Indeterminate dendritic cell neoplasm of the skin: A 2-case report and review of the literature.

Indeterminate dendritic cell neoplasm (IDCN) is an exceedingly rare and mostly cutaneous histiocytosis, frequently associated with other hematopoietic malignancies. We report 2 cases of multilesional cutaneous IDCN. A 55-year-old male with no associated malignancy and complete response to ultraviolet phototherapy; and a 72-year-old male with chronic myelomonocytic leukemia (CMML). Both cases showed histiocytoid cytology, positivity for CD1a and no expression of langerin or BRAFV600E . With our patients, the literature describes 79 cases of IDCNs, including 65 (82%) with only skin involvement, 7 cases (9%) with involvement of skin and a second site, 5 cases (6%) involving lymph nodes only, 1 splenic lesion and 1 systemic disease. Seventeen cases (22%) were associated with other hematopoietic malignancies, most commonly CMML (6 cases), follicular lymphoma (4 cases) and acute myeloid leukemia (3 cases). All IDCNs associated with myeloid malignancies were limited to the skin, while most cases associated with lymphoma were limited to lymph nodes. Reported responses of cutaneous lesions to ultraviolet phototherapy are encouraging, while systemic chemotherapy is appropriate for clinically aggressive cases and treatment of associated malignancies. Recognition of the clinico-morphologic spectrum of IDCNs should prevent misdiagnoses and prompt investigation of possible associated neoplasms.

The role of maintenance phototherapy in cutaneous T-cell lymphoma.

Phototherapy is well-recognized as effective therapy in early stage cutaneous T-cell lymphoma (patch and plaque), often resulting in complete clearance of clinical disease and subsequent remission. Although not curable, long-term remission can often be attained utilizing maintenance phototherapy, consisting of a course of less frequent treatments over time. Herein, the authors review the literature regarding the role of maintenance phototherapy in cutaneous T-cell lymphoma (CTCL) and its success in prolonging clinical remission and disease-free survival in CTCL.

The diagnosis, staging, and treatment options for mycosis fungoides.

BACKGROUND: Cutaneous T-cell lymphoma (CTCL) represents a spectrum of diseases composed of malignant T lymphocytes. The most common type is mycosis fungoides (MF). An accurate diagnosis of early MF may be difficult because of the varied clinical and histologic expressions of the disease. METHODS: The authors review the epidemiology, possible risk factors, clinical manifestations, diagnostic techniques, staging, prognosis, and treatment options for MF. RESULTS: The varied and often nonspecific clinical and histologic presentations of MF may delay diagnosis and staging, thus necessitating further studies such as immunophenotyping and T-cell receptor gene rearrangement analysis. CONCLUSIONS: A multidisciplinary approach to the diagnosis, staging, and treatment of MF assists in optimizing outcomes from management of patients with this disease.