Low Grade Pulmonary Lymphomatoid Granulomatosis with an Endobronchial Mass / 결핵및호흡기질환

Lymphomatoid granulomatosis (LYG) is an angiocentric and angiodestructive neoplastic proliferation of B and T lymphocytes commonly involving the lungs. Epstein-Barr virus is commonly detected in lesional cells. We report a case of a 54-year-old female with underlying monoclonal gammopathy of unknown significance who presented with a 4 week history of dyspnea and cough. Computed tomography scan of the chest showed multiple lung nodules as well as endobronchial narrowing causing atelectasis at the left upper lobe. Bronchoscopic findings revealed obstruction at the lingula segment due to endobronchial mass as a rare presentation. Bronchoscopic biopsy was diagnosed with LYG grade 1. After treatment, the endobronchial mass and lung lesions were completely resolved. However, the patient eventually evolved to malignant lymphoma after 1 year.

Analysis of (18)F-FDG PET/CT features and clinical manifestations in one case of subcutaneous lymphomatoid granulomatosis / 中国实验血液学杂志

This study was aimed to investigate the pathology, MICM classification, PET/CT characteristics and therapeutical experience of subcutaneous soft tissue muscle gap lymphomatoid granulomatosis (LYG) through analysis of a cases of LYG. The pathologic changes of LYG were assayed by using immunohistochemistry method;the immuno-phenotypes were detected by flow cytometry. The nested multiplex PCR was used to detect the expression and mutation of abnormal genes; the real-time fluorescence quantitative PCR was used to detect the EBV-DNA copies. The clinical staging was performed by means of fluorodeoxyglucose positron emission tomography/computed tomography ((18)F-FDG PET/CT). The results showed that at onset of disease the clinical manifestations of patient presented only a mass in right thigh and swelling of right submandibular lymph nodes. However, PET/CT revealed that the abnormal image in multiple soft tissue accompanied by increasing metabolic activity (SUVmax = 12.8), these pathologic changes were involved in lung, thyroid, lymphonodes and stomach. The right thigh mass biopsy confirmed the histological diagnosis of grade II LYG. The bone marrow smear showed no abnormal tumor cell infiltration, the immunophenotyping detection revealed that the proportion of NK cells increased with phenotypic abnormality, the karyotype was 46, XY[24], the expression and mutation of abnormal gene not could be detected, and the EBV-DNA level was <10(2) copies/ml. After 2 cycles of treatment with rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone(R-CHOP), the images of increasing metabolic activity in subcutaneous soft tissue gap disappeared, but the partial increasing metabolism focus could be observed in soft tissue of left knee hollow. The patient achieved partial remission. It is concluded that LYG is an extremely rare hematopoietic malignancy, the incidence rate is very low. Subcutaneous soft tissue muscle gap LYG literature was not reported in domestic and foreign literatures.Its pathogenetic remains unclear. A standard treatment protocol for LYG has not yet been established. PET/CT can find more lesions that not could be found in the clinical examination. The (18)F-FDG PET/CT is an efficient tool for the LYG in diagnosis, staging and treatment. Therefore, increased SUV(max) in FDG-PET may be useful for diagnosis of LYG.

Lymphomatoid Granulomatosis with Spinal Involvement after Childhood Acute Lymphoblastic Leukemia

Lymphomatoid granulomatosis (LYG) is a lymphoproliferative disease involving the lungs most frequently; however, it may also involve the kidneys, skin and especially the central nervous system. Unique initial presentation of spinal involvement is extremely rare and epidural lesion of thoracic spine has not been reported. The prognosis for LYG has been reported to be poor, and there currently exists no satisfactory established treatment protocol. The purpose of this study is to report a case of successful treatment with surgery and rituximab combination therapy in thoracic spinal LYG.

Granulomatosis linfomatoide cerebral primaria en paciente VIH positivo: Caso clínico / Primary cerebral lymphomatoid granulomatosis in a HIV-positive patient: Case report

We report a 34-years-old male, with a history of hepatitis B and human immunodeficiency virus (HIV) infection that was admitted to the hospital with malaise, weight loss, frontal behavior and chest pain. Imaging studies showed two frontal cortical/subcortical nodules. A stereotactic cerebral biopsy showed reactive gliosis and a prominent atypical angiocentric and angiodestructive lymphoid infiltrate containing large pleomorphic CD20 and EBV-positive cells consistent with Lymphomatoid granulomatosis. Other studies were negative. The patient was lost from follow up.

