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1.
Zhonghua Bing Li Xue Za Zhi ; 50(10): 1139-1144, 2021 Oct 08.
Artigo em Chinês | MEDLINE | ID: mdl-34619867

RESUMO

Objective: To investigate the clinicopathological features and prognostic factors of primary mediastinal large B-cell lymphoma (PMBL). Methods: The clinical data of 60 patients with PMBL including 44 biopsy cases and 16 consultation cases from September 2000 to November 2019 in the Department of Pathology, China-Japan Friendship Hospital (14 cases) and Peking Union Medical College Hospital (46 cases) were enrolled. Pathologic features, immunophenotype, immunoglobulin (Ig) gene rearrangement and microRNA expression profile were retrospectively studied. Results: Of the 60 patients, 23 were males and 37 were females, age ranged from 15 to 64 years (median 28 years). Immunohistochemical staining showed that the tumor cells were positive for pan-B cell antigens, CD30 (77.4%, 24/31), CD23 (73.1%, 19/26), MUM1 (45.8%, 11/24), Ki-67 index ≥70 % (90.6%, 29/32). EBER in situ hybridization was analyzed in 21 PMBL, only one case (4.8%) was positive. Ig gene rearrangement was performed in 20 cases, and seven were positive (35.0%). MicroRNA gene expression profiles were analyzed in seven cases of PMBL and nine cases of diffuse large B-cell lymphoma, and there were 33 microRNAs with significant difference (P<0.05). Univariate analysis indicated that the poor prognostic factors included serum lactate dehydrogenase (LDH) level,International Prognostic Index (IPI) score ≥3, stages Ⅲ-Ⅳ, chemotherapy not combined with rituximab and MUM1 positivity (P<0.05). Multivariate analysis showed that the treatment combined with rituximab was independently related to prognosis (P<0.05). Conclusions: PMBL is different from diffuse large B-cell lymphoma in clinicopathologic features, immunophenotypic presentation and molecular features. The prognostic factors, molecular genetics and immunological characteristics reveal that this study has enriched our understanding of the biology of PMBL, thus providing evidence and strategies for treatment.


Assuntos
Linfoma Difuso de Grandes Células B , Neoplasias do Mediastino , MicroRNAs , Adolescente , Adulto , Feminino , Humanos , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Linfoma Difuso de Grandes Células B/genética , Masculino , Neoplasias do Mediastino/tratamento farmacológico , Neoplasias do Mediastino/genética , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Adulto Jovem
2.
Kyobu Geka ; 74(10): 839-844, 2021 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-34548456

RESUMO

The mediastinum contains large blood vessels, airways, and spinal cord, which are anatomically important parts of the human body because they survive by injury, obstruction, and compression, and are involved in activities of daily living( ADL). Therefore, even benign tumors have been indicated for aggressive surgical intervention. Dissection procedures from these anatomically important structures is extremely risky and difficult in reoperation for recurrence of mediastinal tumors, so careful consideration and attention must be paid to the surgical indications and methods. In this paper, we have described the points to be noted and points regarding the reoperation of mediastinal tumors with some review of the literature, including our own cases.


Assuntos
Neoplasias do Mediastino , Atividades Cotidianas , Humanos , Neoplasias do Mediastino/diagnóstico por imagem , Neoplasias do Mediastino/cirurgia , Mediastino , Recidiva Local de Neoplasia , Reoperação
3.
Khirurgiia (Mosk) ; (9): 19-26, 2021.
Artigo em Russo | MEDLINE | ID: mdl-34480451

RESUMO

OBJECTIVE: To improve the results of surgical treatment of cervico-mediastinal tumors by using of partial upper cervicosternotomy. MATERIAL AND METHODS: A retrospective analysis included 24 patients who underwent resection of cervico-mediastinal tumors via partial upper cervicosternotomy for the period from January 2002 to December 2019. Immediate and intermediate postoperative outcomes were analyzed. RESULTS: Mean surgery time was 282.7 min, intraoperative blood loss - 325.0 ml. Duration of pleural cavity (mediastinum) drainage was 3 days, hospital-stay - 14 days. Major postoperative complications developed in 3 (12.5%) patients. No 90-day mortality was observed. No local relapses were detected throughout the follow-up period (median 36.1 months). CONCLUSION: Partial cervicosternotomy is a safe and effective approach ensuring adequate visualization and reliable control of great vessels of the upper mediastinum and neck. This access is valuable for en-bloc resection of cervico-mediastinal tumors located in anterior and posterior parts of the thoracic inlet.


