How is anaplastic large cell lymphoma (ALCL) staged?

Answer

The Cotswold modification of the Ann Arbor staging system is the standard anatomic staging system for non-Hodgkin lymphoma and Hodgkin disease; it is used to evaluate the extent of disease in patients with anaplastic large cell lymphoma (ALCL). Accurate staging allows appropriate therapeutic selection and contributes to predicting the prognosis.

Staging for anaplastic large cell lymphoma (ALCL) is as follows^{[2, 26]}:

Stage I – Involvement of a single lymph node region or lymphoid structure
Stage II – Involvement of 2 or more lymph node regions on the same side of the diaphragm
Stage III – Involvement of lymph node regions or structures on both sides of the diaphragm
Stage IV – Involvement of extranodal sites beyond that designated as E (see below)

Further staging designations include the following:

Suffix A – No symptoms (any disease stage)
Suffix B – Fever (temperature >38°C); drenching sweats; unexplained weight loss (eg, 10% of body weight within preceding 6 mo) (any disease stage)
Suffix E – Involvement of a single extranodal site that is contiguous or proximal to the known nodal site (stages I-III)

Cotswold modifications are as follows:

Suffix X – Denotes bulky disease (a widening of the mediastinum by more than one third or the presence of a nodal mass with a maximal dimension of >10 cm)
Subscripts – Used to indicate the number of anatomic regions
Stage III subdivisions – May be subdivided to include III(1), with or without splenic, hilar, celiac, or portal nodes, and III(2), with para-aortic, iliac, or mesenteric nodes
Further identification – Staging identified as clinical stage (CS) or pathologic stage (PS)

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Media Gallery

Anaplastic large cell lymphoma. This ulcerated nodule of localized primary cutaneous CD30+ anaplastic large cell lymphoma is relatively nonspecific in appearance. Courtesy of Dr. Ronald Grimwood.
Anaplastic large cell lymphoma. Sheets of atypical cells are seen infiltrating through the superficial and deep dermis. The epidermis is hyperkeratotic (hematoxylin and eosin, 40X).
Anaplastic large cell lymphoma. The markedly atypical, large, and pleomorphic epithelioid cells have frequent mitoses. Prominent nucleoli are present (hematoxylin and eosin, 400X).
Anaplastic large cell lymphoma. CD30 strongly stains all the malignant cells in the characteristic membranous pattern (CD30 immunostain, 100X).

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Author

Delong Liu, MD, PhD Professor of Medicine, Division of Oncology/Hematology, New York Medical College; Chief of Hematology, Phelps Memorial Hospital Center; Director of Non-ablative Allogeneic Stem Cell Transplantation Program, Westchester Medical Center; Editor-in-Chief, Journal of Hematology and Oncology

Delong Liu, MD, PhD is a member of the following medical societies: American Society of Hematology, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Coauthor(s)

Muhammad Furqan, MD Assistant Professor, Division of Hematology, Oncology, Blood and Marrow Transplant, Department of Medicine, University of Iowa Hospitals and Clinic

Muhammad Furqan, MD is a member of the following medical societies: American College of Physicians, American Medical Association, American Society of Clinical Oncology, American Society of Hematology

Disclosure: Nothing to disclose.

Christine Urbanski, MD Consulting Staff, Hematology/Oncology Associates

Christine Urbanski, MD is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Koyamangalath Krishnan, MD, FRCP, FACP Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine, James H Quillen College of Medicine at East Tennessee State University

Koyamangalath Krishnan, MD, FRCP, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Society of Hematology, Royal College of Physicians

Disclosure: Nothing to disclose.

Acknowledgements

David Aboulafia, MD Medical Director, Bailey-Boushay House, Clinical Professor, Department of Medicine, Division of Hematology, Attending Physician, Section of Hematology/Oncology, Virginia Mason Clinic; Investigator, Virginia Mason Community Clinic Oncology Program/SWOG

David Aboulafia, MD is a member of the following medical societies: American College of Physicians, American Medical Association, American Medical Directors Association, American Society of Hematology, Infectious Diseases Society of America, and Phi Beta Kappa