Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases (2023)

FAQs

What is subcutaneous panniculitis type T-cell lymphoma? ›

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of skin lymphoma that is localized primarily to the subcutaneous adipose tissue without involvement of the lymph nodes. Clinically, the skin lesions mimic lipomas, while histologically they resemble panniculitis.

Subcutaneous Panniculitis-Like T-Cell Lymphoma Prognosis

The prognosis for people diagnosed with subcutaneous panniculitis-like T-cell lymphoma is generally positive, with overall five-year survival rates between 85 percent and 91 percent for those with primary cutaneous forms of the condition.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an uncommon extranodal lymphoma involving the skin that can mimic other panniculitic conditions. Its prognosis is bad so it is important to exclude other benign panniculitic conditions especially lupus panniculitis and erythema nodosum.

Subcutaneous panniculitis-like T-cell lymphoma

C86. 3 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. The 2023 edition of ICD-10-CM C86. 3 became effective on October 1, 2022.

Studies have shown that most cases are successfully treated with systemic corticosteroids or immunosuppressive agents such as etoposide, cyclosporine A, methotrexate, chlorambucil, and bexarotene[3]. Conventional doxorubicin-based chemotherapy may be employed when the disease is progressive[5].

Cutaneous T-Cell Lymphoma is a form of cancer that causes immune system dysfunction that leads to skin health concerns. Like other forms of cancer, Cutaneous T-Cell Lymphoma can be deadly, especially if the condition goes untreated until it reaches the advanced stages.

Patients who have stage IIB disease with cutaneous tumors have a median survival rate of 3.2 years (10-year survival rate of 42%) Patients who have stage III disease (generalized erythroderma) have a median survival rate of 4-6 years (10-year survival rate of 83%)

According to SEER, the overall survival rate for all types of T-cell non-Hodgkin lymphoma was 63.1 percent. In other words, the data suggests that, on average, people with T-cell non-Hodgkin lymphoma are about 63 percent as likely as people without the cancer to live five or more years after their diagnosis.

Overall, people with non-Hodgkin lymphoma (including T-cell lymphoma along with other types) have a five-year RS of 66.9 percent. Those with T-cell NHL specifically have a slightly lower survival rate of 63.1 percent. Survival may be further broken down by type of T-cell NHL.

Often, panniculitis affects the shins and the calves, then spreads to the thighs and upper body. It usually will clear away within six weeks of forming and leave no scar. If anything, sometimes a slight mark, almost like a bruise, will remain but then fade away.

What biopsy is best for panniculitis? ›

Incisional biopsies including the subcutis (ie fat) are necessary if the differential diagnosis includes a panniculitis (eg erythema nodosum).

Several types of cutaneous T-cell lymphoma exist. The most common type is mycosis fungoides. Sezary syndrome is a less common type that causes skin redness over the entire body. Some types of cutaneous T-cell lymphoma, such as mycosis fungoides, progress slowly and others are more aggressive.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cutaneous T-cell lymphoma which can have initial clinical manifestations of life threatening hemophagocytic lymphohistiocytosis (HLH) as complications. Due to non-specific symptoms, it can also be misdiagnosed as autoimmune diseases initially.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of hematologic malignancy characterized by lesions in subcutaneous fat associated with systemic symptoms. The standard treatment of the disease, currently, is not established, but CHOP or CHOP-like regimens are usually given.

ICD-10 code C84. 4 for Peripheral T-cell lymphoma, not elsewhere classified is a medical classification as listed by WHO under the range - Malignant neoplasms .

T-cell lymphoma is not curable but it is treatable. Yale Medicine's Department of Dermatology offers expert, multidisciplinary care and advanced treatments, including phototherapy for this unusual type of lymphoma.

While CTCL cannot be cured, advances in treating this cancer are making these goals possible for more patients. Today, many people who have CTCL die of another cause.