Assuntos
Neoplasias do Mediastino , Recidiva Local de Neoplasia , Humanos , Mediastino , Duração da Cirurgia , Estudos Retrospectivos
4.
Ann R Coll Surg Engl ; 103(8): e255-e258, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34464560

RESUMO

Liposarcoma is one of the most common soft tissue malignancies, occurring mostly in the extremities and retroperitoneal cavities, and occasionally in the mediastinum of the thoracic cavity. Here, we present a patient undergoing four operations over a period of 33 years because of repeated recurrence of mediastinal liposarcoma. A 34-year-old woman underwent her first surgery for mediastinal liposarcoma in 1986. Ten years later, a recurrent tumour was found during follow-up. The patient underwent a second operation for complete excision. The pathology was liposarcoma, partly myxoid and partly dedifferentiated. The patient remained tumour-free for 22 years, until one year ago, when a third operation was performed to resect the recurrent tumour which was myxoid liposarcoma. Unfortunately, upon computed tomography imaging three months later a fatty mass was spotted which increased rapidly in size. The patient underwent further surgery to achieve radical excision of the recurrent liposarcoma. Postoperative recovery was uneventful, and a follow-up examination showed no recurrence to date. For mediastinal liposarcoma, surgical removal is the treatment of choice. Considering that the tumour is prone to recurrence, the lesion should be removed as thoroughly as possible in the first operation. If the tumour recurs repeatedly, multiple resections are beneficial to patient survival on most occasions.


Assuntos
Lipossarcoma/cirurgia , Neoplasias do Mediastino/cirurgia , Recidiva Local de Neoplasia/cirurgia , Adulto , Feminino , Humanos , Lipossarcoma/diagnóstico por imagem , Lipossarcoma/patologia , Neoplasias do Mediastino/diagnóstico por imagem , Neoplasias do Mediastino/patologia , Recidiva Local de Neoplasia/diagnóstico por imagem , Recidiva Local de Neoplasia/patologia , Tomografia Computadorizada por Raios X
5.
Medicine (Baltimore) ; 100(29): e26480, 2021 Jul 23.
Artigo em Inglês | MEDLINE | ID: mdl-34398004

RESUMO

ABSTRACT: Primary mediastinal yolk sac tumors (PMYSTs) are a rare occurrence. As such, the clinicopathological features, treatment, and prognosis, of this disease still remain unclear. In this study, we aimed to provide further information relating to this rare malignancy in order to facilitate the creation of more specific clinical guidelines for the diagnosis and treatment of patients with PMYSTs.In this retrospective study, we recruited 15 patients who had been diagnosed with PMYST from four medical institutions to create a population-based cohort. We then used Kaplan-Meier analysis and the log-rank test to investigate and compare overall survival (OS) and progression-free survival (PFS).A total of 15 cases were identified. The mean age was 27.3 years (range: 19-34 years). The estimated 1- and 2-year PFS rates were 66.7% and 60.0%, respectively. The 1- and 2-year OS rates were both 73.3%. Computer tomography scans revealed tumors were located in the anterior middle mediastinum (5 cases), the anterior superior mediastinum (1 case), the left anterior mediastinum (3 cases), and the right anterior mediastinum (6 cases). Of the 15 patients receiving extended resections, the majority (40.0%) underwent tumor resection, partial pericardiotomy, pulmonary wedge resection, and mediastinal lymphadenectomy. R0 resections were achieved in eleven patients. Four patients underwent R2 resection and experienced postoperative complications, including pneumonia (2 cases), atelectasis (1 case), and bronchopleural fistula (1 case). Four patients developed postoperative lung metastasis. Three patients died due to progressive diseases. Disease recurred in all patients at a median of 8.0 months (range: 6.0-11.0 months).PMYST is a rare but highly malignant tumor with a poor prognosis. Tumor resection, with optimal extended surgical management, may provide patients with the best chance of a cure although postoperative complications relating to the pulmonary systems should be treated with caution.