Lymphomatoid Granulomatosis: CT and FDG-PET Findings

OBJECTIVE: Lymphomatoid granulomatosis (LG) is a rare, aggressive extranodal Epstein-Barr virus (EBV)-positive B-cell lymphoproliferative disease. The purpose of our study was to analyze the CT and fluorodeoxyglucose positron emission tomography (FDG-PET) findings of pulmonary LG. MATERIALS AND METHODS: Between 2000 and 2009, four patients with pathologically proven pulmonary LG and chest CT were identified. Two of these patients also had FDG-PET. Imaging features of LG on CT and PET were reviewed. RESULTS: Pulmonary nodules or masses with peribronchovascular, subpleural, and lower lung zonal preponderance were present in all patients. Central low attenuation (4 of 4 patients), ground-glass halo (3 of 4 patients), and peripheral enhancement (4 of 4 patients) were observed in these nodules and masses. An air-bronchogram and cavitation were seen in three of four patients. FDG-PET scans demonstrated avid FDG uptake in the pulmonary nodules and masses. CONCLUSION: Pulmonary LG presents with nodules and masses with a lymphatic distribution, as would be expected for a lymphoproliferative disease. However, central low attenuation, ground-glass halo and peripheral enhancement of the nodules/masses are likely related to the angioinvasive nature of this disease. Peripheral enhancement and ground-glass halo, in particular, are valuable characteristic not previously reported that can help radiologists suggest the diagnosis of pulmonary LG.

Pulmonary lymphomatoid granulomatosis: an immunohistochemical and gene rearrangement study / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the immunophenotype and gene rearrangement pattern of pulmonary lymphomatoid granulomatosis.</p><p><b>METHODS</b>Nine cases of pulmonary lymphomatoid granulomatosis, included 5 cases of open lung biopsy, 3 cases of lobectomy specimen and 1 case of autopsy, were retrospectively analyzed by immunohistochemistry, in-situ hybridization for Epstein-Barr virus-encoded RNA, immunoglobulin and T-cell receptor gene rearrangement studies.</p><p><b>RESULTS</b>The age of patients ranged from 3 to 59 years. The male-to-female ratio was 3: 6. Histologically, all cases showed lymphocytic infiltration surrounding the blood vessels and in the perivascular areas. Most of these lymphoid cells expressed T-cell marker CD3. There were also variable numbers of CD20-positive B cells. The staining for CD56 was negative. According to the WHO classification, there were 4 cases of grade I , 1 case of grade II and 4 cases of grade III lesions. Six cases had gene rearrangement studies performed and 3 of them demonstrated clonal immunoglobulin gene rearrangement (including 1 of the grade II and 2 of the grade III lesions). No T-cell receptor gene rearrangement was detected.</p><p><b>CONCLUSIONS</b>Pulmonary lymphomatoid granulomatosis may represent a heterogeneous group of lymphoproliferative disorders. Some of the cases show B-cell immunophenotype and clonal immunoglobulin gene rearrangement, especially the grade II and grade lesions. They are likely of lymphomatous nature.</p>

Lymphomatoid granulomatosis with splenomegaly and pancytopenia / 中国肺癌杂志

Lymphomatoid granulomatosis (LG) is an angiocentric lymphoproliferative disease. It usually involves lung, skin, and central nervous system, but splenomegaly and pancytopenia are the rare manifestations of the disease. We report a 15-year-old boy presented with fever, dry cough and dyspnea from two months ago, after admission patient had nodular lesions on the left leg and hepatosplenomegaly. Then he manifested neurologic signs such as seizure, aphasia and right-sided hemiplegia. Chest X-ray and CT scan revealed bilateral pulmonary nodules predominantly in lower lobes and peripheral lung fields. Laboratory exams showed pancytopenia. Skin biopsy was done, and histopathological examination and immunohistochemistry evaluation confirmed lymphomatoid granulomatosis. He was treated with steroid and cyclophosphamide but succumbed by neurologic involvement.

Granulomatose linfomatóide-relato de caso / Lymphomatoid granulomatosis -case report

Objetivo: descrever um caso com manifestaçõs clínicas e achados histopatológicos de Granulomatose Linromatóide (GL), uma rara doença linfoproliferativa das células B, angiocêntrica e angiodestrutiva associada ao vírus Epstein-Barr (EBV). A apresentação clínica mais comum é no pulmão, podendo acometer qualquer órgão, sendo os achados extra-pulmonares as principais manifestações nestes casos. Relato do caso: mulher de 65, com lesão cutânea vulvar e que evoluiu com quadro neurológico associado a lesão expansiva envolvendo parênquima cerebelar, sem alterações pulmonares. Considerações Finais: pelo seu prognóstico e capacidade de ser confundida com outras vasculites a GL impõe-se como um importante diagnóstico diferencial dessas doenças.