Treatment of Panniculitis

There is no specific definitive treatment for panniculitis. Several strategies have been used with modest results, including nonsteroidal anti-inflammatory drugs, antimalarials, dapsone, and thalidomide.

Hence T-cell lymphomas are worse than B-cell lymphomas.

What is the average age for cutaneous T-cell lymphoma? ›

About Cutaneous T-Cell Lymphoma

The incidence of CTCL increases with age, with an average onset between 50 and 60 years.

Stages 1 and 2 are generally considered early stages, during which cutaneous T-cell lymphoma is more treatable. In advanced-stage disease, including stages 3 and 4, a systemic treatment plan may be necessary to address more complex and widespread cancer.

Most are slow growing and not aggressive. They usually proceed over many years, typically do not make the patient seriously ill, and can be treated multiple times. Some rare types of cutaneous lymphomas can be more aggressive (fast growing and spreading).

Brain metastasis from systemic or cutaneous lymphoma is infrequently encountered. We present a case report of cutaneous T-cell lymphoma (CTCL) that metastasized to the brain. A literature review identified fourteen other cases of brain infiltration with CTCL.

Stage 4 is the most advanced stage of lymphoma. Lymphoma that has started in the lymph nodes and spread to at least one body organ outside the lymphatic system (for example, the lungs, liver, bone marrow or solid bones) is advanced lymphoma.

Key points about cutaneous T-cell lymphoma

The most common symptom is dry, red, itchy, scaly rashes or patches on the skin. This cancer can spread from the skin into the blood, lymph nodes, and other organs.

In most cases, the cause of T-cell lymphomas is unknown. Research has shown that certain viral infections or health conditions can increase the risk of developing certain types of T-cell lymphoma. The human T-lymphotropic virus 1 (HTLV1) is linked with development of adult T-cell leukaemia/lymphoma (ATLL).

Success Rate of CAR T-Cell Therapy in Phase I Clinical Trial

Patients with refractory disease had cancer that did not respond to treatment at all. Within 28 days of being treated with CAR T-cell therapy, 82% of trial patients responded positively – with more than half of them still in remission six months later.

What triggers panniculitis? ›

Possible causes of panniculitis include: infections, such as strep throat, tuberculosis, and pneumonia. autoimmune disorders, including Crohn's disease, ulcerative colitis, lupus erythematosus, and systemic sclerosis. physical causes, such as injury or cold temperatures.

Panniculitis is a group of conditions that cause painful bumps, or nodules, to form under your skin, often on your legs and feet. These bumps create inflammation in the fat layer under your skin. This layer is called the panniculus, or subcutaneous fat layer.

A significant number of patients having mesenteric abnormalities consistent with mesenteric panniculitis on CT scan will have an underlying form of cancer. The most common cancers with mesenteric panniculitis like abnormalities on CT scan are lymphomas.

In most patients, idiopathic nodular panniculitis is associated with fever, a general feeling of ill health (malaise), muscle pain (myalgia) and/or abdominal pain. These symptoms may subside after a few days or weeks and may recur weeks, months or years later and on multiple occasions.

Is mesenteric panniculitis serious? It's not life-threatening and rarely causes serious complications. Most people have mild to moderate symptoms, and they can go away on their own. But it can cause discomfort, and some people may need medication to treat it.

The most common form of panniculitis is erythema nodosum. Panniculitis is classified as mostly septal panniculitis or mostly lobular panniculitis depending on the site of the most intense microscopic inflammation. Most types of panniculitis have both septal inflammation (early) and lobular inflammation (later).

Angioimmunoblastic T-cell lymphoma

Angioimmunoblastic lymphoma (AITL) is a rare type of non-Hodgkin lymphoma (NHL). It is a high grade (aggressive) lymphoma that affects blood cells called T cells.