Assuntos
Tumor do Seio Endodérmico/complicações , Neoplasias do Mediastino/complicações , Prognóstico , Adulto , Tumor do Seio Endodérmico/mortalidade , Feminino , Humanos , Estimativa de Kaplan-Meier , Neoplasias Pulmonares/etiologia , Neoplasias Pulmonares/fisiopatologia , Masculino , Neoplasias do Mediastino/mortalidade , Avaliação de Resultados em Cuidados de Saúde/estatística & dados numéricos , Complicações Pós-Operatórias , Estudos Retrospectivos
6.
Kyobu Geka ; 74(9): 720-723, 2021 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-34446630

RESUMO

Primary mediastinal leiomyosarcoma is extremely rare, and few reports in the literature have described the clinical features of this malignancy. We report a case of a small anterior mediastinal leiomyosarcoma that showed rapid growth within a short period. An 85-year-old woman showed a small anterior mediastinal tumor on chest computed tomography (CT), three months prior to presentation. Contrast-enhanced chest CT revealed rapid tumor growth, and positron emission tomography/CT revealed significant 18-fluorodeoxyglucose uptake, suggestive of malignancy. Thoracoscopic tumor resection was performed via the left thoracic approach. In addition to the tumor and surrounding anterior mediastinal tissue, we resected an area of pericardial infiltration. The tumor was diagnosed as a primary mediastinal leiomyosarcoma based on histopathological and immunohistochemical findings.


Assuntos
Leiomiossarcoma , Neoplasias do Mediastino , Idoso de 80 Anos ou mais , Feminino , Fluordesoxiglucose F18 , Humanos , Leiomiossarcoma/diagnóstico por imagem , Leiomiossarcoma/cirurgia , Neoplasias do Mediastino/diagnóstico por imagem , Neoplasias do Mediastino/cirurgia , Mediastino/diagnóstico por imagem , Mediastino/cirurgia , Tomografia Computadorizada por Raios X
7.
Adv Anat Pathol ; 28(5): 289-290, 2021 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-34294658
8.
Pan Afr Med J ; 38: 330, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34285753

RESUMO

The mediastinal malignant germ cells tumor represents less than 0.5% of thoracic tumors, although the mediastinum is one of the main extragonadic locations of these tumors. In the majority of cases, young people are those most affected. The prognosis of mediastinal malignant germ cells tumors is poor, especially non-seminomatous germ tumors. In this article, we report a rare case of a young 19-years-old patient treated for a mediastinal germ cell tumor of yolk sac. The patient presented a chest pain; the chest computed tomography (CT) showed a right paramedian mediastinal mass with a pleural effusion associated with supraclavicular and cervical lymph nodes. Biopsy revealed a non-seminomatousgerm cell tumor of yolk sac. The exams showed elevated alpha-fetoprotein (AFP), without any meaningful elevation of other serictumor markers. The patient received 4 cycles of chemotherapy based on etoposide, ifosfamide and platinum salts then a complete excision of the mass.


Assuntos
Dor no Peito/etiologia , Tumor do Seio Endodérmico/diagnóstico , Neoplasias do Mediastino/diagnóstico , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Biópsia , Terapia Combinada , Tumor do Seio Endodérmico/patologia , Tumor do Seio Endodérmico/terapia , Etoposídeo/administração & dosagem , Humanos , Ifosfamida/administração & dosagem , Neoplasias do Mediastino/patologia , Neoplasias do Mediastino/terapia , Compostos de Platina/administração & dosagem , Tomografia Computadorizada por Raios X , Adulto Jovem
9.
Ann Afr Med ; 20(2): 150-153, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34213485

RESUMO

Ectopic parathyroid adenomas in the mediastinum are rare causes of primary hyperparathyroidism. We report two cases of mediastinal parathyroid adenoma. Functioning parathyroid lesion was localized with the help of nuclear single-photon emission computed tomography scan in both the patients. Video assisted thoracoscopic surgical (VATS) removal of the parathyroid lesions were done. Intraoperative confirmation of parathyroid adenoma was done by frozen section. Further confirmation was done by routine histopathological examination of specimen postoperatively. One patient had left vocal cord paralysis postoperatively. Localization by functional imaging is essential. Minimally invasive methods such as VATS are useful in removing mediastinal parathyroid hyperfunctioning lesions, which carries early postoperative recovery and less complications.


