typically presents as an asymptomatic, peripheral, sharply rounded mass in the lung, and histologically composed of large cells with a clear cytoplasm rich in glycogen, blended with an abundant network of sinusoid-type vessels. Immunohistochemical and ultrastructural procedures lead to diagnosis. We describe a primary pulmonary clear cell "sugar" tumor observed in a 28-year-old woman, and give a review of the literature. Clinical aspects, differential diagnosis, therapy and histogenetic aspects are discussed.

[Mucoepidermoid carcinoma of the lung. A series of 10 cases]. / Le carcinome mucoépidermoïde du poumon. A propos de 10 cas.

Mucoepidermoid carcinoma of the lung is rare. The microscopic findings distinguish low grade and high grade tumors. Conservative surgical resection is appropriate if possible for low grade tumors. For the high grade tumors, combined wide surgical resection and radiotherapy is recommended. We report 10 cases of mucoepidermoid carcinoma of the lung (5 low grade, 5 high grade) in 8 male and 2 female patients with a mean age of 43.9 years. Only 5 of 10 were smokers. All 10 patients underwent surgery. Operative procedures included 8 lobectomies and 2 pneumonectomies. Two patients received radiation therapy postoperatively. Three patients (2 high grade and 1 low grade) died. The other 7 patients were alive without evidence of recurrence.