PRIMARY CUTANEOUS ANAPLASTIC LARGE CELL LYMPHOMA (pcALCL) is an indolent CD30+ lymphoma usually treated with local therapy. It must be distinguished from systemic ALCL as the latter is an aggressive disease which requires multiagent systemic chemotherapy and from LyP which spontaneously regresses. The definition of "primary" cutaneous disease is somewhat controversial but generally is defined as skin disease without evidence of systemic involvement and no previous history of lymphomatoid papulosis, mycosis fungoides, or HD. Cases with cutaneous disease and nodal involvement limited to a regional draining node are problematic, and it is uncertain if they have a different prognosis or should be treated as primary cutaneous diseaseref. Unlike systemic ALCL, it is negative for epithelial membrane antigen (EMA) and it does not express ALK. Primary cutaneous ALCL is part of the spectrum of CD30+ cutaneous lymphoproliferative disease and clinically overlaps with lymphomatoid papulosisref (Kadin ME. Primary CD30+ cutaneous lymphomas (including lymphomatoid papulosis). In: Mason DY, Harrison NL, eds. Human Lymphoma: Clinical Implications of the REAL Classification. London, Springer; 1999;38:19)
Epidemiology : usually arises in the skin in an older patient (median age 60 years)
Symptoms & signs : isolated reddish violet tumor, which may be ulceratedref. Less commonly, the disease may present as multiple nodules in a circumscribed area, or rarely with widespread skin lesions.
Laboratory examinations :
clinical features
LyP
ALCL
type of lesion papules, nodules nodules, tumors
number multiple single or grouped
size usually <1 cm > 2 cm (> 3 cm more predictive of lymphoma; borderline lesions are usually intermediate in size, 1-2 cm)
sites of disease extremities, trunk extremities, head and neck
regression yes, usually with a scar 25% of cases
histology/immunophenotype
LyP
ALCL
pattern of infiltration wedge-shaped perivascular/ periadnexal more diffuse
subcutaneous involvement absent or minimal present
mixed inflammatory cells many few to many
CD30+ cells scattered single or small clusters large groups or sheets
epithelial membrane antigen (EMA) present in 10-30% of cases present in 10-30% of cases
anaplastic lymphoma kinase (ALK) usually negative usually negative
ALCL produces nodules or tumors that are single, but may be multiple and grouped, and are often ulcerated. LyP typically presents with multiple small papular lesions, usually < 1 cm, on the trunk or extremities that usually ulcerate and heal with a scar in 4-6 weeks. Histologically the infiltrate of LyP is wedge-shaped and perivascular and is composed of small lymphocytes and scattered single and small clusters of large atypical cells often resembling Reed-Sternberg cells. Invasion of the subcutaneous tissue is uncommon. As the lesions progress acute inflammatory cells including eosinophils become more prominent. The large atypical cells mark as T-cells and EMA may be present in up to approximately one-third of LyP. Borderline lesions (sometimes called LyP type C) typically are 1-2 cm in size and have larger clusters of CD30+ cells than typically seen in LyPref. Invasion of the subcutaneous tissue is minimal or absent. If a history of similar regressing lesions is present LyP should be favored
Treatment of localized lesions may be watchful waiting with approximately 25% of patients regressing, local excision with or without radiation, or radiation aloneref. Patients with disseminated skin disease or localized skin disease with nodal extension may benefit from combination chemotherapy, but these patients tend to relapseref. Immunostaining for ALK should be performed and if positive the lesion should be considered systemic ALCLref. Monomorphic histology also suggests systemic disease. A recent study by Wellman et al has recently shown that all systemic ALCL expressed the adhesion molecule clusterin, and none of the primary cutaneous ALCL, but few cases were testedref. EMA expression is not useful as it has been reported in 54% of simultaneous cutaneous and systemic ALCL and 100% of secondary skin involvement and up to one-third of primary cutaneous ALCLref. Even after a diagnosis of primary cutaneous ALCL is made, careful followup is indicated as 25% later develop nodal involvementref.
Prognosis : the importance of ruling out systemic disease with cutaneous spread cannot be overemphasized as systemic ALCL with cutaneous spread has a 5-year survival of 29-44% as opposed to 90-100% in primary cutaneous ALCLref. This poor prognosis in systemic disease with skin involvement may correlate with ALK negativity but has not been studied in detail. Due to the poor prognosis, careful staging is imperative.
  • [lymphomatoid papulosis (LyP) is a clinically benign variant of primary cutaneous ALCL

  • Symptoms & signs : recurrent crops of papules/nodules up to 2 cm in diameter which undergo spontaneous regression, and rarely develop into a lymphoma
    Therapy : LyP is managed by observation, ultraviolet light therapy, or low-dose weekly methotrexate can control the lesions of LyP (Vonderheid EC, Sajjadian A, Kadin ME. Methotrexate is effective therapy for lymphoimatoid papulosis and other primary cutaneous CD30-positive lymphoproliferative disorders. J Amer Acad of Dermatol. 1996;34:470481)
    Prognosis : LyP patients have a predisposition to develop malignant lymphomas, including Hodgkin's lymphoma, mycosis fungoides, and non-Hodgkin's lymphoma, by as yet unknown mechanisms. The prognosis for patients with LyP is otherwise excellentref]

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