Pulmonary Mucormycosis Complicating Cutaneous Blastic Plasmacytoid Dendritic Cell Neoplasm

Abstract

A 64-year-old man presented for outpatient evaluation of multiple cutaneous skin lesions that began as erythematous macules approximately 1 year earlier and progressed to areas of brownish hyperpigmentation. Review of systems was significant for fever, night sweats, fatigue, and epistaxis. Physical examination demonstrated bilateral inguinal lymphadenopathy. Bone marrow and lymph node biopsies were subsequently performed and demonstrated evidence of blastic plasmacytoid dendritic cell neoplasm. Baseline 18-fluoro-2-deoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) showed FDG uptake within enlarged left iliac and bilateral inguinal lymph nodes, as well as moderate splenomegaly. The patient received one cycle of the EPOCH regimen (etoposide, vincristine, doxorubicin, cyclophosphamide, and prednisone) and three cycles of hyperCVAD (course A, cyclophosphamide, vincristine, doxorubicin, and dexamethasone; course B, methotrexate and cytarabine). He initially tolerated chemotherapy without evidence of toxicity or opportunistic infection. A posteroanterior chest radiograph obtained following treatment demonstrated an irregular opacity in the left upper lung zone (Figure ​(Figure11). Subsequent contrast-enhanced chest CT showed an irregular opacity in the anterior segment of the left upper lobe consisting of central ground-glass opacity surrounded by consolidation in a “reversed halo” configuration (Figure ​(Figure22). Follow-up PET/CT showed intense FDG uptake within the region of consolidation, but only minimal FDG uptake within the central ground-glass opacity (Figure ​(Figure33). Figure 1 Posteroanterior chest radiograph demonstrates an irregular opacity within the left upper lung zone. Figure 2 Axial CT images on (a) lung and (b) soft tissue windows show an area of central ground-glass opacity surrounded by consolidation, also known as the reversed halo sign, in the anterior segment of the left upper lobe. Figure 3 Axial PET/CT images on (a) lung and (b) soft tissue windows demonstrate intense FDG uptake within the region of consolidation, but only minimal FDG uptake within the central ground-glass opacity. Bronchoscopy and bronchoalveolar lavage demonstrated one colony of fungal organisms from the genus Paecilomyces, and a regimen of Augmentin and voriconazole was initiated. A left upper lobectomy through video-assisted thoracoscopic surgery was subsequently performed and showed fungal organisms of the order Mucorales. Augmentin was discontinued, and antifungal therapy was modified to amphotericin B and posaconazole. DIAGNOSIS: Pulmonary mucormycosis.