Cutaneous metastasis of lung carcinoma: a retrospective study of 12 cases.

BACKGROUND: The skin is a rare site of metastasis of the internal malignancies. On the contrary, lung cancer is a common primary malignancy of skin metastasis. The latter is either synchronous with the primary lung tumour or can reveal it. OBJECTIVE: To analyse the clinical findings and the outcome of cutaneous metastasis in patients with primary lung cancer. METHODS: Retrospective study including 12 cases of cutaneous metastasis of lung cancer diagnosed in our institution during 6years. RESULTS: Our patients included three women and nine men with a median age of 59.83years. The cutaneous metastases were unique in seven cases. They revealed the primary tumour in two cases, were discovered synchronously with the primary tumour in six cases and after the primary tumour in four cases. They appeared in patients presenting multiple metastases in 10 cases. Clinical appearance consisted on a firm nodule, sometimes ulcerated with a diameter ranging from 0.5 to 5.5cm. The tumour was sited in the thorax, the back, the abdomen; or the upper or lower limbs. Pathological examination of bronchial biopsy revealed an adenocarcinoma in eight cases, a squamous cell carcinoma in two cases and a small cell carcinoma in two cases. In spite of lung cancer therapies, the patients with metastasis to other organs with a median follow-up time of 4months died. CONCLUSION: Cutaneous metastases of lung cancer are of poor prognosis. They are most of the time associated with an advanced neoplastic process with metastasis to other organs. Median survival after skin involvement does not exceed months.

Cardiac cavernous haemangioma.

BACKGROUND: Cardiac haemangioma was first described in 1893. It is an infrequent and benign neoplasm which accounts for about 2.8% of all benign primary cardiac tumours, arising from the cardiac ventricles, valves, atria and rarely the epicardium. METHODS AND RESULTS: We report the case of a 24-year-old woman with a cardiac haemangioma detected by transthoracic echocardiography, computed tomography, and magnetic resonance imaging. The coronary CT showed a mass located in the pericardial cavity with close relationship to the myocardium. The mass surrounded completely the segments 2 and 3 of the left anterior descending coronary artery. At operation, the tumour was incompletely resected, leaving a remnant in the left anterior descending coronary artery contact, one diagonal branch has been sacrificed. Pathologic study diagnosed a cavernous haemangioma. Transthoracic echocardiography eight months later showed a remnant of haemangioma with a left ventricular ejection fraction estimated to 69%. The coronary CT 10 months after surgery showed the remnant of the tumour with normal cardiac cavities and absence of visualisation of the diagonal artery. The patient is currently asymptomatic and doing well 14 months after surgery. CONCLUSIONS: Cardiac haemangioma is a rare cardiac tumour with an unknown aetiology. The diagnosis is aided by imaging techniques. The coronary CT may be useful when more precise evaluation of the tumour extent is required. It allows specifying the relationship of the mass with the coronary vessels. Successful treatment usually requires timely surgery. However, periodic examinations and echocardiography are recommended.

[Diagnostic and therapeutic management of operable bronchopulmonary carcinoid tumours]. / Prise en charge diagnostique et thérapeutique des tumeurs carcinoïdes broncho-pulmonaires opérables.

INTRODUCTION: Bronchial carcinoid tumours (CT), divided into typical carcinoid (TC) or atypical carcinoid (AC), are rare tumours whose therapeutic management remains unspecified. METHODS: Retrospective study collecting cases of bronchial CT operated at the thoracic surgery department of Abderrahmane-Mami hospital of Ariana and recruited from the pneumology departments of Northern Tunisia, during a 12-year period. RESULTS: Ninety patients were collected (74 cases of TC and 16 cases of AC). The mean age was 45 years and the sex ratio H/F=0.5. The chest X-ray was normal in 11 cases, as well as flexible bronchoscopy in seven cases. The tumour was classified: stage IA (10 cases), IIA (28 cases), IIB (31 cases), IIIA (15 cases) and IIIB (six cases). Surgery resulted in a complete resection in 78 patients, an extensive resection in six patients, and a conservative resection in six patients. Adjuvant chemotherapy was given in 10 patients. The survival was 84% at five years and 42% at 10 years. CONCLUSION: The prognosis of CT depends directly on the histological subtype. It is excellent for TC after complete resection, unlike ACs that are similar to well-differentiated bronchial carcinomas.

Multiple myeloma presenting with multiple thoracic manifestations.

