[Primary pulmonary leiomyosarcoma revealed by spontaneous pneumothorax]. / Léiomyosarcome pulmonaire primitif révélé par un pneumothorax spontané

We report the case of a 57-year-old patient admitted for dyspnea and dry cough. Thoracic radiograph showed a right pneumothorax and right paracardiac opacity. Thoracic drainage was carried out allowing the return of the lung at the wall. Chest CT-scan revealed right upper mediastinopulmonary mass taking contrast material associated with a nodular thickening of the pleura. CT-guided biopsy of the mass and the pleural nodules concluded to pulmonary leiomyosarcoma. Death occurred one month after diagnosis confirmation. Pneumothorax is a usual mode of revealing sarcoma's pulmonary metastases; however, it is exceptionally associated with primitive pulmonary sarcomas. Our case is the second published case, to our knowledge, of primitive pulmonary leiomyosarcoma presenting with pneumothorax.

[Sacral chordoma: a rare tumor of the buttocks]. / Chordome sacré : une tumeur fessière rare.

INTRODUCTION: Described and recognized for the first time as a pathological entity by Virchow in 1857, chordoma is a tumour of embryonic origin secondary to an attack of the notochord. In most cases it is asymptomatic, resulting in fairly late diagnosis. PATIENTS AND METHODS: We report the case of a 62-year-old patient presenting a bulky tumefaction, nodular in places, not very painful, and extending towards the anal area, scrotum and the posterior aspect of the upper left thigh. Histopathological examination of a macrobiopsy sample of this tumefaction pointed to chordoma. On magnetic resonance imaging (MRI), the tumour presented multiple ramifications extending towards the scrotal area, the sciatic area and the posterior aspect of the left thigh. Palliative tumorectomy was performed. Given the very slow progression of the tumour and the risk of adverse effects in such a large tumoral exposure field, radiotherapy was ruled out. DISCUSSION: This is a typical observation of a rare tumour that dermatologists may encounter.

[Choroid plexus carcinoma: a case report]. / Carcinome du plexus choroïde : à propos d'un cas.

OBJECTIVE: Choroid plexus carcinomas are rare and occur more frequently in children than in adults. The differential diagnosis includes choroid plexus papilloma and papillary ependymoma. The prognosis is generally poor. The objective of this paper is to discuss the epidemiological characteristics, diagnosis, treatment, and prognosis of this rare childhood tumor. CASE REPORT: A three-year-old child was hospitalized with a history of partial epilepsy and intracranial hypertension. A computed tomography scan showed an intracranial mass measuring 11cm in diameter, extending to the ventricle system with heterogeneous contrast enhancement. He was urgently operated for loss of consciousness. During the operation, the patient developed an abundant hemorrhage and died. The histopathological examination of the excised tumor identified a choroid plexus carcinoma. CONCLUSION: Choroid plexus carcinomas are rare and treatment is currently controversial. Radical excision is quite difficult and associated with excessive blood loss.

[Pure colloid carcinoma of the breast: anatomoclinical study of seven cases]. / Carcinome colloïde pur du sein : étude anatomoclinique de sept cas.

PURPOSE: Breast mucinous carcinoma is a particular histological form characterized by the extracellular production of mucus. The pure form is rarely reported and its prognostic is better than the other types of the breast cancer. The aim of the present study was to discuss the various anatomoclinical, therapeutic aspects and the prognostic factors of the pure mucinous carcinoma of the breast. PATIENTS AND METHODS: We report seven cases diagnosed over a period of 11 years (1993-2003) in the laboratory of anatomy and pathological cytology of the university hospital of Sfax. A review of the clinical files with immunohistochemistry study (hormonal receptor, synaptophysine, chromogranine, Bcl2, Ki67, P53 and Her-2/neu (C-erbB-2)) were carried out for all the cases. RESULTS: pure colloid carcinoma of the breast accounted for 0,5% of the whole of the breast cancers. The average age of patients was 69 years. At the time of the diagnosis, four tumours were classified T4, one T3, two T2, two N1 and one N3; no patient had presented metastasis (M0) according to pTNM classification. The echomammography showed regular contours mass in four cases. The anatomopathological study showed that the neuroendocrine differentiation was found in two cases, all the tumours had presented positivity for the hormonal receptors (oestrogen and progesterone) and negativity for the other antibodies, two cases exhibited the Bcl2 positivity and negativity for the other antibodies. An adjuvant radiotherapy was carried out for all the patients. The average duration of follow-up was of 33 months; the evolution was marked by the occurrence of metastases in two cases. CONCLUSION: The pure colloid carcinoma of the breast constitutes a particular morphological entity which deserves to be individualized; neuroendocrine differentiation of these tumours must be required. Their forecast is relatively favourable.

[Esophageal leiomyomatosis revealing an Alport syndrome]. / Léiomyomatose oesophagienne révélatrice d'un syndrome d'Alport.

