No abstract available.
Lymphomatoid Granulomatosis*

We report a case of cerebral lymphomatoid granulomatosis in a 50-year-old man. Lymphomatoid granulomatosis is an unusual condition which usually presents as a pulmonary manifestation, which occasionally affects the brain, and causes focal inflammatory lesions. Primary cerebral lymphomatoid granulomatosis is very rare. We describe a case of lymphomatoid granulomatosis in the left frontal lobe without pulmonary involvement.
Brain ; Frontal Lobe* ; Humans ; Lymphomatoid Granulomatosis* ; Middle Aged

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.
Biopsy ; Dermis ; Herpesvirus 4, Human ; Histiocytes ; Humans ; In Situ Hybridization ; Lymphocytes ; Lymphomatoid Granulomatosis* ; RNA ; Skin ; Subcutaneous Tissue

Adult ; Brain Neoplasms ; diagnosis ; secondary ; Female ; Humans ; Lymphomatoid Granulomatosis ; complications ; diagnosis ; Male ; Young Adult

Lymphomatoid granulomatosis usually presents as a primary lung affliction with secondary metastatic spread to the central nervous system(CNS), and its initial manifestation purely as a CNS disease is rare. A 57-year-old man with histologically proven lymphomatoid granulomatosis of the brain as the sole manifestation of the disease is presented.
Brain Neoplasms/*pathology ; Frontal Lobe ; Humans ; Lymphomatoid Granulomatosis/*pathology ; Male ; Middle Aged

We report a case of Lymphomatoid granulomatosis in a 54-year-old woman. She presented with a two months history of a tender nodule located on the left for earm associated with fever and pulmonary symptoma aggravated against various treatments. She died 92 days of admission because of respiratory failure. Diagnosis was based on the clinical features and histopathologic findings of the skin lesion.
Diagnosis ; Female ; Fever ; Humans ; Lymphomatoid Granulomatosis* ; Middle Aged ; Respiratory Insufficiency ; Skin

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.
Biopsy ; Cough ; Dyspnea ; Female ; Herpesvirus 4, Human ; Humans ; Lung ; Lymphoma ; Lymphomatoid Granulomatosis* ; Middle Aged ; Paraproteinemias ; Pulmonary Atelectasis ; T-Lymphocytes ; Thorax

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.
Brain ; Central Nervous System* ; Dihydroergotamine ; Humans ; Hypokinesia ; Lung ; Lymphomatoid Granulomatosis* ; Lymphoproliferative Disorders ; Magnetic Resonance Imaging ; Nervous System ; Parkinsonian Disorders* ; Skin

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.
Adolescent ; Humans ; Lymphomatoid Granulomatosis ; diagnosis ; diagnostic imaging ; drug therapy ; Male ; Pancytopenia ; diagnosis ; diagnostic imaging ; drug therapy ; Radiography ; Splenomegaly ; diagnosis ; diagnostic imaging ; drug therapy

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.
Adult ; Fluorodeoxyglucose F18 ; Humans ; Lymphomatoid Granulomatosis ; diagnostic imaging ; pathology ; Male ; Positron-Emission Tomography ; Soft Tissue Neoplasms ; diagnostic imaging ; pathology ; Tomography, X-Ray Computed