Alia Zehani

Carcinomes sarcomatoïdes du poumon : étude rétrospective de 28 cas

INTRODUCTION Sarcomatoid carcinoma (SC) of the lung is defined by the World Health Organization as poorly differentiated non-small cell carcinoma that contains a component of sarcoma or sarcoma-like elements. It represents an overall continuum of epithelial and mesenchymal differentiation. Five subtypes are recognized: pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma, and pulmonary blastoma. The diagnosis is pathological and requires a good sampling of the tumor. PATIENTS AND METHODS Twenty-eight cases of primary sarcomatoid carcinoma, diagnosed between 1993 and 2010, were reviewed retrospectively, noting the clinicopathological characteristics. RESULTS The patient population consisted of 25 males and 3 females with mean age of 62.9 years (48-75 years). The symptomatology was dominated by respiratory symptoms. Imaging features showed a pulmonary mass invading pleura or thoracic wall in 5 cases. The diagnosis was made in all cases on histological examination. These 28 tumors were divided as below: into 19 pleomorphic carcinomas, 4 giant cell carcinomas, 1 spindle cell carcinoma and 4 carcinosarcomas. Twenty-seven tumors were treated surgically. Associated treatments were neoadjuvant (3 cases) or adjuvant chemotherapy (1 case) and preoperative radiotherapy (5 cases). Deaths occurred in 7 patients. Twenty-two patients were lost to follow up. CONCLUSION These tumors are frequently symptomatic, are locally advanced, and have higher rates of recurrence. Its prognosis is worse than that of other non-small cell lung cancer.

Les sarcomes primitifs du médiastin

INTRODUCTION Primary sarcomas of the mediastinum are rare and account for 2 to 8% of malignant mediastinal tumours. The aim of this study is to describe their clinical and pathological characteristics. PATIENTS AND METHODS Fifteen cases of primary mediastinal sarcoma, diagnosed between 1993 and 2009, were reviewed retrospectively, noting the clinical, radiological and pathological findings, and the treatment given. RESULTS The patient population consisted of eight females and seven males with mean age of 40 years (14 to 73 years). The symptomatology was predominately respiratory. Imaging showed a mediastinal mass invading adjacent organs in nine cases. The diagnosis was made in all cases by histological examination. These 15 mediastinal sarcoma comprised 12 malignant peripheral nerve tumours, two liposarcomas and one angiosarcoma. Three were grade III, six grade II and six grade I. Ten were treated surgically, of which seven had radical resections. Associated treatments were neoadjuvant (one case) or adjuvant (one case) chemotherapy and postoperative radiotherapy (five cases). Radiotherapy was undertaken alone in three inoperable tumours. Eight patients (53%) had died. CONCLUSION Management of primary mediastinal sarcoma needs a multidisciplinary approach, and is based mainly on radical resection. The prognosis is poor and depends mainly on surgical excision and histological grade.

Article originalLes sarcomes primitifs du médiastinPrimary mediastinal sarcomas

INTRODUCTION Primary sarcomas of the mediastinum are rare and account for 2 to 8% of malignant mediastinal tumours. The aim of this study is to describe their clinical and pathological characteristics. PATIENTS AND METHODS Fifteen cases of primary mediastinal sarcoma, diagnosed between 1993 and 2009, were reviewed retrospectively, noting the clinical, radiological and pathological findings, and the treatment given. RESULTS The patient population consisted of eight females and seven males with mean age of 40 years (14 to 73 years). The symptomatology was predominately respiratory. Imaging showed a mediastinal mass invading adjacent organs in nine cases. The diagnosis was made in all cases by histological examination. These 15 mediastinal sarcoma comprised 12 malignant peripheral nerve tumours, two liposarcomas and one angiosarcoma. Three were grade III, six grade II and six grade I. Ten were treated surgically, of which seven had radical resections. Associated treatments were neoadjuvant (one case) or adjuvant (one case) chemotherapy and postoperative radiotherapy (five cases). Radiotherapy was undertaken alone in three inoperable tumours. Eight patients (53%) had died. CONCLUSION Management of primary mediastinal sarcoma needs a multidisciplinary approach, and is based mainly on radical resection. The prognosis is poor and depends mainly on surgical excision and histological grade.

