Pathologic Quiz Case: A 17-Year-Old Adolescent Girl With a Short History of Dyspnea

Abstract

Our patient was a 17-year-old, adolescent white girl who had been in good health when she developed progressive dyspnea during a period of 2 weeks. She denied any history of fever, weight loss, drenching night sweats, and other unusual symptoms.The patient underwent a chest x-ray examination, which showed increased density in the left lung hilum with a volume loss. Computed tomography (Figure 1) revealed a 3- to 4-cm mass, occluding the left main bronchus, with near-complete left lung consolidation and atelectasis. Bronchoscopy with bronchial wash, brushings, and a biopsy of the mass were performed. Microscopic examination of the biopsy specimen revealed several fragments of bronchial tissue with an ulcerated mucosa and an infiltrating, partially necrotic submucosal neoplasm. The neoplasm consisted of noncohesive large cells generally with one nucleus, slightly lobated in some cells, that possessed basophilic nucleoli and eosinophilic cytoplasm (Figure 2). For further evaluation, immunohistochemical staining, consisting of keratin, chromogranin, synaptophysin, CD3, CD5, CD10, CD15, CD20, CD30, CD45, CD45Ro, and epithelial membrane antigen (EMA), was performed. The tumor was positive for CD30 (Figure 3), CD45, EMA, CD5, and CD45Ro. The neoplastic cells also expressed anaplastic large cell lymphoma kinase protein.What is your diagnosis?Primary anaplastic large cell lymphomas commonly present as a nodal disease; the extranodal pattern of presentation is seen less frequently.12 The skin is the most common site of involvement among the extranodal lymphomas.1–3 The peak incidence is in childhood. Primary anaplastic large cell lymphomas that arise from extranodal sites other than skin are distinctively rare and have not received much attention in the literature. These sites include bone,4 liver,3 spleen,3 kidney,5 lung and pleura,1–3 and the alimentary tract.67 Endobronchial presentation of anaplastic large cell lymphoma is rare.Ki-1 (CD30+) anaplastic large cell lymphoma was first described by Stein et al8 as a tumor in which all or nearly all neoplastic cells express the CD30 antigen. These lymphomas have a preferential paracortical involvement of lymph nodes with foci of necrosis and intrasinusoidal dissemination.9 Anaplastic large cell lymphomas have been clinically subdivided into primary form (de novo) and secondary form (anaplastic transformation from another lymphoma).10 The most common primary site is the lymph node. These lymphomas are characterized by large tumor cells that have round or indented nuclei with distinct eosinophilic nucleoli and abundant basophilic cytoplasm and that involve sinuses and paracortical areas of lymph nodes, showing a free cell growth pattern or a sheetlike appearance.11 Because of the morphologic heterogeneity, these lymphomas have previously been diagnosed as malignant histiocytosis, Hodgkin disease, and nonhematopoietic tumors.12 Immunocytochemical staining is helpful in distinguishing anaplastic large cell lymphoma from other disorders that mimic it. These lymphomas are positive for CD30, CD45, EMA, and BNH9. Most anaplastic large cell lymphomas express one or more T-cell antigens, CD2 and CD4 are expressed in a significant proportion of cases, and CD43 is expressed in two thirds of the cases.Approximately 90% of anaplastic large cell lymphomas show clonal rearrangement of the T-cell receptor gene. Most are positive for anaplastic large cell lymphoma kinase protein. This expression is due to genetic alteration of the anaplastic large cell lymphoma kinase locus on chromosome 2. The most frequent translocation is t (2:5)(p23:35).These lymphomas, if treated with chemotherapy, have a favorable prognosis.10 According to a study performed by Tilly et al,12 75% of the patients obtained complete remission following chemotherapy, with a 5-year survival rate of 66%.