Assuntos
Adenoma/cirurgia , Hipercalcemia/etiologia , Neoplasias do Mediastino/cirurgia , Neoplasias das Paratireoides/cirurgia , Cirurgia Torácica Vídeoassistida/métodos , Adenoma/patologia , Adulto , Feminino , Humanos , Hipercalcemia/sangue , Hipercalciúria/sangue , Hipercalciúria/etiologia , Hiperparatireoidismo/etiologia , Masculino , Pessoa de Meia-Idade , Hormônio Paratireóideo/sangue , Neoplasias das Paratireoides/patologia , Paratireoidectomia , Glândula Tireoide/patologia
10.
J Comput Assist Tomogr ; 45(3): 490-494, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34297519

RESUMO

OBJECTIVE: This study explored the feasibility of dual-energy computed tomography (DECT) for the diagnosis of mediastinal lymph node (LN) metastasis in patients with lung cancer. METHODS: Forty-two consecutive patients with lung cancer, who underwent DECT, were included in this retrospective study. The attenuation value (Hounsfield unit) in virtual monochromatic images and the iodine concentration in the iodine map were measured at mediastinal LNs. The slope of the spectral attenuation curve (K) and normalized iodine concentration (in thoracic aorta) were calculated. The measurement results were statistically compared using 2 independent samples t test. Receiver operating characteristic curve analysis, net reclassification improvement, and integrated discrimination improvement were used to evaluate the diagnostic performance of DECT for mediastinal LN metastasis. RESULTS: A total of 74 mediastinal LNs were obtained, including 33 metastatic LNs and 41 nonmetastatic LNs. The attenuation value at the lower energy levels of virtual monochromatic images (40-90 keV), K, and normalized iodine concentration demonstrated a significant difference between metastatic LNs and nonmetastatic LNs. The attenuation value at 40 keV was the most favorable biomarker for the diagnosis of mediastinal LN metastasis (area under curve, 0.91; sensitivity, 0.94; specificity, 0.81), which showed a much better performance than the LN diameter-based evaluation method (area under curve, 0.72; sensitivity, 0.66; specificity, 0.82; net reclassification improvement, 0.359; integrated discrimination improvement, 0.330). CONCLUSIONS: Dual-energy computed tomography is a promising diagnostic approach for the diagnosis of mediastinal LN metastasis in patients with lung cancer, which may help clinicians implement personalized treatment strategies.


Assuntos
Neoplasias Pulmonares/diagnóstico por imagem , Metástase Linfática/diagnóstico por imagem , Neoplasias do Mediastino/diagnóstico por imagem , Neoplasias do Mediastino/secundário , Imagem Radiográfica a Partir de Emissão de Duplo Fóton/métodos , Idoso , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Medicina de Precisão , Curva ROC , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
11.
BMC Res Notes ; 14(1): 278, 2021 Jul 21.
Artigo em Inglês | MEDLINE | ID: mdl-34289875

RESUMO

OBJECTIVE: Mediastinal schwannomas are sometimes confused with other neoplasms during initial radiological studies, especially when there is a history of cancer in another area. In these cases, a more accurate analysis using computed tomography (CT) or even magnetic resonance (MRI) is required. Our study aimed to perform a retrospective analysis of the clinical and imaging features for a series of patients with mediastinal schwannomas that were confirmed by histology and immunohistochemistry. RESULTS: We found eight patients, five men and three women, with an average age of 51 years for this study. The main signs and symptoms at diagnosis were chest pain, dyspnea, cough, and dysphagia. CT showed that the tumor was located in the posterior compartment of the chest in 7/8 cases. Tumors > 10 cm were more heterogeneous and showed cystic changes. All patients underwent posterolateral thoracotomy, and radiological follow-up showed no evidence of recurrence. Histological analysis was considered the gold standard to confirm diagnosis, along with at least one neurogenic IHC marker. In conclusion, mediastinal schwannomas are benign encapsulated tumors. According to CT, schwannomas > 10 cm show cystic degeneration more frequently. Posterolateral thoracotomy allows complete resection and is considered the surgical approach of choice.


Assuntos
Neoplasias do Mediastino , Neurilemoma , Feminino , Humanos , Masculino , Neoplasias do Mediastino/diagnóstico por imagem , Neoplasias do Mediastino/cirurgia , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Neurilemoma/diagnóstico por imagem , Neurilemoma/cirurgia , Estudos Retrospectivos , Toracotomia
12.
J Med Case Rep ; 15(1): 375, 2021 Jul 28.
Artigo em Inglês | MEDLINE | ID: mdl-34315532

RESUMO

BACKGROUND: Non-Hodgkin lymphoma is the fourth most common malignancy in children, and it is not considered to be a hereditary disorder. However, it could affect members from the same family. CASE PRESENTATION: We are presenting two cases of Caucasian female siblings who were diagnosed with mediastinal lymphoblastic lymphoma in the same year. The two young females were presented to the emergency department with respiratory symptoms. After doing radiological investigations and biopsies, they were diagnosed with lymphoblastic lymphoma. The elder sister died before confirming the diagnosis, and the other is on chemotherapy now, with good treatment outcomes. CONCLUSIONS: This case emphasizes the crucial role of precursor genetics in lymphoblastic lymphomas and suggests a strong relation between these genetics and age at symptom presentation. This is the first report of non-Hodgkin lymphoma in a pair of siblings in the pediatric population.


