2021년 제72차 대한내과학회 추계학술대회 563
￭S-523 ￭ Multiple cutaneous histiocytic sarcoma: a case series
연세대학교 의과대학 내과학교실
이동욱, 김원식, 오지은, 김유리
Histiocytic sarcoma is an extremely rare hematologic malignancy with various clinical features. Clinical manifestation of histiocytic sarcoma varies depending on involving organs, such as intestinal tract, skin, and soft tissues, although they have no definite pattern.
Histiocytic sarcoma is diagnosed based on the pathologic evaluation including immunohistochemical staining, because a distinction between histiocytic sarcoma and other hematological disorders is demanded. Here, we describe a 78-year male patient, presenting multiple skin nodule (figure 1). Histiocytic sarcoma was confirmed with skin biopsy. The patient was started with CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy, and the treatment is ongoing. We reviewed 8 cases of histiocytic sarcoma, diagnosed and treated in Severance Hospital from 2007 to 2021 (table 1). Half of cases were unifocal diseases, which were treated with complete excision alone or with adjuvant radiotherapy. Patients with unifocal disease showed long term survival over 50 months following excision. The other half cases with multifocal disease, those patients were treated with chemotherapy like CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone). These patients showed inferior survival compared to the patients with unifocal disease. Although there are only a small number of patients, this case series showed that clinical outcome is affected by the disease extent. As histiocytic sarcoma is known to have poor prognosis, the early suspicion and pathological confirmation seems to be important for improving outcome. It is important to for multifocal diseases to start rapid chemotherapy, and more data is needed to evaluate proper chemotherapeutic regimen.