Multiple myeloma is a malignant proliferation of plasma cells that affects mainly bone marrow but may also involve other organs as well. We report thoracic involvement in the form of left-sided pleural effusion, osseous lesions, bronchial infiltration, and mediastinal lymphadenopathy in a 61-year-old woman, non-smoker presented with chest pain, dyspnoea, cough and deterioration in general health over the preceding seven months. Immunoelectrophoresis and immunofixation showed raised kappa-light chain immunoglobulin G (IgG) in serum and pleural fluid. Bronchial and pleural biopsies documented myelomatous infiltration and bone marrow aspirate revealed extensive plasma cell infiltration. At eight months, following the fourth cycle of melphalan, endoxan and prednisone based chemotherapy, the patient died.

A rare case of squamous inclusion cyst in cervical lymph node.

A 38-year-old man, with no history of malignancy, was found to have a 2 cm jugular lymph node, for which a lymph node tuberculosis was suspected. The specimen revealed a cystic structure lined by mature keratinizing squamous epithelium with a prominent granular cell layer consistent with a squamous inclusion cyst in a lymph node, but a metastatic squamous cell carcinoma could not be excluded. This is the first case report of a rapidly enlarging squamous inclusion cyst in a jugular lymph node. Our case demonstrates the diagnostic challenges related to a squamous inclusion cyst in cervical lymph node and serves to inform the readers to consider this lesion in the differential diagnosis for similar situations.

[Primary thymic carcinomas. Three cases and a review of the literature]. / Carcinomes thymiques primitifs : à propos de trois cas avec revue de la littérature.

Thymic carcinoma is a very rare malignancy. In 1999, a World Health Organization committee published histologic criteria for distinct thymoma entities (labelled as type A, AB, B1, B2, B3 thymomas) and for the heterogeneous group of thymic carcinomas, collectively called type C tumour. Thymic carcinoma differs from thymoma in that it displays cytologically malignant features, extensive local invasion, and a substantial potential for metastasis. It constitutes a heterogeneous group of tumours that display different biological behaviours and prognoses. The majority of thymic carcinomas are either squamous carcinomas or lymphoepithelioma-like carcinomas. This study included three male patients aged 20, 46 and 19years respectively with histologically proven thymic carcinoma diagnosed at the author's institution. All of the patients presented a large mass of the anterior mediastinum. Histological examination of the different tumours revealed three distinct variants of thymic carcinoma, namely: epidermoid carcinoma, clear cell carcinoma and lymphoepithelioma-like carcinoma.

[Primary breast lymphoma: a case report]. / Lymphome primitif du sein: à propos d'un cas infraclinique.

BACKGROUND: Non-hodgkinian's lymphoma (NHL) represents 0.04 to 0.53% of all breast cancers. The clinical aspects and therapeutic models of the disease are a subject of debate. AIM: The purpose of this paper is to report the clinical, imaging (sonographic, mammographic and MRI) and pathological features of breast lymphoma, a rare but aggressive tumor, based on a case report review. CASE REPORT: We report a case of primary non-hodgkinian's lymphoma of the breast in one patient aged of 52 years. The patient went to a systematic screening of breast cancer. Physical examen was normal. Mammography showed breast with transitional density, BIRADS type 2, micro-calcifications behind the.... but sometimes it had linear...., neither not distorsion were identified. Ultrasonography did not show a tumor. The disease was revealed by breast MRI. The additional value of MRI in diagnosis is validated in our patient. In fact, when cancer is occult, size evaluation is difficult at standard imaging (7 mm). The diagnosis of non-hodgkinian's lymphoma was confirmed on histological examination of tumor biopsies. It was a B-cell non-Hodgkins-type lymphoma. The clinical features have been reviewed and the tumor have been evaluated both on a morphologic and an immunohistochemical basis. With chemotherapy, the course was favourable. CONCLUSION: The breast is an uncommon site of development of malignant lymphomas. Secondary disease is more common than the primitive form. Primary breast lymphoma is a difficult diagnosis because it is very rare. The diagnosis is mainly histological. Chemotherapy is the principal therapeutic mean. Treatment combines radiotherapy and chemotherapy. Prognosis is generally bad.

[Desmoid fibromatosis of the posterior mediastinum]. / Fibromatose de type desmoïde du médiastin postérieur.

Fibromatosis are uncommon connective tissue tumours arising from musculo-aponeurotic tissue and characterised by spindle cell fibroblastic and myofibroblastic proliferation. The exact aetiology is unknown but several factors are considered to be positively correlated with their development and growth (genetic and hormonal factors and trauma). Although they are considered histologically benign they behave aggressively locally and relapse repeatedly after surgical excision. Mediastinal localisation is very rare. We describe a case of de novo fibromatosis of the posterior mediastinum in a 61 year old man with no history of thoracotomy or trauma. Although mediastinal fibromatosis is very uncommon physicians should be aware of this disease in order to ensure appropriate surgical treatment.