Assuntos
Linfoma não Hodgkin , Linfoma , Neoplasias do Mediastino , Leucemia-Linfoma Linfoblástico de Células Precursoras , Pré-Escolar , Feminino , Humanos , Lactente , Linfoma não Hodgkin/diagnóstico , Linfoma não Hodgkin/tratamento farmacológico , Linfoma não Hodgkin/genética , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Irmãos
13.
Adv Anat Pathol ; 28(5): 307-334, 2021 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-34074861

RESUMO

Lymphoproliferative disorders comprise 50% to 60% of all mediastinal malignancies in both children and adults. Primary mediastinal involvement is rare (∼5%), whereas secondary mediastinal involvement by systemic disease is more common (10% to 25%). Primary mediastinal disease is defined as involvement by a lymphoproliferative disorder of mediastinal lymph nodes, the thymus, and/or extranodal mediastinal organs without evidence of systemic disease at presentation. In this review, the clinical, radiologic, histopathologic, immunohistochemical, and genetic features of some of the most characteristic mediastinal lymphoproliferative disorders are presented. The entities discussed here include: classic Hodgkin lymphoma with emphasis on nodular sclerosis and mixed cellularity types, and non-Hodgkin lymphomas, including primary mediastinal (thymic) large B-cell lymphoma, mediastinal gray zone lymphoma, mediastinal diffuse large B-cell lymphoma, thymic marginal zone lymphoma, mediastinal plasmacytoma, T-lymphoblastic lymphoma, and anaplastic large cell lymphoma. Although not a malignant process, hyaline vascular Castleman disease is also discussed here as this disorder commonly involves the mediastinum. Despite multiple advances in hematopathology in recent decades, the day-to-day diagnosis of these lesions still requires a morphologic approach and a proper selection of immunohistochemical markers. For this reason, it is crucial for general pathologists to be familiar with these entities and their particular clinicoradiologic presentation.


Assuntos
Linfoma não Hodgkin/patologia , Transtornos Linfoproliferativos/patologia , Neoplasias do Mediastino/patologia , Mediastino/patologia , Doença de Hodgkin/patologia , Humanos , Linfonodos/patologia
18.
Medicine (Baltimore) ; 100(25): e26384, 2021 Jun 25.
Artigo em Inglês | MEDLINE | ID: mdl-34160415

RESUMO

RATIONALE: A hormone-active metastatic Hürthle cell thyroid carcinoma (HCTC) and Graves disease (GD) present a therapeutic challenge and is rarely reported. PATIENT CONCERNS: We present a 64-year-old male patient, who had dyspnea and left hip pain lasting 4 months. He had clinical signs of hyperthyroidism and a tumor measuring 9 cm in diameter of the left thyroid lobe, metastatic neck lymph node and metastases in the lungs, mediastinum, and bones. DIAGNOSIS: Laboratory findings confirmed hyperthyroidism and GD. Fine-needle aspiration biopsy and cytological investigation revealed metastases of HCTC in the skull and in the 8th right rib. A CT examination showed a thyroid tumor, metastatic neck lymph node, metastases in the lungs, mediastinum and in the 8th right rib measuring 20 × 5.6 × 4.5 cm, in the left acetabulum measuring 9 × 9 × 3 cm and parietooccipitally in the skull measuring 5 × 4 × 2 cm. Histology after total thyroidectomy and resection of the 8th right rib confirmed metastatic HCTC. INTERVENTIONS: The region of the left hip had been irradiated with concomitant doxorubicin 20 mg once weekly. When hyperthyroidism was controlled with thiamazole, a total thyroidectomy was performed. Persistent T3 hyperthyroidism, most likely caused by TSH-R-stimulated T3 production in large metastasis in the 8th right rib, was eliminated by rib resection. Thereafter, the patient was treated with 3 radioactive iodine-131 (RAI) therapies (cumulative dose of 515 mCi). Unfortunately, the tumor rapidly progressed after treatment with RAI and progressed 10 months after therapy with sorafenib. OUTCOMES: Despite treatment, the disease rapidly progressed and patient died due to distant metastases. He survived for 28 months from diagnosis. LESSONS: Simultaneous hormone-active HCTC and GD is extremely rare and prognosis is dismal. Concomitant external beam radiotherapy and doxorubicin chemotherapy, followed by RAI therapy, prevented the growth of a large metastasis in the left hip in our patient. However, a large metastasis in the 8th right rib presented an unresolved problem. Treatment with rib resection and RAI did not prevent tumor recurrence. External beam radiotherapy and sorafenib treatment failed to prevent tumor growth.