Alport syndrome is a rare progressive hematuric nephropathy associated with sensorineural deafness. Leiomyomatosis associated with Alport syndrome is quite rare. We report a particular case of Alport syndrome which was diagnosed in the setting of an oesophageal leiomyomatosis. Alport syndrome and leiomyomatosis are caused by mutation of the genes encoding for the alpha chain of type IV collagen. In view of the important clinical and genetic implications, renal function and urinary status should be controlled in any patient with oesophageal leiomyomatosis.

[Polymorphous low-grade adenocarcinoma: a palatine and a labial location]. / Adénocarcinome polymorphe de bas grade: deux localisations palatine et labiale.

INTRODUCTION: Polymorphous low-grade adenocarcinoma (PLGA) is a variant of malignant tumor of minor salivary glands. We had for aim to discuss morphology, evolution and differential diagnosis of this rare tumor. CASE REPORTS: The first case was a 65-year-old woman admitted for a two-month history of a right submaxillary swelling. The examination revealed a tumor of the right side of the palate. Biopsy was positive for a pleomorphic adenoma. The mass was enlarged and surgical resection was performed. The diagnosis was a partially resected PLGA. The patient also received adjuvant radiotherapy. There was a local recurrence 28 months after complete treatment. The second case was a 57-year-old woman who consulted for a 12-year history of lower lip swelling. The examination revealed a painless 2cm long located nodule in the mucosal side of the lower lip. An excisional biopsy was performed. The pathological examination concluded to a completely resected PLGA. The patient was free of disease at 54 months follow-up. DISCUSSION: The morphologic diversity and cytological uniformity of PLGA may make the differential diagnostic difficult especially with adenoid cystic carcinoma and pleomorphic adenoma. Its aggressiveness is proved by a local infiltrative growth pattern requiring a large surgical excision.

[Breast metastasis: anatomoclinical study of six cases]. / Les métastases mammaires: étude anatomoclinique de six cas.

INTRODUCTION: Breast metastases are rare. They represent 0.4 to 6% of all breast cancers. Our aim is to discuss the means of diagnosis and the clinicopathological features. PATIENTS AND METHODS: We report a retrospective survey of six cases of breast metastases diagnosed over a period of 11 years (1992-2003) in the laboratory of anatomy and pathological cytology of the university hospital of Sfax. The diagnosis was carried on a material of cytoponction in two cases, a biopsy in three cases, a surgery specimen in one case. Immunohistochemical study was performed in four cases. Clinical, therapeutic and evolutionary data were collected from the files of patients. RESULTS: Metastases to the breast constituted 0.43% of all breast cancers. The primary tumors understood a case of gingival-maxillary non-Hodgkin's lymphoma, a case of retroauricular melanoma, a case of soft tissue leiomyosarcoma, a case of uterine choriocarcinoma, a case of rectal neuroendocrine carcinoma and a case of gastric signet cell carcinoma. All patients were women, the middle age was 45.5 years. In three cases the metastases to the breast was concomitant to the diagnosis of the primitive tumour. Clinically it was a nodule in five cases, the size average was 2.3cm, and a subareolar thickening in one case. The mammary involvement was bilateral in two cases. The middle survival after the diagnosis was eight months. CONCLUSION: Metastases to the breast must be distinguished from primary breast cancers whose treatment and outcome are different. A confrontation of clinical and pathological data is recommended for an accurate diagnosis. Immunohistochemical study is of great interest particularly when the breast tumour is revealing the disease.

Glomus tumour of uncertain malignant potential.

A 71-year-old man presented with a several year story of a slowly enlarging, painful mass of the knee. Surgical findings showed a subcutaneous, well-circumscribed mass fixed to the patella, and a wide resection was performed. Macroscopically, the tumour measured 6 x 5 x 5 cm and was well circumscribed. The tumour was characterised by superficial location, large size and focal marked atypia. In addition, areas of benign glomus tumour (GT) were identified. The findings appear to be consistent with the diagnosis of GT of uncertain malignant potential.

[Metastatic signet ring cell carcinoma to the breast from stomach]. / Métastase mammaire d'un adénocarcinome à cellules en bague à chaton gastrique.

Metastatic tumors in the breast are quite rare and constitute 0.5 to 6% of all breast malignancies. They often occur in a polymetastatic context. The most frequent primitive tumors are lymphoma, leukaemia and malignant melanoma. The gastric origin is seldom reported. We report here the observation of a 40-years woman operated in urgency for an acute abdominal syndrome. A gastric tumor was discovered intraoperatively with ovarian metastasis and peritoneal carcinosis. The pathological examination revealed a gastric signet ring cell carcinoma with an infiltration of the right ovary. Four months later, the patient presented with a lump of the right breast. The histologic examination corresponded to a mammary metastasis by a signet ring cell carcinoma from stomach. The objective of our work is to discuss through this observation the anatomoclinical and evolutionary characteristics of breast metastasis.