The human T-lymphotropic virus type 1 is also known by the acronym HTLV-1, or as human T-cell leukaemia virus type 1. The virus can cause a type of cancer called adult T-cell leukaemia/lymphoma (ATL). HTLV-1 is transmitted primarily through infected bodily fluids including blood, breast milk and semen.

Angioimmunoblastic T-Cell Lymphoma (AITL) is a rare, aggressive type accounting for about seven percent of all patients with T-cell lymphomas in the United States.

Pancreatic panniculitis is rare form of panniculitis with associated pancreatic disease. The skin manifestations can occur at any time of the pancreatic pathology.

What mimics panniculitis? ›

Pseudolymphoma and some forms of lymphoma can mimic panniculitis.

Panniculitis (inflammation of the subcutaneous fat) is a relatively uncommon condition that usually presents with inflammatory nodules or plaques. A wide variety of subtypes of panniculitis exist, including panniculitides related to infection, external insults, malignancy, and inflammatory diseases (table 1).

There are several ways cutaneous T cell lymphoma can appear. In its early stages, it can look like flaky, dry skin, so it's often mistaken for eczema. It can also appear scaly like psoriasis and even as blisters on the skin. As the disease progresses, it can continue to develop into thicker patches known as plaques.

Early cutaneous T-cell lymphomas (CTCL) may share many clinical and histological features with other autoimmune and inflammatory conditions.

Cutaneous T-cell lymphoma (CTCL) is an uncommon type of non-Hodgkin lymphoma (NHL). CTCL is a cancer that starts in mature T cells in the skin. Other types of B-cell and T-cell lymphoma can affect the skin, but they are different from CTCL. CTCL usually occurs in adults who are 50 to 60 years old.

Adult T-cell lymphoma/leukemia (ATL) is a rare T-cell lymphoproliferative neoplasm caused by human T-lymphotrophic virus 1. In its more common, aggressive forms, ATL carries one of the poorest prognoses of the non-Hodgkin lymphomas.

ICD-10 code C91. 51 for Adult T-cell lymphoma/leukemia (HTLV-1-associated), in remission is a medical classification as listed by WHO under the range - Malignant neoplasms .

Peripheral T-cell lymphoma, unspecified (PTCLU) is a type of non-Hodgkin lymphoma (NHL) that starts in mature T cells. PTCLU includes a group of mature T-cell lymphomas that do not have consistent features, so they don't fit into a specific type of T-cell lymphoma.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cutaneous T-cell lymphoma which can have initial clinical manifestations of life threatening hemophagocytic lymphohistiocytosis (HLH) as complications. Due to non-specific symptoms, it can also be misdiagnosed as autoimmune diseases initially.

The most common type of cutaneous T-cell lymphoma is mycosis fungoides and its leukemic variant, Sezary syndrome. The other more commonly seen forms of cutaneous lymphoma include the CD30 positive lymphoproliferative disorders (CD30+ LPDs).

What is the life expectancy of someone with T-cell lymphoma? ›

According to SEER, the overall survival rate for all types of T-cell non-Hodgkin lymphoma was 63.1 percent. In other words, the data suggests that, on average, people with T-cell non-Hodgkin lymphoma are about 63 percent as likely as people without the cancer to live five or more years after their diagnosis.

Overall, people with non-Hodgkin lymphoma (including T-cell lymphoma along with other types) have a five-year RS of 66.9 percent. Those with T-cell NHL specifically have a slightly lower survival rate of 63.1 percent. Survival may be further broken down by type of T-cell NHL.

Cutaneous T-cell lymphoma can cause rash-like skin redness, slightly raised or scaly round patches on the skin, and, sometimes, skin tumors. Several types of cutaneous T-cell lymphoma exist. The most common type is mycosis fungoides. Sezary syndrome is a less common type that causes skin redness over the entire body.

Most CTCLs are indolent (slow growing) and not life threatening. CTCLs are treatable, but they are not curable unless the patient undergoes a stem cell transplant (see later). There are several different types of CTCL.