Assuntos
Adenoma Oxífilo/diagnóstico , Doença de Graves/diagnóstico , Neoplasias da Glândula Tireoide/diagnóstico , Adenoma Oxífilo/complicações , Adenoma Oxífilo/secundário , Adenoma Oxífilo/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biópsia por Agulha Fina , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/secundário , Neoplasias Ósseas/terapia , Quimiorradioterapia Adjuvante/métodos , Evolução Fatal , Doença de Graves/complicações , Doença de Graves/terapia , Humanos , Radioisótopos do Iodo/uso terapêutico , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/secundário , Neoplasias Pulmonares/terapia , Metástase Linfática/diagnóstico , Metástase Linfática/terapia , Masculino , Neoplasias do Mediastino/diagnóstico , Neoplasias do Mediastino/secundário , Neoplasias do Mediastino/terapia , Pessoa de Meia-Idade , Terapia Neoadjuvante/métodos , Glândula Tireoide/diagnóstico por imagem , Glândula Tireoide/patologia , Glândula Tireoide/cirurgia , Neoplasias da Glândula Tireoide/complicações , Neoplasias da Glândula Tireoide/patologia , Neoplasias da Glândula Tireoide/secundário , Neoplasias da Glândula Tireoide/terapia , Tireoidectomia
19.
Isr Med Assoc J ; 23(6): 350-352, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-34155847

RESUMO

BACKGROUND: Granulomatosis with polyangiitis (GPA) is a rare small vessel vasculitis. It usually involves the respiratory tract and kidney. Rarely, tumor-resembling inflammatory changes ensue. OBJECTIVES: To report three unique cases of GPA presenting with tumor-like lesions in various organs. METHODS: We presented three cases of GPA. Case 1 presented with typical upper respiratory symptoms of GPA and a mediastinal mass. Case 2 presented with low back pain, a large retroperitoneal mass, and nodular skin lesions. Case 3 presented with epigastric pain and a paravertebral inflammatory mass. RESULTS: The patients were treated successfully with rituximab. CONCLUSIONS: Clinicians should be aware of this presentation of granulomatosis with polyangiitis, which is known as Tumefaction Wegener's granulomatosis.


Assuntos
Granulomatose com Poliangiite , Neoplasias Renais/diagnóstico , Neoplasias do Mediastino/diagnóstico , Neoplasias Retroperitoneais/diagnóstico , Rituximab/administração & dosagem , Adulto , Anticorpos Anticitoplasma de Neutrófilos/sangue , Biomarcadores/sangue , Biópsia/métodos , Diagnóstico Diferencial , Feminino , Granuloma/patologia , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/patologia , Granulomatose com Poliangiite/fisiopatologia , Humanos , Imunossupressores/administração & dosagem , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Mieloblastina/imunologia , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
20.
Adv Anat Pathol ; 28(5): 351-381, 2021 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-34050062

RESUMO

Mesenchymal tumors of the mediastinum are a heterogenous group of rare tumors with divergent lineages. Mediastinal mesenchymal tumors are diagnostically challenging due to their diversity and morphologic overlap with nonmesenchymal lesions arising in the mediastinum. Accurate histologic diagnosis is critical for appropriate patient management and prognostication. Many mediastinal mesenchymal tumors affect distinct age groups or occur at specific mediastinal compartments. Neurogenic tumors, liposarcoma, solitary fibrous tumor, and synovial sarcoma are common mesenchymal tumors in the mediastinum. Herein, we provide an update on the diagnostic approach to mediastinal mesenchymal tumors and a review of the histologic features and differential diagnosis of common benign and malignant mesenchymal tumors of the mediastinum.


Assuntos
Lipossarcoma/patologia , Neoplasias do Mediastino/patologia , Mediastino/patologia , Humanos
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