Lymphoma & related disorders

Mature T/NK cell disorders

Cutaneous / soft tissue involvement

Primary cutaneous acral CD8+ lymphoproliferative disorder



Last author update: 11 November 2022
Last staff update: 11 November 2022

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PubMed Search: Primary cutaneous acral CD8+ lymphoproliferative disorder

Busra Bacik Goksu, M.D.
Carlos A. Murga-Zamalloa, M.D.
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Cite this page: Goksu BB, Murga-Zamalloa CA. Primary cutaneous acral CD8+ lymphoproliferative disorder. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomanonbacraltcell.html. Accessed December 10th, 2022.
Definition / general
  • Rare indolent lymphoproliferative disorder characterized by slow growing papules or nodules in acral sites with a dermal infiltrate of CD8+ T lymphocytes
Essential features
Terminology
ICD coding
  • ICD-10: C84.A0 - cutaneous T cell lymphoma, unspecified, unspecified site
Epidemiology
Sites
  • Usually localized to the dermis in the ear (Br J Dermatol 2022;186:887)
  • Multiple sites of involvement may be present
  • Other sites may include the nose, hands, elbows and feet
  • No extracutaneous involvement is present
Pathophysiology
  • Pathophysiology is not known
Etiology
  • Cell of origin is activated mature T lymphocyte, CD3+ / CD8+ type
Clinical features
  • Painful, slowly growing single papule or nodule localized in the ear
  • Always an indolent course (Blood 2016;127:2375)
  • Cure is expected after local excision or localized therapy in 70% of patients; persistent disease in 20%
Diagnosis
  • Biopsy or excision
Radiology description
  • Single lesion localized to dermis
  • No systemic dissemination
  • Rare multifocal
Prognostic factors
  • Recurrences are rare and are limited to the dermis
  • Surgical excision or radiotherapy usually result in cure (70%) with an overall low relapse rate (20%) (Br J Dermatol 2022;186:887)
  • Relapsing / progressing disease more frequent in younger patients (Br J Haematol 2019;185:598)
    Case reports
    • 16 year old boy with an enlarging solitary lesion on the right lower eyelid of 1 month duration and presence of CD56 expression and high Ki67 proliferation (J Cutan Pathol 2021;48:1489)
    • 35 year old man and 45 year old woman with nasal involvement and similar clinical and histomorphological features (J Cutan Pathol 2010:37;977)
    • 52 year old man and 41 year old man with indolent CD8+ lymphoid proliferation of the ear (J Cutan Pathol 2011:38;209)
    • 53 year old woman with a 12 month history of an asymptomatic solitary right leg nodule with atypical BCL6 / GATA3 positivity (Am J Dermatopathol 2021;43:137)
    • 57 year old man with history of slowly growing ear nodule with signet ring features (Histopathology 2009;55:468)
    • 69 year old man with a 10 year history of a solitary slow growing plaque on the left ear and a 4 year history of an identical plaque on the right ear with presence of double positive CD8 / CD4 and associated with thymic carcinoma (J Cutan Pathol 2019;46:231)
    • 69 year old man with history of relapsing asymptomatic nodules confined to the left ear with extracutaneous involvement (J Cutan Pathol 2017;44:964)
    • 3 cases with largest review of the literature, including 52 additional cases (J Cutan Pathol 2016;43:125)
    • 3 cases with ear involvement and review of the literature (Am J Dermatopathol 2019;41:644)
    • First report defining the entity included 4 cases with ear involvement (Am J Surg Pathol 2007:31:1887)
    Treatment
    • Surgical excision or radiotherapy
    Microscopic (histologic) description
    • Dense, diffuse dermal infiltrate with sparing of the epidermis and presence of a thin grenz zone
    • Infiltrate is composed of medium to large size atypical lymphocytes (blast-like) with irregular nuclear membranes, small nucleoli and scant cytoplasm; signet ring cells can be present
    • No fat rimming, angiodestruction or necrosis is identified
    • Focal epidermotropism and exocytosis of atypical lymphocytes have been noted in rare cases
    • References: Arch Pathol Lab Med 2017;141:1469, Br J Dermatol 2022;186:887
    Microscopic (histologic) images

    Contributed by Roberto N. Miranda, M.D. and Alexandra Hristov, M.D.

