RESUMO
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.
Assuntos
Técnicas de Diagnóstico do Sistema Respiratório , Displasia Fibrosa Óssea/diagnóstico por imagem , Microscopia , Costelas/diagnóstico por imagem , Adulto , Feminino , Displasia Fibrosa Óssea/diagnóstico , Displasia Fibrosa Óssea/patologia , Displasia Fibrosa Óssea/cirurgia , Humanos , Masculino , Microscopia/métodos , Radiografia Torácica , Estudos Retrospectivos , Costelas/patologia , Costelas/cirurgia , Procedimentos Cirúrgicos Torácicos , Tomografia Computadorizada por Raios XRESUMO
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.
Assuntos
Tumor Carcinoide/patologia , Tumor Carcinoide/cirurgia , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/cirurgia , Adolescente , Adulto , Idoso , Broncoscopia , Tumor Carcinoide/mortalidade , Feminino , Humanos , Neoplasias Pulmonares/mortalidade , Metástase Linfática , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Estudos Retrospectivos , Tunísia/epidemiologia , Adulto JovemRESUMO
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.
Assuntos
Diabetes Mellitus Tipo 1/diagnóstico , Átrios do Coração , Cardiopatias/diagnóstico , Pneumopatias Fúngicas/diagnóstico , Infecções Oportunistas/diagnóstico , Trombose/diagnóstico , Zigomicose/diagnóstico , Adolescente , Biópsia , Broncoscopia , Terapia Combinada , Diagnóstico Diferencial , Feminino , Átrios do Coração/patologia , Cardiopatias/patologia , Cardiopatias/terapia , Humanos , Pulmão/patologia , Pneumopatias Fúngicas/patologia , Pneumopatias Fúngicas/terapia , Infecções Oportunistas/patologia , Infecções Oportunistas/terapia , Trombose/patologia , Trombose/terapia , Tomografia Computadorizada por Raios X , Zigomicose/patologia , Zigomicose/terapiaRESUMO
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.
Assuntos
Asma/terapia , Neurilemoma/cirurgia , Neoplasias da Traqueia/cirurgia , Adulto , Broncoscopia , Diagnóstico Diferencial , Humanos , Masculino , Neurilemoma/diagnóstico por imagem , Neurilemoma/patologia , Tomografia Computadorizada por Raios X , Neoplasias da Traqueia/diagnóstico por imagem , Neoplasias da Traqueia/patologiaRESUMO
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.
Assuntos
Neoplasias da Mama/patologia , Fibroadenoma/patologia , Glândulas Mamárias Humanas/patologia , Adulto , Neoplasias da Mama/diagnóstico , Neoplasias da Mama/cirurgia , Diagnóstico Diferencial , Feminino , Fibroadenoma/diagnóstico , Fibroadenoma/cirurgia , HumanosRESUMO
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.
Assuntos
Neoplasias Pulmonares/diagnóstico , Neoplasias Primárias Múltiplas/diagnóstico , Blastoma Pulmonar/diagnóstico , Biomarcadores Tumorais/análise , Broncoscopia , Proliferação de Células/fisiologia , Células Epiteliais/patologia , Humanos , Pulmão/patologia , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/cirurgia , Excisão de Linfonodo , Masculino , Células-Tronco Mesenquimais/patologia , Pessoa de Meia-Idade , Necrose , Estadiamento de Neoplasias , Neoplasias Primárias Múltiplas/patologia , Neoplasias Primárias Múltiplas/cirurgia , Pneumonectomia , Blastoma Pulmonar/patologia , Blastoma Pulmonar/cirurgia , Tomografia Computadorizada por Raios XRESUMO
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.
Assuntos
Actinomicose/diagnóstico , Fístula Cutânea/etiologia , Pneumopatias Fúngicas/diagnóstico , Actinomicose/complicações , Actinomicose/tratamento farmacológico , Actinomicose/patologia , Adulto , Azatioprina/uso terapêutico , Fístula Cutânea/microbiologia , Fístula Cutânea/patologia , Doxiciclina/uso terapêutico , Humanos , Pneumopatias Fúngicas/tratamento farmacológico , Pneumopatias Fúngicas/patologia , Masculino , Necrose , Penicilina G/uso terapêutico , Parede Torácica , Tórax , Resultado do TratamentoRESUMO
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.