[Congenital cystic adenomatoid malformation of the lung. Report of 3 cases with late presentation]. / Malformation adénomatoïde kystique congénitale du poumon: à propos de trois cas de révélation tardive.

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare congenital developmental abnormality, representing about 25% of all congenital lung lesions. In many cases, respiratory distress occurs during the neonatal period, and in about 80- 85% of patients, CCAM is diagnosed before the age of two years due to respiratory infection. It is very rare that presentation is delayed until adulthood. We report three cases of CCAM presenting in adults. The diagnosis was based on clinical and radiological findings in one case and two patients were not diagnosed until surgery. The lesion was present in the right lung in two and in left lung in one patient. All patients underwent surgical resection. The result of histopathological examination confirmed CCAM Stocker type 1, without malignancy. The post operative follow up showed an excellent recovery. Clinicians and pathologists need to be aware of the fact that CCAM can be present for the first time in adolescents or in adults. The clinical diagnosis is suggested by radiographic findings and is confirmed at pathology as surgery is generally indicated.

[Benign mature teratomas of the mediastinum]. / Tératomes matures bénins du médiastin.

INTRODUCTION: Mature teratomas of the mediastinum are rare. However, they represent the most common mediastinal germ cell tumours. The aim of this study is to describe their clinical and pathological characteristics. PATIENTS AND METHODS: Fourteen cases of mediastinal mature teratoma, diagnosed between January 1992 and December 2006, were reviewed retrospectively, noting the clinical, radiological, surgical, and pathological findings. RESULTS: The patient population consisted of 10 females and 4 males with mean age of 29 years (5-56 years). Chest pain was the main symptom. Imaging features comprised a heterogeneous anterior mediastinal mass containing soft-tissue, fluid, fat, or calcium attenuation, or any combination of the four. Macroscopically all the tumours were cystic, or predominantly cystic, containing mostly grumous material. The mean size was 9 cm (5-12 cm). Histological examination constantly revealed skin with or without appendages. Other components identified were bronchial mucosa (12 cases), fat (12 cases), muscle (10 cases), cartilage (8 cases), bone (7 cases), gastrointestinal mucosa (7 cases), pancreas (5 cases), urothelial epithelium (3 cases), nervous and prostatic tissues (one case each). All the teratomas were mature, and 7 of them were intrathymic. CONCLUSION: Every form of teratoma occurs in the mediastinum (mature, immature and with malignant transformation) but, in our study, they were exclusively mature. The histology is essentially similar to that of teratomas of gonads. However, pancreatic tissue occurs frequently in mediastinum (54% in literature, 25% in our study) but not in the gonads. On the other hand, thyroid follicles have not yet been seen in mediastinal teratomas.

[Fibrous dysplasia of the rib. Ten case reports]. / Dysplasie fibreuse costale. A propos de dix cas.

PURPOSE OF THE STUDY: Fibrous dyplasia is a rare sporadic disease accounting for 0.8% of primary bone tumors. This benign pseudotumor results from proliferation of fibrous tissue in bone and the production of immature bone tissue without an osteoblastic crown. The disease can involve one or more bones, ribs are rarely involved. We report a series of 10 cases of costal fibrous dysplasia. CASE REPORTS: This series of 10 cases was collected over a period of 10 years (1996-2005). There were five men and five women, mean age 38.4 years, range 27-52 years. One rib was involved in eight patients, two ribs in two. Pain was the most frequent symptom. Plain X-rays showed signs suggestive of fibrous dysplasia. To confirm the diagnosis, rib resection was performed in all ten patients. Pathology examination provided the diagnosis. The postoperative period was uneventful in all patients and all are recurrence free at mean 50 months follow-up. DISCUSSION: Management of fibrous dysplasia can be simple surveillance in the majority of patients. Nevertheless, in the event of a single focus, particularly in a rib, fibrous dysplasia can raise a difficult problem of differential diagnosis with malignant tumors. Surgical resection is therefore required in selected cases.

[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.

[Epithelioid hemangioendothelioma mimicking hypersensitivity pneumonitis]. / Hémangioendothéliome épithélioïde pulmonaire mimant une pneumopathie d'hypersensibilité

Epithelioid haemangioendothelioma is a rare vascular tumour of slow growth and unfavourable outcome. The diagnosis of the pulmonary localisation is difficult and can mimic by clinical and radiological features other diagnosis as hypersensitivity pneumonitis. We report the case of a 28-year-old man, farmer handling with palm tree pollens, admitted to the hospital for dry cough. Clinical and thoracic computed tomography findings revealed diffuse infiltrating pneumopathy; bronchoalveolar lavage results and professional exposure were suggestive for hypersensivity pneumonitis. Surgical lung biopsy with immunochemistry study concluded to pulmonary epithelioid haemangioendothelioma. Extra pulmonary localisation research was negative. No treatment was indicated. At three years, the patient is steel asymptomatic. Epithelioid haemangioendothelioma is a tumour of intermediate malignancy, of which pulmonary localisation has a nonspecific clinical presentation mimicking diffuse infiltrating pneumonitis. Diagnosis is essentially made by surgical lung biopsy with pathological and immunohistochemical study.

