# Radiation Oncology/NHL/Anaplastic large cell

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Anaplastic Large Cell Lymphoma (ALCL; CD30+)

## Overview

* T-cell or null-cell lymphoma, defined by membrane expression of CD30+
* First identified in 1985, added to REAL classification in 1994
* Represents ~5% of NHL in adults and ~15% of NHL in children
* Clinical subtypes (see below)
** Systemic
** Primary cutaneous

## Systemic ALCL Overview

* Pathologic subtypes of systemic ALCL
** ALK+
*** Predominately in young male patients
*** Aggressive presentation (75% systemic symptoms, 60% extranodal, 30% BM+)
*** Better prognosis (5-year OS 70-80%)
** ALK-
*** Predominately in older patients
*** Less aggressive presentation
*** Worse prognosis (5-year OS 30-40%)
* No specific RT information available as yet
* Typically managed as Aggressive Lymphomas:
** CHOP is first line of treatment, CHOP-R if CD20+
** RT as per institutional preference
* Overall better prognosis than other Aggressive Lymphomas

## Cuteneous ALCL Overview

* Accounts for 25-30% of primary cutaneous T-cell lymphomas
* Spectrum of diseases including lymphomatoid papulosis and anaplastic large-cell lymphoma
* Lymphomatoid papulosis
** Indolent lymphoproliferative disorder
** Typically multifocal
** Frequently recurrent, but resolves spontaneously without treatment over weeks to months
* Cutaneous CD30+ ALCL
** Indolent lymphoma
** Typically solitary or localized group of nodules and/or satellite papules. Localized is considered by the Dutch (see below) to be 1 anatomic area not exceeding 15 x 15 cm
** Spontaneous regression sometimes occurs, but frequently relapse
** Typically ALK- (unlike the systemic manifestation)
** Favorable prognosis, with 10-year DFS >90%
** Treatment is usually localized RT or surgical excision, but evidence is lacking
* Yale; 2008 (2001-2006) PMID 18037577 -- "Treatment of primary cutaneous CD30+ anaplastic large-cell lymphoma with radiation therapy." (Yu JB, Int J Radiat Oncol Biol Phys. 2008 Apr 1;70(5):1542-5. Epub 2007 Nov 26.)
** Retrospective. 8 patients. Localized C-ALCL, no other history, N0, treatment with RT alone. Median age 67, M=F. RT 40/20 (34 - 44 Gy), using 6-9 MeV with 0.5-2cm bolus. Median F/U 12 months
** Outcome: 100% CR
** Toxicity: Grade 1-2 dermatitis
** Conclusion: RT alone for localized C-ALCL is well tolerated and clinical response is excellent. Longer follow-up necessary
* Stanford; 2003 PMID 14639383 -- "CD30+ cutaneous lymphoproliferative disorders: the Stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma." (Liu HL, J Am Acad Dermatol. 2003 Dec;49(6):1049-58.)
** Retrospective. 56 patients with CD30+ lymphoproliferative disorders (LyP n=31, PC-ALCL n=25). Treatment for PC-ALCL excision, RT, chemotherapy or combination. RT dose not specified
** Outcome: 10-year DFS LyP 92%, PC-ALCL 85%; 5-year DFS localized PC-ALCL 91% vs generalized 50% (NS). Highly responsive to treatment, but relapse rate 42%. Progression to extracutaneous 3 patients
** Conclusion: Overall excellent prognosis, but cases with poor outcome exist
* Dutch Cutaneous Lymphoma Group; 2000 PMID 10845893 -- "Primary and secondary cutaneous CD30(+) lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment." (Bekkenk MW, Blood. 2000 Jun 15;95(12):3653-61.)
** Retrospective. 219 patients, primary or secondary cutaneous CD30+ lymphoproliferative disorders. Lymphomatoid papulosis (Group 1, n=118); primary C-ALCL (Group 2, n=79); C-ALCL with regional LN+ (Group 3, n=11); secondary (extensive cutaneous and extracutaneous) C-ALCL (Group 4, n=11). RT dose not specified
** Treatment: LyP often no treatment. Skin limited Group 1-2 treated with RT or excision, and if treated with chemo, all had relapse.
** Outcome: 10-year risk for systemic disease Group 1 4%, Group 2 16%, Group 3 20%. 5-year DFS 100%, 96%, 91%, 24%
** Conclusion: Favorable prognosis. Chemotherapy only for developing/existing extracutaneous disease
* EORTC; 1995 PMID 7751878 -- "CD30/Ki-1-positive lymphoproliferative disorders of the skin--clinicopathologic correlation and statistical analysis of 86 cases: a multicentric study from the European Organization for Research and Treatment of Cancer Cutaneous Lymphoma Project Group." (Paulli M, J Clin Oncol. 1995 Jun;13(6):1343-54.)
** Retrospective. 92 cases reviewed by expert panel.
** Prognosis: favorable - age <60, spontaneous regression; unfavorable - age >60, extracutaneous disease
** Conclusion: CD30+ cutaneous lymphoproliferative disorders are a spectrum of closely related skin lesions
* European Multicenter; 1993 PMID 8382999 -- "Primary cutaneous CD30-positive large cell lymphoma: definition of a new type of cutaneous lymphoma with a favorable prognosis. A European Multicenter Study of 47 patients." (Beljaards RC, Cancer. 1993 Mar 15;71(6):2097-104.)
** Consensus report for definition. 47 patients from 5 center analyzed.
** Features: solitary or localized skin lesions, partial/complete spontaneous remission of skin lesions (~25%), frequent cutaneous relapse (~30%). Extracutaneous disease developed in 25%
** Outcome: favorable prognosis. DFS 77%, death from disease 9%
** Conclusion: CD30+ cutaneous lymphoma can be considered a distinct type of cutaneous T-cell lymphoma

## Links

* Wikipedia ALCL
* Atlas of Genetics & Cytogenetics
* E-Medicine