Importance of diagnostics and risk of secondary malignancies in primary cutaneous lymphomas

Abstract

Summary Background and Objectives Primary cutaneous lymphomas (PCL) often strongly differ in clinical behavior and prognosis from systemic lymphomas of the same histopathologic type. The aim of the study was to investigate the distribution of PCL subtypes, the average time from disease manifestation to diagnosis, the importance of diagnostic procedures, the occurrence of secondary malignancies and the different treatment modalities. Patients and Methods Retrospective analysis of 152 patients with PCL examined at the Department of Dermatology of the University Hospital Tübingen from 2010–2012. Results 105 patients with CTCL (69.1 %) and 47 patients with CBCL (30.9 %) were included. The average time from disease manifestation to diagnosis was four years. The most common diagnosed lymphoma was mycosis fungoides (MF) (47.4 %). First‐line therapies here include phototherapy only (psoralen‐UV‐A [PUVA], n = 48; UVB 311 nm, n = 7) or combination therapies primarily phototherapy with systemic retinoids (n = 18). Most frequent second‐line therapy was interferon (INF)‐α plus PUVA (n = 15). The outcome was favorable (45.2 % remission, 28.6 % stable disease, 22.6 % progressive disease). Malignant comorbidities were observed more frequently compared to a healthy control group. Conclusions The diagnosis of lymphoma often takes several years. The value of staging procedures is still low and the treatment modalities for MF in earlier stages are mainly based on phototherapy.

Recurrent urticarial plaques in an old man

“…Mycosis fungoides (MF) is a clonal proliferation of T cells, being the most common primary cutaneous T-cell lymphoma (CTCL) and accounting for 50 % of all primary cutaneous lymphomas [2][3][4][5][6][7]. It progresses in three phases that include patches, plaques and tumors, which may overlap.…”

“…In recent years an increasing range of skin diseases that can be mimicked by MF have been described [1][2][3]5]. To date more than twenty entities have been reported: atopic dermatitis, contact dermatitis, nummular eczema, psoriasis, bullous diseases, pityriasis alba, alopecia, pigmented purpuric dermatosis, sarcoidosis, vitiligo, erythema multiforme, granuloma annulare etc.…”

Rezidivierende urtikarielle Plaques bei einem alten Mann

“…Nicht bei jedem Patienten entwickeln sich zwangsläufig alle Phasen [3,7,8]. Die frühen Stadien sind durch erythematöse Flecken und/oder schuppige und atrophische Plaques an sonnengeschützten Stellen gekennzeichnet und haben in der Regel eine günstige Prognose (88 % Fünfjahresüberleben) [2,3,[5][6][7][8]. Aufgrund des erhöhten Risikos der Entwicklung sekundärer Malignome, wie sekundärer Lymphome oder solider Tumore, ist eine angemessene Überwachung erforderlich [5].…”

Versorgungsstruktur der Patienten mit Mycosis fungoides und Sézary‐Syndrom in Deutschland – Versorgungsforschung auf Basis von GKV‐Routinedaten

“…Die CTCL zeichnen sich durch monoklonale T-Zell-Proliferationen aus, bei der Zellen vom Blut in die Haut wandern [8]. Die Diagnosestellung und Stadieneinteilung kutaner Lymphome erfolgt nach der aktuellen WHO-Klassifikation und basiert auf einer klinisch-pa-thologischen Korrelation [5,[9][10][11]. Das Robert Koch-Institut (RKI) schätzte für die Jahre 2011-2013 auf Basis der Erhebungen des Zentrums für Krebsregisterdaten die Anzahl der durchschnittlich jährlichen CTCL-Neuerkrankungen in Deutschland auf 610 (230 Frauen und 380 Männer) [12].…”