[Clinical and pathological profile of the pleural malignant mesothelioma: A retrospective study about 30 cases]. / Le profil anatomoclinique du mésothéliome pleural malin : une étude rétrospective à propos de 30 cas.

BACKGROUND: The malignant pleural mesothelioma (MPM) is a rare tumour usually associated to asbestos exposure. The delay between the exposure and the occurrence of the cancer can reach 40 years. This caused the pick of incidence described in many countries including Tunisia. The diagnosis is suspected based on clinical features but positive diagnosis is microscopic. Our aim was to describe the clinical and microscopic features of MPM through a single institution experience. PATIENTS AND METHODS: We conducted a retrospective study about 30 MPM diagnosed over a 20-year-period (1995-2015). We included only patients with complete records including clinical, radiologic and microscopic features. All the microscopic diagnoses were reviewed by 2 pathologists. A mean of 12 slides per case was reviewed. The diagnosis was based on the 2015 WHO classification. RESULTS: The mean age of the patients was 61 years, average 22 to 80 years. The sex ratio was 6,5. An asbetose exposition was reported in 21 cases. The most frequent symptoms was chest pain reported in 25 cases. Physical exam was normal in 9 cases. It revealed pleural syndorm in most patients (60 %). Imaging findings consisted mainly in diffuse pleural thickening in 17 cases. Twelve tumours were classified as stage I, 3 stage II, 14 stage III et 1 stage IV. Pleural biopsy was performed using needle in 18 cases, through thoracoscopy in 16 cases, thoracotomy in 3 cases and allowed the diagnosis in respectively 7 cases/18, 16 cases/16 and 3 cases/3. A lymph node biopsy was performed through mediastinoscopy in one case and yelded the diagnosis. The diagnosis was performed on surgical specimen in 2 patients: one bullectomy and one right upper lobectomy. The microscopic exam concluded to an EM in 17 cases, sarcomatoid mesothelioma (SM) in 4 cases and biphasic mesothelioma (BM) in 9 cases. Pan-cytokeratin antibody was used in all cases in association with 2 antibodies with positive diagnostic value and 2 antibodies with negative diagnostic value. It was repeated in 15 cases and the most used antibodies were the anti-calretinin and the TTF1. This was due to the lack of fixation in one case and in order to reach a quality criteria in the other cases. Surgical resection was possible in 2 patients. 15 patients were lost of view after a mean follow-up period of 3 months. Thirteen patients died before or during the follow-up. CONCLUSION: This work was about a Tunisian experience in the diagnosis and management of MPM. The major limits faced were the incomplete databases, the small number of patients included. Microsocpic positive diagnosis necessitates a degree of expertise and every laboratory has to determine the most valuable antibodies through its experience in order to optimize the diagnosis and to reduce the delay of diagnosis.

[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.

[Solitary plasmocytoma of the rib: a rare tumor not to miss]. / Plasmocytome solitaire à localisation costale: une tumeur rare à ne pas méconnaître.

Solitary plasmocytoma is a rare tumor accounting for 5% of all plasma cell neoplasias. The diagnosis is based on identification of the localized tumor composed of monoclonal plasma cells identical to those observed in multiple myeloma, and absence of the signs in favor of a disseminated form. We report the case of a52-year-old man who presented a growth of the chest wall in the left axillary region. Imaging disclosed a mass of tissue with a large zone of osteolysis of the 6th rib and infiltration of the chest wall. Surgical biopsy for pathology study and immunohistochemistry enabled the diagnosis of costal plasmocytoma. Blood protein immunoelectrophoresis revealed a monoclonal kappa type IgG. Bence-Jones proteinuria was positive. Search for other localizations was negative and the diagnosis of solitary plasmocytoma was retained. Radiotherapy was delivered and the patient has remained in remission at one year. Costal localization is rare for solitary plasmocytoma. The diagnosis is based on imaging findings and pathology. Radiotherapy is the treatment of choice but with the risk of progression with other bone lesions, the development of medullary plasmocytosis and multiple myeloma. Factors predictive of systemic recurrence have not been identified. Regular surveillance is required.

[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.

[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.