Assuntos
Neoplasias do Sistema Nervoso Central/patologia , Melanoma/patologia , Adulto , Neoplasias Encefálicas/patologia , Neoplasias do Sistema Nervoso Central/complicações , Neoplasias do Sistema Nervoso Central/cirurgia , Diagnóstico Diferencial , Feminino , Humanos , Hipertensão Intracraniana/etiologia , Imageamento por Ressonância Magnética , Masculino , Melanoma/complicações , Melanoma/cirurgia , Neoplasias da Medula Espinal/patologiaRESUMO
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.
Assuntos
Lipossarcoma Mixoide/secundário , Neoplasias Orbitárias/secundário , Exoftalmia/etiologia , Enucleação Ocular , Humanos , Perna (Membro) , Lipossarcoma Mixoide/cirurgia , Masculino , Pessoa de Meia-Idade , Neoplasias Orbitárias/cirurgia , Recusa do Paciente ao TratamentoRESUMO
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.
Assuntos
Neoplasias Abdominais/patologia , Adolescente , Adulto , Feminino , Humanos , MasculinoRESUMO
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.
Assuntos
Neoplasias Encefálicas , Tumores Neuroectodérmicos Primitivos , Lobo Parietal , Lobo Temporal , Adolescente , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/terapia , Humanos , Masculino , Tumores Neuroectodérmicos Primitivos/patologia , Tumores Neuroectodérmicos Primitivos/terapiaRESUMO
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.
Assuntos
Neoplasias da Medula Espinal/patologia , Teratoma/patologia , Adulto , Humanos , Imageamento por Ressonância Magnética , Masculino , Procedimentos Neurocirúrgicos , Região Sacrococcígea , Neoplasias da Medula Espinal/cirurgia , Teratoma/cirurgiaRESUMO
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.
Assuntos
Tornozelo , Cistos/etiologia , Phaeophyceae/isolamento & purificação , Phaeophyceae/patogenicidade , Adulto , Cistos/patologia , Feminino , HumanosRESUMO
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.
Assuntos
Encefalopatias/microbiologia , Equinococose/diagnóstico , Encéfalo/diagnóstico por imagem , Encéfalo/microbiologia , Encefalopatias/diagnóstico por imagem , Pré-Escolar , Equinococose/epidemiologia , Feminino , Humanos , Radiografia , TunísiaRESUMO
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.
Assuntos
Cordoma/cirurgia , Neoplasias Meníngeas/cirurgia , Meningioma/cirurgia , Biomarcadores Tumorais/análise , Cordoma/diagnóstico , Cordoma/patologia , Diagnóstico Diferencial , Epilepsia Tônico-Clônica/etiologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Neoplasias Meníngeas/diagnóstico , Neoplasias Meníngeas/patologia , Meninges/patologia , Meninges/cirurgia , Meningioma/diagnóstico , Meningioma/patologia , Pessoa de Meia-Idade , Mucina-1/análise , Exame Neurológico , Tomografia Computadorizada por Raios X , Vimentina/análiseRESUMO
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.
Assuntos
Neoplasias Ósseas/diagnóstico , Condroblastoma/diagnóstico , Costelas/patologia , Adulto , Biópsia , Feminino , Seguimentos , Humanos , Radiografia Torácica , Tomografia Computadorizada por Raios XRESUMO
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.
Assuntos
Pneumopatias Fúngicas/diagnóstico , Zigomicose/diagnóstico , Idoso , Diabetes Mellitus Tipo 2/complicações , Feminino , Humanos , Hospedeiro Imunocomprometido , Pneumopatias Fúngicas/tratamento farmacológico , Pessoa de Meia-Idade , Zigomicose/tratamento farmacológicoRESUMO
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.
Assuntos
Adenocarcinoma de Células Claras/diagnóstico , Neoplasias Pulmonares/diagnóstico , Adenocarcinoma de Células Claras/química , Adenocarcinoma de Células Claras/diagnóstico por imagem , Adenocarcinoma de Células Claras/patologia , Adenocarcinoma de Células Claras/cirurgia , Adulto , Antígenos de Neoplasias , Biomarcadores Tumorais/análise , Citoplasma/química , Diagnóstico Diferencial , Feminino , Glicogênio/análise , Humanos , Imuno-Histoquímica , Neoplasias Pulmonares/química , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/cirurgia , Antígenos Específicos de Melanoma , Proteínas de Neoplasias/análise , Pneumonectomia , Radiografia , Resultado do TratamentoRESUMO
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.