Complete Remission of Previously Intractable Peripheral Cutaneous T-Cell Lymphoma of the Lower Extremity Using Isolated Hyperthermic Limb Perfusion with Melphalan (1-Phenylalanine Mustard)

Abstract

The patient is a 74-year-old woman first diagnosed with a peripheral cutaneous T-cell lymphoma (PCTCL) in April of 1994. Initially she presented with subcutaneous indurated areas in the right forearm, scapula, and submadibular region. After chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), she went into remission for 2 years before relapse of her PCTCL localized to the right lower extremity. Persistent isolated disease in the extremity since then led to numerous chemotherapy regimens and localized radiation therapy. Due to dramatic limb threatening progression of the disease in 2001, she underwent isolated hyperthermic limb perfusion with melphalan (1-phenylalanine mustard). Although limb preservation could not be achieved, this treatment resulted in complete clinical and pathological regression of the lesions of the perfused extremity.

Isolated Limb Perfusion with Melphalan for the Treatment of Intractable Primary Cutaneous Diffuse Large B-Cell Lymphoma Leg Type

“…Previous case reports have already shown the effectivity of both hyperthermic isolated limb infusion and limb perfusion in low-grade B and T non-Hodgkin lymphoma (NHL) [15,16,17]. This report as well as previous results from other groups indicate that isolated limb perfusion with melphalan might represent an effective treatment of NHL of the lower leg, resulting in an average disease-free survival of 7 months [5,15,16,17].…”

“…Finally, late consideration of this therapeutic option might, according to our experience, seriously hamper the technical feasibility due to massive necrotic ulceration of the leg. This is further supported by the report of another group [17]. Accordingly, in our mind, this option should be considered early after the first recurrence occurred, especially for those elderly patients unable to stand intensive treatment.…”

“…Therefore, two techniques are available: the isolated limb perfusion and the isolated limb infusion. Both were found to be effective in the present setting [16,17], and both show similar results, although the infusion technique is reserved for more distal tumors and do not require a long operation procedure, as collateral veins are not clamped and the venous efflux is constantly discarded [16]. Isolated limb infusion could then possibly be more likely for patients unable to stand a prolonged anesthesia [14].…”

“…Previous case reports have already shown the effectivity of both hyperthermic isolated limb infusion and limb perfusion in low-grade B and T non-Hodgkin lymphoma (NHL) [15,16,17]. This report as well as previous results from other groups indicate that isolated limb perfusion with melphalan might represent an effective treatment of NHL of the lower leg, resulting in an average disease-free survival of 7 months [5,15,16,17]. These data are in line with the average disease-free survival reported in DLBCL leg type after first recurrence [2], as well as with our own experience with DLBCL leg type, even with the use of R-CHOP as first-line treatment.…”

Isolated limb perfusion as a treatment option for rare types of tumours

“…ILP can also be used for other more rare locally advanced tumours confined to the extremities, such as squamous cell carcinomas (SCC) [8], lymphomas [9][10][11], desmoid tumours [12] and Merkel cell tumours (MCC) [13]. The aim of this study is to describe our experience of ILP for these more rare types of tumours, and also include results from the, to our knowledge, first two patients treated with pigmented villonodular synovitis (PVNS) and giant cell tumours.…”