Oral and Cutaneous Lymphomas other than Mycosis Fungoides and Sézary Syndrome in a Mexican Cohort: Recategorization and Evaluation of International Geographical Disparities

Abstract

Background: Nonmycosis fungoides/Sézary syndrome (non-MF/SS) primary cutaneous lymphomas (PCL) are currently categorized under the 2005-World Health Organization/European Organization for Research and Treatment of Cancer (WHO-EORTC) classification for PCL. These differ in behavior from secondary cutaneous lymphomas (SCL) and to lymphomas limited to the oral cavity (primary oral lymphomas [POL]) both categorized under the 2016-WHO classification for lymphoid neoplasms.

Aims: This study aims to report the first series of non-MF/SS PCL, SCL, and POL in a Mexican cohort, examine the applicability of current classification systems and compare our findings with those from foreign cohorts.

Materials and methods: Eighteen non-MF/SS PCL, four SCL, and two POL with available tissue for morphology and immunophenotypic assessment were reclassified according to the 2005-WHO/EORTC and 2016-WHO classifications.

Results: Non-MF/SS PCLs were primarily of T-cell origin (61%) where CD30+ lymphoproliferative disorders predominated, followed by Epstein-Barr virus-induced lymphomas, and peripheral T-cell lymphomas, not otherwise specified. Primary cutaneous B-cell lymphomas (BCL) were primarily of follicle center cell origin followed by postgerminal lymphomas of the diffuse large BCL variety.

Conclusions: Most non-MF/SS PCL, SCL, and POL can be adequately categorized according to the 2005-WHO/EORTC and 2016-WHO classification systems, even when dealing with clinically atypical cases. The relative frequencies in our cohort hold closer similarities to Asian registries than from those of Europe/USA, supporting the concept of individual and/or racial susceptibility, and the notion of geographical variances in the rate of lymphomas. In particular, such disparity may arise from viral-induced lymphomas which might show partial geographical restriction.

Keywords: Primary cutaneous lymphomas; primary oral lymphomas; secondary cutaneous lymphomas.

Figure 1

Lymphomatoid papulosis. (a) Agminated erythematous papules on the trunk, (b) superficial wedge-shaped dermal infiltrate with moderate epidermotropism (H and E, ×40), (c) membranous immunoreactivity among small and large lymphocytes with anti-CD8 antibodies (×400), (d) large lymphocytes are immunoreactive with anti-CD30 antibodies (×400)

Figure 2

Primary cutaneous anaplastic large cell lymphoma. (a). Large ulcerated tumor involving the lower extremity, (b) diffuse dermal infiltrate by large mononuclear cells underlying marked acanthosis (H and E, ×100), (c) large atypical mononuclear cells with prominent nucleoli (H and E, ×400), (d) diffuse immunopositivity with anti-CD3 antibodies (×100), (e) diffuse expression of CD30 among large neoplastic cells (×200)

Figure 3

Subcutaneous panniculitis-like T-cell lymphoma. (a) Erythematous and indurated deep-seated nodule involving left cheek, (b) pleomorphic mononuclear infiltrate exclusively involving adipose lobules (H and E, ×100), (c) prominent adipocyte rimming by atypical lymphocytes (H and E, ×200), (d) membranous immunoreactivity with anti-CD8 antibodies among neoplastic lymphocytes (×200)

Figure 4

Primary cutaneous extranodal natural killer/T-cell lymphoma. (a) Indurated and ulcerated erythematous plaque involving nose, left eyelids, and cheek, (b) diffuse dermal and subcutaneous infiltrate with prominent angiocentricity and angiodestruction (H and E, ×40), (c) immunopositivity with anti-CD3 antibodies (×100), (d) prominent expression of cytotoxic proteins (granzyme B) among neoplastic cells (×100), (e) strong immunoreactivity with anti-CD56 antibodies (×200), (f) nuclear expression of Epstein–Barr virus RNA by in situ hybridization (EBER, ×100)

Figure 5

Primary cutaneous aggressive epidermotropic CD8 + T-cell lymphoma. (a and b) Disseminated flaccid bullae progressing to large ulcers, (c) prominent epidermotropism by atypical lymphocytes (H and E, ×400), (d) neoplastic epidermotropic lymphocytes show strong immunoreactivity with anti-CD8 antibodies (×400)

Figure 6

Hydroa vacciniforme-like lymphoproliferative disorder. (a) Facial edema, hemorrhagic vesicles, small crusted ulcers, and smallpox-like scars involving sun-exposed skin, (b and c) superficial perivascular infiltrate with scant epidermotropism, comprised medium-sized pleomorphic cells (b: H and E, ×100 and c: H and E, ×200), (d) neoplastic cells are immunopositive with anti-CD8 antibodies (×100), (e) nuclear expression of Epstein–Barr virus RNA by in situ hybridization (EBER, ×100)

Figure 7

Primary cutaneous follicle-center lymphoma. (a) Multinodular erythematous plaque involving scalp, forehead, and right cheek, (b) superficial and deep dermal nodular infiltrate by mononuclear cells (H and E, ×40), (c) neoplastic lymphocytes are predominantly small to mid-sized lymphocytes with indented nuclei (centrocytes) (H and E, ×200)

Figure 8

Primary cutaneous diffuse large B-cell lymphoma, leg type. (a) Multinodular erythematous plaque involving the lower extremity of an elderly woman, (b and c) pandermal and subcutaneous nonepidermotropic infiltrate by large round atypical cells (H and E, ×40 and × 100 respectively)

Figure 9

Primary oral extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue. (a) erythematous, indurated, and ulcerated plaque involving the soft palate, (b) multinodular submucosal mononuclear infiltrate (H and E, ×40), (c) perivascular infiltrate composed primarily of mid-sized indented lymphocytes, i.e., centrocyte-like marginal zone cells (H and E, ×200)