Subcutaneous panniculitis-like T-cell lymphoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

Synonyms and keywords: SPTCL; Subcutaneous panniculitis-like T-cell lymphoma alpha/beta subtype; Subcutaneous panniculitis-like T-cell lymphoma gamma/delta subtype; SPTCL-AB; SPTCL-GD

Overview

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of T-cell non-Hodgkin lymphoma (NHL). Subcutaneous panniculitis-like T-cell lymphoma is a cytotoxic T-cell lymphoma, which preferentially infiltrates subcutaneous tissue, primarily involving the extremities and trunk. According to the World Health Organization-European Organization for Research and Treatment of Cancer, SPTCL is classified under Primary Cutaneous T-Cell Lymphomas and expresses the TCRαβ phenotype (SPTCL-AB). TCRγδ phenotypes are now a separate entity from SPTCL, known as the Cutaneous γδ T-cell Lymphoma. On microscopic histopathological analysis, atypical lymphoid cells, fat necrosis,and karyorrhexis are characteristic findings of subcutaneous panniculitis-like T-cell lymphoma. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary and the 5-year survival rate of patients with subcutaneous panniculitis-like T-cell lymphoma is approximately 80%. Symptoms of the SPTCL include fever, weight loss, night sweats, and painless swellings on extremities and trunk. Subcutaneous nodule biopsy is diagnostic of SPTCL. The predominant therapy for SPTCL is chemotherapy. Adjunctive radiotherapy and stem cell transplant may be required.

Classification

Subcutaneous panniculitis-like T-cell lymphoma is classified under Primary cutaneous lymphomas and expresses the TCRαβ phenotype.

Name	Description
Alpha/Beta subtype (SPTCL-AB)	Now considered the only type of subcutaneous panniculitis-like T cell lymphoma Slow-growing (indolent) disease with a favourable prognosis.
Gamma/Delta subtype (SPTCL-GD)	Included in the cutaneous T-cell lymphoma group. More aggressive type. Hemophagocytic syndrome is most often associated with SPTCL-GD and has a less favourable prognosis.

Pathophysiology

Subcutaneous panniculitis-like T-cell lymphoma is a cytotoxic T-cell lymphoma, which preferentially infiltrates subcutaneous tissue, primarily involving the extremities and trunk.

Microscopic Pathology

On microscopic histopathological analysis, atypical lymphoid cells, fat necrosis, and karyorrhexis are characteristic findings of subcutaneous panniculitis-like T-cell lymphoma.

• 

  Biopsy of soft tissue : at 10x magnification shows lymphoid infiltrate with pattern resembling lobular and septal panniculitis

• 

  Biopsy of soft tissue : at 40x magnification shows lymphoid infiltrate with abundant cytoplasm.

• 

  Biopsy of soft tissue : At 100x magnification with oil-immersion biopsy shows atypical lymphoid cells with atypical and irregular nuclei as well as numerous fat cells.

• 

  Biopsy of soft tissue : At 100x magnification with oil-immersion biopsy shows numerous single-cell necroses and atypical lymphoid infiltrate.

• 

  Biopsy of soft tissue : At 100x magnification with oil-immersion biopsy shows mitotic figure in the center with areas of necrosis and fibrosis.

• 

  Biopsy of soft tissue : At 100x magnification with oil-immersion biopsy shows mitotic figure with scattered histiocytes and area of necrosis.

• 

  Biopsy of soft tissue : At 100x magnification with oil-immersion biopsy shows area of necrotic debris.

Causes

There are no established causes for subcutaneous panniculitis-like T-cell lymphoma.

Differentiating SPTCL from other Diseases

Subcutaneous panniculitis-like T-cell lymphoma must be differentiated from other diseases such as:

• Benign panniculitis
• Extranodal NK-T-cell lymphoma
• Lupus erythematosus panniculitis
• Primary cutaneous CD56+ Natural Killer-like T-Cell lymphoma
• Primary cutaneous anaplastic large cell lymphoma
• Mycosis Fungoides
• Blastic plasmacytoid dendritic cell neoplasm
• Primary cutaneous lymphomas

Epidemiology and Demographics

Age

Subcutaneous panniculitis-like T-cell lymphoma commonly affects young adults (35 years).

Gender

Females are more commonly affected with subcutaneous panniculitis-like T-cell lymphoma than males.

The female to male ratio is 2:1.

Risk Factors

There are no established risk factors for subcutaneous panniculitis-like T-cell lymphoma however, many patients have an associated autoimmune disorder at diagnosis.

Screening

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for subcutaneous panniculitis-like T-cell lymphoma.

Natural History, Complications and Prognosis

• Painless nodules form under the skin in the subcutaneous tissue (subcutaneous nodules).
• Skin nodules may demonstrate regression with lipoatrophy at the site of the lesion.
• New nodules can appear at different sites over the course of the disease.
• The nodules commonly occur in the extremities or trunk. Other areas include: face, neck and back.
• Skin nodules range in size from 0.5 cm to several centimeters in diameter. Larger nodules may become necrotic, ulceration is rare.
• The infiltrate involves the fat lobules, usually sparing the septa. The overlying dermis and epidermis are typically uninvolved. Dissemination to lymph nodes and other organs is rare.
• People with this type of lymphoma can also develop a very serious condition where certain parts of the immune system are activated (hemophagocytic syndrome).
• Hemophagocytic syndrome causes:

• Fever
• Enlarged liver and spleen (hepatosplenomegaly)
• A lower number of red blood cells, white blood cells and platelets in the blood (pancytopenia)
• Failure to thrive
• Maculopapular rash
• CNS symptoms
• Lymphadenopathy
• Coagulopathy
• Abnormal LFTs
• High serum Ferritin

• Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary and the 5-year survival rate of patients with subcutaneous panniculitis-like T-cell lymphoma is approximately 80%

Diagnosis

Symptoms

Symptoms of SPTCL include:

• Fever
• Chills
• Weight loss
• Night sweats
• Myalgias
• Painless swellings on extremities and trunk

Physical Examination

Vitals

• Fever is often present

Skin

• Subcutaneous nodules

Abdomen

• Hepatosplenomegaly

Laboratory Findings

Laboratory tests for subcutaneous panniculitis-like T-cell lymphoma include:

• Complete blood count (CBC): cytopenia
• Blood chemistry studies: Increased liver function tests
• Cytogenetic analysis
• Flow cytometry
• Immunophenotyping

• Positive: BetaF1; CD8; CD30; CLA; T-cell intracellular antigen (T1A1); and perforin
• Negative: CD3; CD5; and CD56

Biopsy

Subcutaneous nodule biopsy is diagnostic of subcutaneous panniculitis-like T-cell lymphoma.

Other Imaging Findings

CT, MRI, and PET scan may be helpful in the diagnosis of subcutaneous panniculitis-like T-cell lymphoma.

Treatment

Medical Therapy

Treatment of subcutaneous panniculitis-like T-cell lymphoma

Therapy	Description
Chemotherapy	Combination therapy : CHOP – Cyclophosphamide AND Doxorubicin AND Vincristine AND Prednisone
Radiation therapy	External beam radiation therapy may be used.
Stem cell transplant	May be used in some people with subcutaneous panniculitis-like T-cell lymphoma when their lymphoma recurs after treatment.