Mucoepidermoid bronchial carcinoma. Review of the literature apropos of a new case]

Muco-epidermoid bronchial tumours are rare and characterized by the coexistence of epidermoid, mucus-secreting and intermediate cells. The authors report the case of a 22-year old unmarried woman hospitalized for exploration of a febrile dyspnoea related to a right superior lobar atelectasis. Endoscopy showed a smooth, pediculate tumour arising from the right superior lobar bronchus where bronchial biopsy was negative. Right superior lobectomy was performed through thoracotomy and established the diagnosis of muco-epidermoid bronchial tumour. Surgery was followed by radiotherapy of the chest. Over a 5-year follow-up period there was no local or extrathoracic recurrence, and the patient is in good condition.     

Right pulmonary artery-left atrial fistula. Review of the literature apropos of a case]

This is a rare congenital abnormality with only 17 other reported cases in the world literature. The most frequent sign is cyanosis with peripheral arterial desaturation. Angiography with selective injection in the main pulmonary shows early opacification of the left atrium, so confirming the diagnosis. Occasionally, a catheter can be passed through the fistula. Without surgical treatment there is a danger of systemic arterial embolism and two fatal cases were found in our review of the literature. The fistulous connection is often aneurysmal. It arises either from the right main pulmonary artery or from its branch to the inferior lobe. Associated vascular and pulmonary malformations are not rate. Surgical closure of the fistual is curative with immediate regression of the cyanosis and suppression of possible embolic complications.     

Pinkus' lymphoma. Review of the literature apropos of a case

We report one case of T cell type lymphoma with multilobated nuclei, a distinct variant described by G. Pinkus. The soft palate and subcutaneous tissues were the successive localisations. Despite an aggressive chemotherapy with autologous bone marrow transplantation, a fatal course happened in 18 months. This entity is different from cutaneous lymphomas, mantle zone lymphomas of Waldron, Japanese T lymphomas and from the cases described recently by Weisenburger. The link between these different anatomo-clinical and histological varieties is their belonging to the T cell line.     

Traumatic tricuspid insufficiency. Review of the literature, apropos of a case

The authors report a case of traumatic tricuspid incompetence due to rupture of an anterior pillar and the chordae, associated with pericardial rupture, a first degree atrioventricular block, a persistent complete right bundle branch block and acute recurrent pericarditis. The diagnosis, suggested by echocardiography was confirmed by right cardiac catheterisation. The patient underwent tricuspid plasty six months after the accident. On the basis of a review of the literature, the authors discuss traumatic tricuspid incompetence (its symptoms and associated lesions), the diagnostic methods and the therapeutic approach.     

Multiple proximal coronaro-pulmonary fistulae. Review of the literature apropos of a new case

The authors report a new case of multiple proximal coronaro-pulmonary fistula between right coronary arteries, anterior interventricular artery and the trunk of the pulmonary artery, in a 64 year-old female patient with chest pain and a continuous murmur located in the third left intercostal space. The coronary steal is demonstrated by a myocardial scintigraphy during stress with return to normal after surgical ligation. A review of the literature enabled to find 33 cases of this major congenital anomaly of the coronary arteries, defined as an abnormal communication between at least two main coronary vessels and the trunk of the pulmonary artery. This results in a left-right shunt, usually minor without any repercussions on the right cavities and pulmonary pressures. The entire clinical, electrocardiographic, radiological, sonographic, scintigraphic, haemodynamic and angiographic picture is reported for these 33 cases. A physiopathological discussion is proposed. The course of this disease is usually favorable (only one case of myocardial infarction was published, without cardiac failure. Osler's endocarditis or sudden death); this seems to authorize simple monitoring as a logical therapeutic approach except when a myocardial ischemia secondary to coronary steal is demonstrated, imposing a surgical correction.     

Systemic scleroderma and malignant diseases. A review of the literature]

Whether or not the association of systemic scleroderma and carcinoma is fortuitous is a much debated subject. Since 1886, approximately 320 cases of this association have been published. The most frequent malignancy involved is lung cancer (102 cases), followed by breast cancer (52 cases), malignant blood diseases (46 cases), cancer of the oesophagus (20 cases) and other gynaecological or gastrointestinal malignant tumours. A recent epidemiological study has shown that lung cancer is significantly more frequent than other malignancies, but statistical data on the latter are lacking. However, it would be wise to recommend that patients with systemic scleroderma should be regularly examined for gynaecological, haematological and gastrointestinal malignant diseases.     

Gastric tuberculosis. Review apropos of a case

Abdominal tuberculosis (TB) is infrequent in the Western world. This disease occurs more commonly among at-risk populations, mainly among older patients and patients with HIV infection. Abdominal TB usually manifests as intestinal TB, peritoneal TB, and mediastinal lymphadenitis. Gastric TB is a rare manifestation of abdominal TB. We present the case of an 80-year-old man, who had been diagnosed with anemia 2 years previously without establishing the etiology. Treatment consisted of oral iron administration without improvement. Symptoms included epigastralgia, nausea and vomiting, as well as asthenia, anorexia and weight loss (approximately 20 kg in 2 years). A computed tomography scan showed mediastinal and mesenteric adenopathy, ascites, splenomegaly, and thickening of the gastric wall. Diagnosis was made by endoscopic biopsy of the affected areas in the antral region, the result being granulomatous chronic gastritis suggestive of tubercular origin.     