lntroduction: this report describe a case of Lymphomatoid Granulomatosis (LG,s a rare linfoproliferative disorder of B cell showing angiocentric growth and destructive behaviour caused by Epstain-Barr virus (EBV). The predominant clinical presentations are confined to the pumonary system, however ; extra-pumonary manifestations can sometimes be the main feature of the dieseses. Method: here we report a 65 year old female, with a skin vulvar lesion, and another in cerebelus, without pulmonary disease. Conclusion: the LG can mimics systemic vasculits and is a diagnostic challenge.

Central Nervous System Lymphomatoid Granulomatosis Presenting with Parkinsonism

Lymphomatoid granulomatosis (LG) is a potentially malignant lymphoproliferative disorder. The lung is the most common involved site, followed by the skin and nervous system. However, LG of the central nervous system presenting with Parkinsonism is very rare. We report a patient with LG who presented with parkinsonian features such as bilateral rigidity, bradykinesia, and agitation. Brain magnetic resonance imaging showed multifocal punctuate enhanced lesions in both supra- and infratentorial areas. Steroid pulse therapy resulted in a dramatical improvement in the symptoms and MRI abnormalities.

Proliferaciones linforreticulares del pulmón / Pulmonary lymphoproliferative lesions

The present review describes the current classification of the pulmonary lymphoproliferative lesions as proposed by the WHO in 2004 with emphasis in the clinical picture and histopathological features. The definition of these entities includes the clinical picture, histopathology, immunohistochemistry and molecular features. The differential diagnosis of the most important entities is also briefly discussed

En el presente trabajo de revisión se describe la clasificación actual de las lesiones linfoproliferativas del pulmón propuesta por la OMS el año 2004 con énfasis en el cuadro clínico y los aspectos histopatológicos. La definición de estas entidades incluye cuadro clínico, histopatología, inmunohistoquímica y características moleculares. Se discute brevemente el diagnóstico diferencial de las formas más importantes

Granulomatosis limgomatoidea: manifestaciones pulmonares de una entidad infrecuente / Limgomatoidea granulomatosis: manifestations of a rare lung

La Granulomatosis Linfomatoidea (GL) es una enfermedad linfoproliferativa poco frecuente caracterizada por afectar sitios extraganglionares y tener gran afinidad por los vasos sanguíneos (“linfoma angiocéntrico”). El órgano más frecuentemente afectado es el pulmón, siendo las manifestaciones radiográficas más frecuentes los nódulos pulmonares múltiples (80%) e infiltrados reticulonodulillares difusos. Presentamos el caso de un paciente masculino de 77 años de edad, con múltiples imágenes nodulares cavitadas bilaterales evidenciadas en radiografía convencional de tórax (RX) y tomografía computada (TC), con mala evolución clínica y diagnóstico por autopsia de GL. La afectación principalmente pulmonar, en un contexto clínico apropiado junto a sus manifestaciones radiológicas y topográficas, nos permiten sospechar, diagnosticar y tratar precozmente esta entidad poco frecuente y con una sobrevida media de 2 años.

Lesión del surco pulmonar superior: a propósito de un caso interesante / Lesion of the superior lung sulcus: apropos of an interesting case

Los tumores del surco pulmonar superior representan un porcentaje no despreciable entre los tumores del pulmón. A su vez, los tumores malignos entre ellos constituyen el 90 por ciento , mientras en el 10 por ciento restante, constituido por tumores benignos, procesos inflamatorios o infecciosos del vértice pulmonar, se destacan las enfermedades granulomatosas. Existe dificultad en el diagnóstico clínico precoz de un síndrome de Pancoast por la aparición tardía de los síntomas característicos de esta enfermedad. Se presenta un caso con manifestaciones clínicas que enfocaron los estudios iniciales y la clínica hacia la búsqueda de una enfermedad maligna pulmonar. El proceder diagnóstico fue la toracotomía y resultó tratarse de una enfermedad granulomatosa del pulmón(AU)

The tumors of the furrow lung superior represent a non worthless percentage among the tumors of the lung. In turn, the wicked tumors among them constitute the 90 percent, while in the 10 percent remaining, constituted by benign tumors, inflammatory or infectious processes of the lung vertex, stand out the illnesses granulomatosas. Difficulty exists in the precocious clinical diagnosis of a syndrome of Pancoast for the late appearance of the characteristic symptoms of this illness. A case is presented with clinical manifestations that focused the initial studies and the clinic toward the search of a lung wicked illness. Proceeding diagnosis was the toracotomía and it turned out to be an illness granulomatosa of the lung(AU)

Arthritis in lymphomatoid granulomatosis: report of a case and review of literature.

