PRIMARY CUTANEOUS ANAPLASTIC LARGE CELL LYMPHOMA

Expert Review by

Steven M. Horwitz, MD, Assistant Attending, Lymphoma Service, Memorial Sloan-Kettering Cancer Center

You Are Not Alone
CLF Board Member Joe Eischens shares his personal experience of being diagnosed with cutaneous lymphoma and his suggestions for those who are newly diagnosed.

Joe Eischens
Kansas City, MO

Definition
What is Primary Cutaneous Anaplastic Large Cell Lymphoma?

Primary cutaneous T-cell lymphomas (CTCL) are part of a group of rare non-Hodgkin lymphomas that arise from the T-cell type lymphocytes. Included in this group is primary cutaneous anaplastic large cell lymphoma (PCALCL). Anaplastic large cell lymphoma (ALCL) comprises only about three percent of all lymphomas in adults and between ten percent and 30 percent of all lymphomas in children. The disease is characterized by the size and shape of the cells under the microscope and by the uniform expression of a special marker on the lymphoma cells called CD30. There are two types of anaplastic large cell lymphoma: systemic, which can affect the skin and/or lymph nodes and other internal organs; and primary cutaneous, which affects the skin only. However, in rare instances, over time, primary cutaneous ALCL may extend beyond the skin to internal organs.

Primary cutaneous anaplastic large cell lymphoma is an indolent, or slow growing, lymphoma and is associated with a rare condition called lymphomatoid papulois (LyP), which, while classified as a lymphoma, always goes away by itself. Lymphomatoid papulois is occasionally a precursor to the development of cutaneous anaplastic large cell lymphoma or other lymphomas. There are no known risk factors for primary cutaneous anaplastic large cell lymphoma. Although the disease can affect people of all ages, including children, it is most commonly found in adults 45 to 60 years old, and typically occurs more often in men than women.

The characteristic features of primary cutaneous anaplastic large cell lymphoma include the appearance of solitary or multiple raised red skin lesions, nodules or tumors, which do not go away and have a tendency to ulcerate and may itch. The lesions can appear on any part of the body and often grow very slowly and may be present for a long time before being diagnosed.

What is PCALCL?
Cutaneous lymphoma experts provide answers to the question: "Primary Cutaneous Anaplastic Large Cell Lymphoma: What is it?"

EXPERT PRESENTERS

Lauren Pinter-Brown, MD, Professor, Department of Medicine, Division of Hematology/Oncology, UC Irvine Medical Center
(At time of publication: Clinical Professor of Medicine, David Geffen School of Medicine, UCLA School of Medicine)

Stuart Lessin, MD, Medical Director, KGL Skin Study Center

Christiane Querfeld, MD, Director, Cutaneous Lymphoma Program, City of Hope Comprehensive Cancer Center

Diagnosis
How How is Primary Cutaneous Anaplastic Large Cell Lymphoma Diagnosed and Staged?

Distinguishing primary cutaneous anaplastic large cell lymphoma from systemic anaplastic large cell lymphoma requires a biopsy (removal of a small piece of tissue) of the skin lesion, followed by a series of other diagnostic tests, including blood, CT (computerized axial tomography), MRI (magnetic resonance imaging) and/ or PET (positron emission tomography) scans. After a sample of the skin lesion is removed, it is examined by a pathologist (a doctor who studies tissues and cells to identify diseases) under a microscope to determine a diagnosis. Because a pathological diagnosis of cutaneous anaplastic large cell lymphoma can sometimes be difficult to determine, it may be necessary to have the pathology report reviewed by a dermatopathologist or a hematopathologist, a pathologist who is an expert in diagnosing lymphomas.

In addition to these diagnostic tests, in some instances, for example, if imaging tests show lymph node or other organ involvement, a bone marrow biopsy, in which soft tissue is removed from inside the bone, might also be performed to determine the extent, or stage, of the disease.

The four stages of cutaneous anaplastic large cell lymphoma include:

Stage 1

The cancer affects only a single spot or single area of the skin. Lymph nodes are not enlarged.

Stage 2

Either of the following may be true: the skin contains dry, red, scaly patches, but no tumors. Enlarged lymph nodes may be present, but they do not contain cancer cells. Or, there are tumors on the skin and lymph nodes are either normal or larger than normal, but they do not contain cancer cells.

