ABSTRACT
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive lymphoma with an overall incidence of 0.04 cases per 100,000 people. BPDCN is a hematopoietic clonal neoplasm that originates from plasmacytoid dendritic cell precursors. A 63-year-old man presented with multiple erythematous nodules over his whole body, including his face, trunk, and both upper and lower extremities that appeared 1 month ago. Skin biopsy showed diffuse dermal infiltration by monomorphic atypical lymphocytes with large, irregular nuclei and scant cytoplasms. Immunohistochemical staining was positive for CD4, CD56, and CD123. The karyotype test showed abnormalities in male chromosomes 47, XY, ＋8 [2]/46, and XY [25], and mutations in DNMT3A, TET2, SRSF2, and ATRX genes were identified in a next-generation sequencing (NGS)-based acute myeloid leukemia gene panel test. The patient was diagnosed with BPDCN and treated with a KALLA 1406 regimen; however, he died on the 17th day of treatment.
ABSTRACT
Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare neoplasm that is frequently located in the distal extremities. Emerging evidence suggests that MIFS can also affect the proximal limbs, trunk, and scalp, and aggressive clinical courses have been noted. We report a case of MIFS that occurred suddenly in the patient’s forearm and grew rapidly within 2 weeks. A level of Ki-67 was observed in the patient’s lesion, which constitutes a considerable finding compared with most MIFS cases. The patient underwent surgical tumor removal, and no evidence of recurrence was noted. We highlight this case in view of its sudden occurrence and rapid local progression, which contradicts the usual features of this disease, suggesting that this clinical course might be attributable to the high Ki-67 value.