Endobronchial solitary fibrous tumor

Solitary fibrous tumor (SFT) is a mesenchymal neoplasm that appears primarily in the pleura and rarely in intrapulmonary or endobronchial topography. The authors report the case of a 47-year-old woman who presented obstructive respiratory symptoms for 4 years. The chest computed tomography and bronchoscopy showed an obstructive polypoid lesion located between the trachea and the left main bronchus associated with distal atelectasis of the left lung. A resection of the lesion was performed and, macroscopically, the mass was oval, encapsulated, and firm, measuring 2.3 × 1.7 × 1.5 cm. Histology revealed low-grade mesenchymal spindle cell neoplasm, with alternating cellularity, myxoid areas, and mature adipose tissue outbreaks, as well as blood vessels with irregular walls. The immunohistochemical study was positive for CD34, CD99, and BCL2. The diagnosis was SFT in an unusual topography. The patient’s symptoms remitted after tumor excision, and no systemic problems were evident. SFTs primarily affect adults and often follow a benign course; however, their behavior is unpredictable. The presence of necrosis and mitotic activity may portend a poor prognosis. Endobronchial SFTs are rare but should be evaluated and monitored similar to SFTs at other sites, with a long-term follow-u

Myelolipoma of the posterior mediastinum in a patient with chronic dyserythropoietic anemia

Myelolipoma (ML) is an uncommon benign mesenchymal neoplasia composed of mature adipose and hematopoietic tissues of uncertain etiology. Less than 3% of MLs occur in the mediastinal topography. The main differential diagnosis involves extramedullary hematopoiesis; therefore, pathological evaluation is essential for the definitive diagnosis. The authors report the case of a 50-year-old man diagnosed with congenital dyserythropoiesis and secondary hemosiderosis, who presented a posterior mediastinal tumor. The tumor was resected. It was macroscopically characterized by mature fat tissue with fibrous areas and soft consistency, which was yellowish at the cut surface. Histology revealed a well-defined nodule composed of adipocytes and hematopoietic tissue represented by erythroid, granulocytic, and megakaryocytic series, which was consistent with the diagnosis of ML located in the posterior mediastinum. There was no recurrence of the lesion during the 3-year follow-up. The aim of this report is to show the diagnosis of an unusual mediastinal lesion in the context of a chronic hematologic diseas

Endobronchial solitary fibrous tumor

Solitary fibrous tumor (SFT) is a mesenchymal neoplasm that appears primarily in the pleura and rarely in intrapulmonary or endobronchial topography. The authors report the case of a 47-year-old woman who presented obstructive respiratory symptoms for 4 years. The chest computed tomography and bronchoscopy showed an obstructive polypoid lesion located between the trachea and the left main bronchus associated with distal atelectasis of the left lung. A resection of the lesion was performed and, macroscopically, the mass was oval, encapsulated, and firm, measuring 2.3 × 1.7 × 1.5 cm. Histology revealed low-grade mesenchymal spindle cell neoplasm, with alternating cellularity, myxoid areas, and mature adipose tissue outbreaks, as well as blood vessels with irregular walls. The immunohistochemical study was positive for CD34, CD99, and BCL2. The diagnosis was SFT in an unusual topography. The patient’s symptoms remitted after tumor excision, and no systemic problems were evident. SFTs primarily affect adults and often follow a benign course; however, their behavior is unpredictable. The presence of necrosis and mitotic activity may portend a poor prognosis. Endobronchial SFTs are rare but should be evaluated and monitored similar to SFTs at other sites, with a long-term follow-u

Traqueobroncopatia osteocondroplástica em portador de tumor de Klatskin: relato de caso e revisão da literatura

A traqueopatia osteocondroplástica (TO) é uma desordem idiopática, incomum, caracterizada pela presença de nódulos osteocartilaginosos na submucosa das vias aéreas, causando rigidez e estreitamento da árvore respiratória. Afeta principalmente homens acima dos 50 anos com manifestações clínicas devidas à obstrução e/ou a infecções locais. Sua patogênese é desconhecida. Relatamos um caso de TO encontrada acidentalmente em autopsia de mulher com 73 anos de idade, que apresentava carcinoma ductal biliar extra hepático (tumor de Klatskin).Tracheopathia osteochondroplastica (TO) is an unusual idiopathic disorder, characterized by osteocartilaginous nodules in the submucosa of the respiratory airway, which causes rigidity and narrowing of the respiratory tree. It affects mainly men over 50 and clinical manifestations are due to obstruction and/or local infections. The pathogenesis is uncertain. We report a case of TO found incidentally in an autopsy of a 73 year-old woman, who had an extrahepatic biliary ductal carcinoma (Klatskin tumor)

Tuberculose em necropsias realizadas no Serviço de Anatomia Patológica da Faculdade de Medicina de Botucatu