Thymoma and hypogammaglobulinemia. Good's syndrome: apropos of a case and review of the literature]

INTRODUCTION: Good's syndrome is defined as the association of thymoma, hypogammaglobulinemia, and repetitive bronchopulmonary infections. We report one case and review the clinical and immunological features of this syndrome. EXEGESIS: We describe the case of a 67-year-old man who presented with both severe bronchopulmonary infection resistant to antibiotherapy and thymoma. Definite diagnosis was based on the existence of hypogammaglobulinemia. Treatment with intravenous immunoglobulins led to positive outcome. CONCLUSION: Good's syndrome occurs in only 5% of parathymic diseases. To prevent further bronchial tube destruction and pulmonary surgery, it must be diagnosed and treated early.     

Familial acromegaly. Apropos of a case. Review of the literature]

Two brothers and a sister with acromegaly--one of them with acromegalogigantism--are described. They all presented pituitary adenomas, and the diagnosis was made between the ages of 17 and 29. There was no feature of multiple endocrine neoplasia. The familial investigation consisted of detailed interrogation, basal hormonal evaluation and HLA determination (haplotypes A and B) in 14 members, including the patients themselves. Despite basal plasma GH levels ranging from 8.00 to 10.00 ng/ml in 3 other family members, the normality of both GH response to induced-hyperglycemia and CT-imaging of the pituitary gland excluded the diagnosis of acromegaly in these subjects. No correlation with the HLA haplotypes was observed. Differential diagnosis with familial multiple endocrine neoplasia (MEN type 1) is discussed. Previous reports of familial acromegaly are reviewed and analysed. It appears that familial acromegaly may be considered as a specific entity for two main reasons: 1) owing to the low incidence of acromegaly in the general population, familial cases are unlikely to be fortuitous. 2) most of the reported cases share some common clinical features, such as a male predominance, a high incidence of acromegalogigantism, and the presence of a pituitary macroadenoma. However, the primitive disorder and the genetic transmission of the disease are still unknown.     

Systemic mastocytosis: a case report. Cytological, cytochemical and ultrastructural considerations.

A case of systemic mastocytosis with unusual clinical manifestations, appearing as an isolated splenohepatomegaly, is described. The proliferative character is evident from the cytological characteristics of immaturity and the presence of a moderate percentage of mast cells in the peripheral blood. These cells make up 40% of the total cells in the bone marrow. Special attention is given to the optical, morphological, cytochemical, and ultrastructural studies of the disease. Some anomalies were found at the subcellular level which apparetnly have not been recorded until present. Various dyshematopoietic features of this case are reported, which may be considered as manifestations of a paraneoplastic syndrome.     

Cyclophosphamide-induced interstitial pneumopathy. Course data of bronchoalveolar lavage apropos of a case and review of the literature]

A patient treated with cyclophosphamide for breast cancer developed functional and clinicoradiological signs of sub-acute diffuse interstitial pneumopathy. Bronchoalveolar lavage revealed lymphocyte alveolitis. Differential diagnoses were excluded and the course was favorable after cyclophosphamide withdrawal. The bronchoalveolar lavage results obtained initially and at follow-up and two previous lavages reported in the literature demonstrate the importance of this examination in the diagnosis of drug-induced pneumopathy.     

Systemic mastocytosis with acute myelomonocytic leukemia: a case report

Bone marrow mastocytosis may be associated with many clonal non mast cell hematological neoplasms and its association with acute myeloid leukemia especially with t (8; 21) has been described. We describe an interesting case of coexistence of systemic mastocytosis with acute myelomonocytic leukemia in a young child. Diagnosis of acute myelomonocytic leukemia was based on bone marrow aspirate findings coupled with cytochemistry. Systemic mastocytosis was diagnosed on the basis of bone marrow biopsy findings showing blasts as well as multifocal dense mast cell infiltrates with spindling and atypical morphology which was confirmed on toluidine blue staining.     

Pleuropneumopathy caused by bromocriptine in a patient with Parkinson disease. Review of the literature apropos of a new case

Bromocriptine, a derivate of ergot of rye, is employed in high doses for the treatment of Parkinson's disease, and may induct pleuropulmonary affections. We report the case of a 75-years-old patient, on bromocriptin for 5 years, who presented a progressive dyspnea, due to a pleural thickening, and moderate interstitial infiltrate, associated with an inflammatory syndrome. The outcome was favorable with 3 months of bromocriptin cessation. These complications are rarely described, probably underestimated, and justify a respiratory follow-up for these patients, who are difficult to stabilize in their neurologic treatment.     

Cutaneous plasmacytoma: review of the literature apropos of a case

Extramedullary plasmocytomas are ubiquitous plasmocytic tumours which are principally located in bones and mucosae but rarely found on the skin. Clinically, they present as purplish-blue cutaneous nodules or, less frequently, as papular or urticarial eruptions. The diagnosis rests on histology and immunostaining. Cutaneous plasmocytomas are usually divided into two types: (1) primary plamocytomas which occur in the absence of myeloma, present as solitary or multiple skin tumours, accompanied or not by monoclonal gammopathy, and have an imperfectly known course and prognosis; (2) secondary plasmocytomas--a case of which is reported here--which appear in the course of a large tumoral mass myeloma and have a very poor prognosis.