Extramedullary granulocytic sarcoma of the skin, mediastinum, and pericardium

Abstract

A 27-year-old man, with a past history of developmental delay, presented on 18 November 2005 for the evaluation of an acute onset of multiple red–violaceous nodules on the head, neck, and trunk of 5 days’ duration. The patient had no associated fever, chills, weight loss, night sweats, chest pain, dyspnea, lymphadenopathy, or organomegaly. He had no previous history of malignancies. A biopsy indicated a diagnosis of leukemia cutis (Fig. 1). His initial complete blood count (CBC) was within normal limits. The 2-week follow-up revealed enlargement of the previous lesions and the development of new lesions (Fig. 2). By the third week, the patient had developed dyspnea, but with normal breath sounds and oxygen saturation. Chest computed tomography demonstrated a mediastinal mass measuring 16 × 5.2 cm and pericardial thickening (Fig. 3). The diagnosis of granulocytic sarcoma of the skin lesion and mediastinal mass was established on the basis of immunohistochemical stains, with positivity to CD43 and Leder's preparation and negativity to CD3, CD4, CD5, CD8, CD10, CD20, CD23, CD30, CD34, CD56, bcl-1, terminal deoxynucleotidyl transferase (TdT), and granzyme. The bone marrow was negative for malignant cells. CBC and chemistry panel were all normal. Nevertheless, the patient experienced increased dyspnea and developed a pericardial effusion which required a pericardial window. Cytology of the pericardial fluid was consistent with granulocytic sarcoma. Once the diagnosis of granulocytic sarcoma was established, the patient started a regimen of cytarabine, daunorubicin, and etoposide. Despite this, the skin lesions and mediastinal mass showed minimal response. Repeat computed tomography showed a mediastinal mass measuring 14.5 × 4.4 cm. The patient's respiratory status required intubation and, 2 weeks later, his family requested that he be withdrawn from life support. Figure 1Open in figure viewerPowerPoint Immature myelocytic infiltrate in the dermis (hematoxylin and eosin, ×4) Figure 2Open in figure viewerPowerPoint Clinical image of granulocytic sarcoma Figure 3Open in figure viewerPowerPoint Computed tomography of the chest illustrating mediastinal pericardial involvement