Evidence Of Parenchymal Disease Research Articles

Adult anaplastic large cell lymphoma involving the central nervous system: a rare clinical scenario

Dear Editor, Anaplastic large cell lymphoma (ALCL) is a rare T-cell non-Hodgkin lymphoma with distinct pathologic features characterized by CD30 expressing large pleomorphic lymphoid cells with or without anaplastic lymphoma kinase (ALK) gene rearrangement. The disease commonly spreads to bone marrow, skin, lung, liver, and, rarely, other sites [1]. ALCL rarely occurs in the central nervous system (CNS), of which most are primary ALCL reported in pediatric or immunocompromised patients [2]. ALCL with secondary CNS involvement is extremely rare [3]. Here, we present two adult patients having ALCL with secondary CNS involvement, both treated with standard chemotherapy and autologous hematopoietic stem cell transplant (auto-SCT), and both patients had a relatively long survival. To date, only three other similar cases have been reported and all had much shorter survival with no auto-SCT treatment. A 27-year-old man who presented with a mediastinal mass and general lymphoadenopathy was subsequently diagnosed with ALK-positive ALCL. The tumor cells expressed CD30 and ALK. He received cyclophosphamide, hydroxydaunorubicin (doxorubicin), Oncovin (vincristine), and prednisone/prednisolone (CHOP) chemotherapy along with prophylactic high-dose intrathecal methotrexate for three cycles. One year after the initial diagnosis of ALCL, the patient developed seizures. Contrast-enhanced brain MRI revealed diffuse edema in both the parietal and occipital regions. There was also evidence of parenchymal disease both above and below the tentorium in addition to diffuse pial enhancement and dural involvement (Fig. 1a and b). Cerebrospinal fluid (CSF) cytospin examination revealed a population of large anaplastic lymphoid cells in the background of few granulocytes and macrophages. Flow cytometry of the CSF identified abnormal T cells expressing CD30 and CD4 with loss of other pan-T-cell markers (CD3, CD5, and CD7). A T-cell gene rearrangement analysis of the CSF DNA sample detected a prominent but dim T-cell receptor gamma monoclonal peak. No circulating lymphoma cells were identified in the peripheral blood at that time point. The patient received intrathecal methotrexate and cytarabine followed by wholebrain radiation. His disease was well controlled although it was complicated with severe encephalopathy and cerebral infarct, which caused peripheral vision loss. Five years later, he developed severe abdominal pain and diarrhea. PET/CT scan revealed hypermetabolic activity in the distal duodenum, and biopsy was consistent with recurrent ALCL X. Zhang Department of Pathology and Cell Biology, University of South Florida College of Medicine, Tampa, FL, USA

59-Year-Old Woman With Progressive Dyspnea on Exertion

© 2004 Mayo Foundation for Medical Education and Research A 59-year-old woman with long-standing seropositive rheumatoid arthritis (RA) presented to the Mayo Clinic in Rochester, Minn, with symptoms of dyspnea on exertion that had worsened gradually during the past year. The patient was previously extremely active but currently had difficulty walking up a flight of stairs. Her dyspnea was persistently present with exertion, and she had noted no other exacerbating or relieving factors. She had episodic wheezing and an infrequent nonproductive cough but no chest pain, pleurisy, orthopnea, or pedal edema. She had a 5-pack-year history of smoking and had quit smoking 30 years previously. Her local physician had treated her empirically with inhaled bronchodilators and inhaled corticosteroids, which were not helpful. She had taken methotrexate for RA diagnosed 20 years previously; however, her arthritis had been quiescent in recent years. The patient was taking no immunosuppressants. She had taken no systemic corticosteroids within the past year and had never taken penicillamine or any other disease-modifying antirheumatic drugs. She had no personal or family history of thrombophilia, pulmonary disease, or cardiac disease. The patient worked as an educator and had not traveled outside the midwestern United States. Examination of the patient’s lungs revealed diffuse expiratory wheezing with no crackles or dullness on percussion. Her extremities showed evidence of chronic symmetrical synovial inflammation in the metacarpophalangeal, proximal interphalangeal, and wrist joints bilaterally with mild fixed deformities compatible with RA. She had no clubbing, cyanosis, elevated jugular venous pressure, or hepatojugular reflux. Findings on examination of the patient’s heart and abdomen were unremarkable, as were findings on the rest of her physical examination. Posteroanterior and lateral chest radiography revealed no masses, infiltrates, effusions, pneumothorax, or evidence of parenchymal disease. 1. Which one of the following would be most helpful in the initial evaluation of our patient? a. Pulmonary function testing b. Echocardiography c. Rheumatoid factor titer d. Bronchoscopy with bronchoalveolar lavage e. Corticotropin-stimulation testing

Imaging

Imaging

Diagnosis and treatment of isolated tuberculous mediastinal lymphadenopathy in adults.