Lymphomatoid granulomatosis (LG) is a rare systemic vasculitis caused by Epstein Barr virus induced transformation of the B-cells in a T-cell rich environment. The predominant clinical presentations are confined to the pulmonary system however; extra-pulmonary manifestations can sometimes be the main feature of the disease. Here in we describe a 52-year-old female who presented with symmetric polyarthritis and generalized stiffness for 7 months and papular lesions over extremities for 3 months duration. She in addition had generalized lymphadenopathy. Histopathological examination of the cutaneous lesions confirmed LG. Patient died despite therapy with cyclophosphamide and prednisolone. This is the first report of LG mimicking rheumatoid arthritis from India.

Linfoma angiocéntrico mediofacial simulando granulomatosis de Wegener limitada. Reporte de un caso / Midfacial angiocentric lymphoma simulting Wegener granulomatosis. A case report

Se reporta el caso de un paciente masculino de 21 años con lesiones granulomatosas en nariz y órbitas, quien tuvo síndrome febríl y pérdida de peso. Se sospechó el diagnóstico de Granulomatosis de Wegener limitada con base en el cuadro clínico y en los hallazgos de la biopsia. Sin embargo, por inmunotinción el diagnóstico definitivo fue de linfoma angiocéntrico mediofacial.

Lymphomatoid granu;lomatosis with long survival

We present a case of lymphomatoid granulomatosis presenting in a 35-year-old Saudi lady with long survival. She responded to treatment with intermittent cyclophosphamide infusion in addition to corticosteroids. This is the first case of lymphomatoid granulomatosis to be reported in the Arab world. The prolonged survival and response to intermittent cyclophosphamide infusion is discussed

A Case of Pulomonary Lymphomatold Granulomatosis / 대한내과학회지

Lymphomatoid granulomatosis was described by Liebow as a peculiar angiocentric and angiodestructive lymphoreticular proliferative granulomatous disease. Although the lung is most frequently involved, lymphomatoid granulomatosis is also commonly found in the upper respiratory tract, skin, kidneys and central nervous system. Pulmonary infiltrates are histologically polymorphous and show variable degree of cytologic atypic in the lymphoid cells. Infrequent involvement of the bone marrow, spleen and peripheral lymph nodes initially supported the distinction of lymphomatoid granulomatasis from lymphoma. We experienced a rare case of 26-year-old female diagnosed as lymphomatoid granulomatosis by per cutaneous needle aspiration biopsy and transbronchial lung biopsy with symptoms of fever, cough, sputum and right chest pain. After 4th chemotherapy it showed partial remission and chemotherapy is going on.

Esptein-Barr Virus Associated Cutaneous Angiocetric Immunoproliferative Lesion Showing Histologic Features of Classical Lymphomatoid Granulomatosis

We describe a patient with cutaneous angiocentric immunoproliferative lesion (AIL) associated with the Epstein-Barr virus (EBV). An organ system survey revealed no evidence of internal involvement. A skin biopsy specimen .showed infiltrating cells involving mainly deeper dermis and subcutaneous tissue. An examination of the reticular dermis revealed polymorphous angiocentric and angioinvasive infiltrate containing some atypical lymphocytes and histiocytes. EBV encoded RNA (EBER) was demonstrated in lesional skin by the in situ hybridization technique. On the basis of these findings, we conclude that our case may represent a form of AIL associated with EBV showing histologic features of classical lymphomatoid granulomatosis.

A Case of Angiocentric Lymphoma

Angiocentric lymphoma is a lymphoma like lesion which has been known as a lethal midline granuloma, lymphomatoid granulomatosis and polymorphic reticulosis. It involves predominantly lungs, and other extranodal sites such as upper respiratory tract, skin, kidneys, peripheral nervous system and CNS. The authors experienced a case of angiocentric lymphoma which was misdiagnosed as orbital cellulitis because there were persistent epiphora, erythematous swelling in dacryocyst area and diffuse edema in upper and lower eyelids which occurred after functional endoscopic sinus surgery. This case was diagnosised as angiocentric lymphoma through multiple tissue biopsies. This disease is rare, especially in orbit and eyelid. It leads to the destruction and inflammation of tissue. To avoid misdiagnosis to orbital cellulitis, accurate biopsy is needed with the interchange of clinical informations with pathologist. We report this case with the review of literatures.