A case of adult anaplastic cerebellar ganglioglioma

Background: Anaplastic posterior fossa ganglioglioma in adults is exceedingly rare. To date, only one case of adult anaplastic posterior fossa ganglioglioma has been reported in the English literature and none has been described at the cerebellum. To our knowledge, this report is the third case of malignant posterior fossa ganglioglioma in adults and the first at the cerebellum. In general, this entity can be misdiagnosed preoperatively as a primary posterior fossa neoplasm, and by reporting our clinical and radiographic observations we want to add to the existing literature on this rare entity. Case Description: A 40-year-old man presented with a history of headaches and dizziness and progressive gait disturbance and was diagnosed with anaplastic ganglioglioma in the posterior fossa. Conclusions: Although rare, our case demonstrates that anaplastic ganglioglioma should be considered in the differential diagnosis of infratentorial tumors in adult patients.

Hodgkin Lymphoma revealed by epidural spinal cord compression

Context: Hodgkin Lymphoma is rarely diagnosed as spinal cord compression syndrome. Caused by an epidural mass, this complication is often encountered in a late stage of the disease. We report the case of a 40-year-old man presenting with symptoms of low thoracic spinal cord compression due to an epidural tumor on the MRI. Findings: Emergent surgery was undertaken on this patient, consisting in laminectomy and tumor resection. After surgery, pain relief and mild neurological improvement were noticed. The histological study revealed a Hodgkin Lymphoma and the patient was referred to chemotherapy and radiotherapy. Conclusion: Though chemotherapy is the gold standard treatment for Hodgkin Lymphoma, surgical spinal decompression may be required in epidural involvement of the disease. Diagnosis may be suspected in the presence of lymphadenopathy and general health decay.

Masse kystique du rein

Il s’agissait d’une femme âgée de 23 ans, sans antécédents pathologiques, qui consultait pour des douleurs lombaires droites évoluant depuis 6 mois. Le scanner abdomino-pelvien objectivait une masse rénale mesurant 15 cm de grand axe, kystique, cloisonnée avec une portion charnue, rehaussée après injection de produit de contraste. Cette masse prenait naissance au niveau du pôle supérieur du rein droit avec un développement exo-rénal (Fig. 1). Cette tumeur était classée de type IV selon la classification de Bosniak. Une néphrectomie élargie droite était réalisée. À l’examen macroscopique, le rein était le siège d’une tumeur bien limitée, mesurant 15 cm de grand axe, de siège polaire supérieur, faisant protrusion dans le bassinet. Elle était mi-solide mi-kystique, indurée et de couleur blanc grisâtre. À l’examen microscopique, cette néoformation correspondait à une prolifération tumorale à double composante épithéliale et mésenchymateuse (Fig. 2). Le contingent épithélial était fait de structures tubulo-papillaires et de kystes de taille variable (Fig. 3a). Les cellules tumorales étaient cubo-cylindriques, éosinophiles, munies de noyaux réguliers, non mitotiques. Le deuxième contingent mésenchymateux était de densité variable, souvent hypercellulaire, parfois de type stroma ovarien (Fig. 3b). Il était constitué de cellules fusiformes dotées de noyaux réguliers et dépourvus de figures de mitose. En immunohistochimie, le contingent épithélial exprimait la cytokératine et l’EMA (Fig. 4a). La composante mésenchymateuse exprimait de façon intense et diffuse la vimentine, l’actine et la desmine et focalement le CD10 (Fig. 4b). Les récepteurs hormonaux n’étaient pas exprimés. Les suites opératoires étaient simples.