    Dermal infiltrate with grenz zone

    Morphology of infiltrates

    Characteristic CD68 staining

    Cytotoxic immunophenotype

    Positive stains
    Negative stains
    Molecular / cytogenetics description
    • Clonal TCR gene rearrangements present by PCR testing in almost 100% of cases
    Sample pathology report
    • Skin, left ear, punch biopsies:
      • CD8+ positive cutaneous lymphoproliferative disorder (see comment)
      • Comment: The morphological features and immunohistochemical findings are most consistent with a low grade CD8+ cutaneous lymphoproliferative disorder. If the lesion is unique and localized to the ear, the findings are most consistent with primary cutaneous acral CD8+ lymphoproliferative disorder (Leukemia 2022;36:1720).
    Differential diagnosis
    • Hydroa vacciniforme-like lymphoma:
      • Can show a similar infiltrate with CD8+ cytotoxic immunophenotype but is an EBV+ T cell lymphoproliferative disease that occurs in children (mean age 8 years)
      • Atypical infiltrate is mainly located around adnexa and vessels with angiodestruction
    • Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T cell lymphoma:
      • Features skin papules or nodules with an aggressive clinical course, characterized by rapid progression, ulceration, poor response to chemotherapy and a 5 year overall survival rate of 32% (Mod Pathol 2017;30:761)
      • Morphologically characterized by full thickness pagetoid epidermotropism
      • Occasional cases show predominant dermal infiltrates; however, this is associated with atrophic epidermis and infiltration of adjacent adnexal structures (Mod Pathol 2017;30:761)
      • Tumor cells display a high Ki67 proliferation index (more than 50%)
    • Primary cutaneous gamma delta T cell lymphoma:
      • Morphologically involves the subcutaneous tissue and commonly has epidermotropism or ulceration
      • Usually the tumor cells are CD4- / CD8-; however, a few cases are CD4- / CD8+
      • Characteristically this disease features CD56 and TCR gamma expression
      • Aggressive clinical course
    • Subcutaneous panniculitis-like T cell lymphoma:
      • Solitary or multiple deeply seated plaques or subcutaneous nodules
      • Only subcutis involved, morphologically lobular panniculitis with septal sparing; adipocyte rimming with fat necrosis
      • High Ki67 proliferation index within CD8+ atypical lymphocytes rimming the fat lobules
    • Mycosis fungoides, cytotoxic:
      • Rare variant of mycosis fungoides
      • Presents as other cases of MF, with generalized, scaly patches and plaques, ulcerated nodules
      • Predilection for buttocks and sun protected areas
      • Morphologically has epidermotropism and shows Pautrier microabscesses, as well as intraepidermal vesiculation
    • Primary cutaneous peripheral T cell lymphoma, unspecified:
      • Solitary or multiple ulcerating nodules
      • Medium to large sized cells with moderate to marked pleomorphism
      • Positive expression of T cell markers, including loss of antigens; may coexpress TIA1, granzyme B or perforin
      • Aggressive clinical behavior
    Board review style question #1

    Which of the following statements is correct regarding primary cutaneous acral CD8+ lymphoproliferative disorder?

    1. Characterized by aggressive course with progressive disease and resistance to therapy
    2. Epidermotropism is usually absent
    3. Loss of expression of pan-T cell markers CD2, CD5 and CD7 is rarely observed
    4. Neoplastic cells feature a Ki67 proliferation index that ranges from 50 to 90%
    5. Restricted to the skin of the ear
    Board review style answer #1
    B. Epidermotropism is usually absent

    Comment Here

    Reference: Primary cutaneous acral CD8+ lymphoproliferative disorder
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