Entre 6.316 necropsias realizadas, no período de 1969 a 2000, no Departamento de Patologia da Faculdade de Medicina de Botucatu da Universidade Estadual Paulista (FMB/UNESP), foram encontradas 240 necropsias com diagnóstico de tuberculose, sendo 117 registradas como doença principal (grupo tuberculose/doença principal) e 123, como doença associada (grupo tuberculose/doença associada). No grupo tuberculose/doença principal, 100% apresentavam tuberculose ativa, com 80 ocorrências em homens e 37 em mulheres e média de idade de 47,7 anos. Caquexia (37,2%) e cor pulmonale (23,7%) foram as co-morbidades mais prevalentes nesse grupo. Os pulmões estavam comprometidos em 95,7% dos casos, seguido pelos linfonodos (38,9%) e pleuras (27,1%). Os padrões morfológicos das lesões pulmonares foram: 1. miliar, 58,4%; 2. cavernas, 56,6%; 3. fibrose, 41,5%; 4. bronquiectasias, 26,5%; 5. enfisema, 19,4%; e 6. cistos, 1,7%. Cavernas foram observadas em diferentes áreas, com discreto predomínio no pulmão direito, sendo a localização apical preferencial (100%) e quase sempre bilaterais (94%). Lesões miliares, bronquiectasias e fibrose estavam distribuídas, de forma difusa e aleatória, nos pulmões. No grupo tuberculose/doença associada também houve predomínio de homens (70,8%). Nesses casos, a AIDS e o alcoolismo crônico foram as doenças principais mais freqüentes, representando 22% e 16,3%, respectivamente. Os pulmões estavam acometidos em 85,2% dessas necropsias (100% nos casos de AIDS), seguidos pelos linfonodos (31,9%) e baço (28,6%). Nossos dados corroboram a importância da necropsia no estudo e no conhecimento da tuberculose, fornecendo subsídios para uma melhor abordagem clínica e epidemiológica dessa doença em nossa região.Among 6,316 necropsies performed in the period 1969-2000 at the Department of Pathology of Botucatu Medical School UNESP, 240 were of tuberculosis. Among them, 117 were registered as the principal disease (tuberculosis/principal disease group) and 123, as associated to another disease (tuberculosis/associated disease group). In the tuberculosis-principal disease group, 100% had active tuberculosis, 80 patients were male and 37 were female, with mean age of 47.7 years. Cachexia (37.2%) and cor pulmonale (23.7%) were the most prevalent comorbities in this group. The lungs were affected in 95.7% of the cases, followed by lymph nodes (38.9%) and pleura (27.1%). The morphology and frequency of pulmonary lesions were: 1. miliar: 58.4%; 2. caverns, 56.6%; 3. fibrosis, 41.5%; 4. bronchiectasias, 26.5%; 5. emphysema, 19.4% and 6. cysts, 1.7%. Caverns were observed in different areas of both lungs, with discreet predominance in the right lung, were located mainly in the apices (100%), and the great majority (94%) was bilateral. The others lesions were distributed randomly in both lungs. In the tuberculosis/associated disease group, 70.8% was male, and the two most frequent principal diseases were AIDS (22.1%) and alcoholism (16.3%). Also, the lung was the most affected organ (85.2%), followed by lymph nodes (31.9%) and spleen (28.6%). In 100% of AIDS there were pulmonary lesions. This study in necropsies reinforces epidemiological data that tuberculosis is an important disease, and, also, constitutes an invaluable tool to gather information for a better understanding of the pathology and the epidemiology of tuberculosis in our region

Skin and pulmonary models using coated bentonite particles for the study of the inflammation evoked by Paracoccidioides brasiliensis antigens in previously immunized mice

Bentonite particles coated with polysaccharide antigen or crude soluble antigen of Paracoccidioides brasiliensis were injected intradermally or intravenously in mice. In control animals that were not pre-immunized with P. brasiliensis antigens, coated and uncoated bentonite caused minimal and nonspecific inflammation around the cutaneous injection site or around the bentonite thrombi in small lung vessels after intravenous injection. However, in mice previously immunized with P. brasiliensis antigens, the coated bentonite particles boosted the humoral and cellular immune responses to P. brasiliensis and evoked intense inflammatory reactions. Twelve days after intradermal injection, the inflammatory reaction around the bentonite was rich in neutrophils, macrophages, lymphocytes and plasma cells associated with young granulation tissue. In intravenously injected mice, the pulmonary inflammation was maximal at day 2, and was characterized by a florid neutrophilic and macrophagic cellular infiltration around bentonite thrombi; in some foci, there was incipient organization to mature granuloma. However, in both models, there was no formation of epithelioid granulomata, demonstrating that in paracoccidioidomycosis cellular immunity alone, without the presence of intact micro-organisms, may not be enough for the development of this type of granuloma

Teratoma With Mediastinal Embryonic Carcinoma Concomiting Hystiocytic Sarcoma In The Bone Marrow

Background: Association of extragonadal germ cell tumors EGCT with haematological malignancies is rare with a very limited prognosis. Methods and findings: We report a case of a man, 21 years old, with progressive dyspnea, chest pain, night sweats, generalized edema, hemoptysis, pancytopenia and increased serum levels of alpha-fetoprotein (AFP), beta-fraction of the hormone chorionic gonodotrophin (beta-HCG) and lactic dehydrogenase. Chest computed tomography described a mediastinum mass measuring 11.3 x 7.3 cm whose biopsy revealed malignant germ cell neoplasia expressing positivity for cytokeratin, CD30 and AFP, indicating an embryonal carcinoma with teratoma. Bone marrow biopsy revealed interstitial infiltration by malignant neoplastic cells positive for CD68, CD163 and lysozyme, allowing the diagnosis of histiocytic sarcoma. The patient had a poor evolution, with death, sixteen days after admission. Conclusion: Histiocytic proliferations are very rare and rapidly fatal. Etiopathogenic theories for the simultaneity of these malignancies have been described based on processes of transformation from a same cellular precursor