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

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

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.
Antibodies, Monoclonal, Murine-Derived ; Central Nervous System ; Clinical Protocols ; Herpesvirus 4, Human ; Kidney ; Lung ; Lymphomatoid Granulomatosis ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prognosis ; Skin ; Spine ; Rituximab

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.
Adult ; Biopsy, Needle ; Fluorodeoxyglucose F18/*diagnostic use ; Humans ; Lung Neoplasms/pathology/*radiography/*radionuclide imaging ; Lymphomatoid Granulomatosis/pathology/*radiography/*radionuclide imaging ; Male ; Middle Aged ; *Positron-Emission Tomography ; Radiopharmaceuticals/*diagnostic use ; *Tomography, X-Ray Computed

Adolescent ; Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cisplatin ; administration & dosage ; Dexamethasone ; administration & dosage ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Lymphoma, Extranodal NK-T-Cell ; pathology ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; pathology ; Lymphoma, T-Cell, Cutaneous ; drug therapy ; pathology ; Lymphomatoid Granulomatosis ; pathology ; Male ; Middle Aged ; Natural Killer T-Cells ; pathology ; Neoplasm Recurrence, Local ; Skin Neoplasms ; drug therapy ; pathology ; Young Adult

OBJECTIVETo study the immunophenotype and gene rearrangement pattern of pulmonary lymphomatoid granulomatosis.

METHODSNine 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.

RESULTSThe 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.

CONCLUSIONSPulmonary 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.

Adult ; Antigens, CD20 ; metabolism ; CD3 Complex ; metabolism ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Gene Rearrangement, B-Lymphocyte, Heavy Chain ; Humans ; Immunohistochemistry ; Lung Neoplasms ; genetics ; metabolism ; pathology ; surgery ; Lymphomatoid Granulomatosis ; genetics ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Neoplasm Grading ; Pneumonectomy ; methods ; Retrospective Studies ; Young Adult

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

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

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

Adrenal Cortex Hormones ; therapeutic use ; Antiviral Agents ; therapeutic use ; Diagnosis, Differential ; Granulomatosis with Polyangiitis ; diagnosis ; Humans ; Lung Neoplasms ; diagnostic imaging ; drug therapy ; pathology ; Lymphomatoid Granulomatosis ; diagnostic imaging ; drug therapy ; pathology ; Prognosis ; Radiography ; Sarcoidosis ; diagnosis ; Tuberculosis, Pulmonary ; diagnosis