Stage 3

Nearly all the skin is covered in dry, red, scaly lesions. The lymph nodes  are either normal or larger than normal, but they do not contain cancer cells.

Stage 4

In addition to the skin being involved, cancer cells are found in the lymph nodes or the cancer has spread to other organs such as the liver or lung.

Treatment Options
How is Primary Cutaneous Anaplastic Large Cell Lymphoma Treated?

While most people will have disease that is always confined to the skin, over a lifetime, between five percent and 15 percent of people with primary cutaneous anaplastic large cell lymphoma may develop involvement of the lymph nodes or other sites outside the skin, in which case the cancer is treated more aggressively with combination regimens of chemotherapy and sometimes radiation therapy. Very aggressive treatments, such as bone marrow transplantation, are rarely considered.

Treatment Options and Side Effects for PCALCL
Cutaneous lymphoma experts provide answers to the question: "Primary Cutaneous Anaplastic Large Cell Lymphoma: What are the treatment options and side effects?"

EXPERT PRESENTERS

Stuart Lessin, MD, Medical Director, KGL Skin Study Center

Christiane Querfeld, MD, Director, Cutaneous Lymphoma Program, City of Hope Comprehensive Cancer Center

Lauren Pinter-Brown, MD, Professor, Department of Medicine, Division of Hematology/Oncology, UC Irvine Medical Center (At time of publication: Clinical Professor of Medicine, David Geffen School of Medicine, UCLA School of Medicine)

There are many therapies available to treat primary cutaneous ALCL both for single and multiple lesions.

For localized disease, meaning the existence of a single or a few clustered lesions, nodules or tumors limited to one area of the body, radiotherapy is preferred, although surgical excision or topical treatments such as bexarotene (Targretin) or nitrogen mustard (Mustargen) may be used. For people with multiple sites, systemic treatment is usually needed and may include:

  • Bexarotene (Targretin) capsules
  • Brentuximab Vedotin
  • CVP (cyclophosphamide, vincristine, prednisone) chemotherapy
  • Low doses of methotrexate (Trexall)
  • PUVA (Psoralen and ultraviolet-A light), a type of photodynamic therapy
  • PDX (pralatrexate)
  • Romidepsin
  • Vorinostat (Zolinza), a histone deacety-lase inhibitor

All of these therapies are effective in treating the disease, although relapse is common and, therefore, long-term follow-up care is required.

What Can Individuals with PCALCL Expect Long-term?

EXPERT PRESENTERS

Lauren Pinter-Brown, MD, Professor, Department of Medicine, Division of Hematology/Oncology, UC Irvine Medical Center
(At time of publication: Clinical Professor of Medicine, David Geffen School of Medicine, UCLA School of Medicine)

Christiane Querfeld, MD, Director, Cutaneous Lymphoma Program, City of Hope Comprehensive Cancer Center

Stuart Lessin, MD, Medical Director, KGL Skin Study Center

Clinical Trials
PARTICIPATING IN CLINICAL TRIALS

Clinical trials are crucial in identifying effective drugs, prognostic strategies and determining optimal doses for lymphoma patients. If you are interested in participating in a clinical trial, talk to your doctor about an appropriate trial for you.

Alternative Therapies
Are Complementary and Alternative Therapies Safe and Effective?

Complementary and alternative medicines are nonstandard therapies that may help patients cope with their cancer and its treatment, but that should not be used in place of standard treatment. No alternative therapy has ever been proven effective against lymphoma. However, complementary therapies such as meditation, yoga, acupuncture, exercise, diet and relaxation techniques have been shown to be effective in combating some treatment side effects. Before embarking on any complementary therapies, patients should discuss the matter with their healthcare team. Certain unproven treatments, including some herbal supplements, can interfere with standard lymphoma treatments or may cause serious side effects.

Learn More

Skin-Directed Therapies

Individuals with disease limited to the skin (stages I and II) can often achieve a good response with one of the readily available skin-directed therapies. Learn more about skin-directed therapies.

Systemic Therapies

Individuals who have only partial or short responses to skin-directed therapies, or more advanced stages of disease, may benefit from systemic therapy. Learn more about available systemic therapies.

Clinical Trials: A Pathway to Treatment

Physician & Patient
Clinical trials are research studies that investigate new or experimental treatments in patients living with a specific disease. Clinical trials empower patients to play an active role in their own care and open them up to access new treatments before they are widely available.
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