To evaluate the diagnostic yield of bronchoscopy and mediastinoscopy in adults with isolated mediastinal tuberculous lymphadenitis and to assess the effect of antituberculous treatment. Prospective longitudinal cohort study of 34 patients with mediastinal tuberculous lymphadenitis followed for 6 to 19 months after completion of treatment. Tertiary care hospital, Kuwait. 34 consecutive patients who presented with isolated mediastinal lymphadenopathy from 1996 to 1998. Bronchoscopy and cervical mediastinoscopy for all patients. Diagnostic yield of bronchoscopy and mediastinoscopy, and the outcome of treatment in patients with tuberculous lymphadenopathy. The mean age was 35 years (range 15-58). The most common symptoms were cough, fever, and weight loss. The chest radiographs and computed tomograms showed abnormal mediastinal shadows with no evidence of parenchymal disease. All patients had right sided paratracheal lymphadenopathy. Tuberculin skin test gave a weal of >15 mm in 17 patients (50%). Sputum smears and cultures failed to grow acid-fast bacilli in any patient. Seven patients had an endobronchial abnormality and samples taken at bronchoscopy gave a definite diagnosis in 3 (9%). Paratracheal lymph node biopsy and culture by mediastinoscopy diagnosed tuberculosis in all cases. All patients were treated by a six month course of rifampicin and isoniazid supplemented initially by pyrazinamide for two months. Twenty-eight patients had a good response and the remaining patients were treated for a further 3 months. Bronchoscopy has a low diagnostic yield in mediastinal tuberculous lymphadenopathy in the absence of a parenchymal lesion. Mediastinoscopy is a safe but invasive procedure and provides a tissue diagnosis in most cases. Six months treatment with rifampicin and isoniazid supplemented initially by pyrazinamide is adequate treatment for most adults with tuberculous mediastinal lymphadenopathy.

Recognition of Central Nervous System Metastases in Children with Metastatic Primary Extracranial Neuroblastoma

In children with primary extracranial neuroblastoma (NB), intrinsic central nervous system (CNS) metastases (brain parenchyma or leptomeninges) are thought to occur rarely. This study was done to evaluate our anecdotal experience, which suggested that CNS involvement is becoming more frequent. Reports of computed tomographic (CT) and magnetic resonance (MR) imaging scans, biopsies, cerebrospinal fluid (CSF) cytologies, and autopsies were reviewed for children with stage TV NB diagnosed in 1978-1993 and followed at the Children's Hospital of Pittsburgh. Of 43 children over the age of 1 year, CNS metastases were documented in 7 (16.2%). Six patients developed signs or symptoms best explained by the presence of CNS tumor and had radiographic and/or histologic evidence of parenchymal disease (cortical masses on CT and MR, n = 3; suprasellar mass on CT, n = 1; diffuse leptomeningeal carcinomatosis by MR and/or autopsy, n = 2). CSF cytologies were positive in the one patient so tested. An additional asymptomatic patient had extensive CNS involvement at autopsy. In two of these children, the CNS was the first or only site of recurrent disease. It is concluded that intrinsic CNS disease is not uncommon in children with NB over the age of 1 year and there has been a trend toward its increasing recognition in recent years. Whether this is a function of wider use of diagnostic tools or a true change in natural history over time with increased intensity of chemotherapy is not clear. A study that prospectively monitors children with advanced neuroblastoma, radiographically and with CSF cytologies (prior to treatment and at 6-monthly intervals), is under way and should help to better define the natural history in the context of current therapies.

Pulmonary lymphangiomyomatosis: correlation of CT with radiographic and functional findings.

In 14 patients with biopsy-proved lymphangiomyomatosis, disease extent at computed tomography (CT) was correlated with findings at chest radiography and pulmonary-function testing. The CT scans and chest radiographs were read independently by two chest radiologists. Disease extent was assessed on CT scans by using a visual score (0%-100% involvement of the lung parenchyma) and on radiographs by using an adaptation of the International Labour Office classification of the pneumoconioses. There was good concordance between the two observers for CT and radiographic scores (Kendall tau greater than or equal to .86, P less than .01). A significant but relatively low correlation was present between CT findings and radiographic severity of disease (r = .59, P less than .05). Impairment in gas exchange as assessed with the diffusing capacity correlated better with disease extent seen on CT scans (r = .69) than with chest radiographic findings (r = .59). Three patients had evidence of parenchymal disease on the CT scans but not on the radiographs. In one patient CT findings were negative despite a positive finding on chest radiographs. The authors conclude that CT is superior to chest radiography in the assessment of patients with lymphangiomyomatosis.

Persistence of Mycobacterium tuberculosis in sputum without chest roentgenographic evidence of active disease.

Mycobacterium tuberculosis has been isolated consistently from sputum over an 11-year period in an asymptomatic 64-year-old man whose chest radiographs show evidence of healed primary tuberculosis (Ranke complex) but no evidence of parenchymal disease. A lymph node-bronchial fistula associated with the Ranke complex is the likely site of active tuberculosis; however, repeated examinations of the upper and lower respiratory tract have been normal. We attribute the persistence of infection in our patient to erratic usage of antituberculous drugs.

Report of A Case With Lung Abscess, Simulating Carcinoma of the Lung, Diagnosed on Bronchoscopy

A 66 years old female with a chief complaint of cough and sputum with hemoptysis was noted to have an enlarging hilar mass in her right lung, similar to a carcinoma, without any evidence of parenchymal disease. At bronchoscopy the right bronchial mucosa was noted to be highly vascular, edematous with localized white tenecious secretion.The bronchial lumen was distorted and stenosed but without evidence of malignancy. Although highly vascular, the mucosa was not friable and throughout the examination there was no bleeding.Multiple cultures were non specific. A mucosal biopsy and smear showed acute and chronic inflammatory changes.The patient was started on vigorous antibiotic therapy.Radiographic follow up over a seven month period showed slow progressi ve diminution in the size of the mass with marked clinical improvement. This therefore represents a case of a malignant appearing hilar mass which was caused by an acute and chronic inflammatory process.