Oral-Cutaneous CD4-Positive T-cell Lymphoma: A Study of Two Patients

Abstract

We describe two slowly progressive cases of T-cell lymphoma that involved both acral skin and oral cavity. One patient presented with a tongue nodule, completely responded to chemotherapy and then developed recurrent lymphoma involving tongue and skin a few months later that also responded to therapy. The second patient presented with a skin nodule that spontaneously resolved without therapy, and subsequently recurred in tongue and skin a few years later. In both cases, the neoplasms were composed of atypical lymphoid cells with epidermotropism and were of T-helper cell lineage (CD4+). The initial lesions were also negative for CD30. Identical T-cell receptor gene rearrangements were detected in the initial and recurrent lesions of one case. Although these neoplasms were classified as unspecified peripheral T-cell lymphoma because of the unusual distribution of disease, both cases also had histopathologic features of mycosis fungoides. These cases are strikingly similar, and may represent an unusual clinicopathologic type of T-cell lymphoma that can hone to cutaneous and oral mucosal sites with a slowly progressive natural history.

Clinical and Histopathological Features of Folliculotropic Mycosis Fungoides: A Norwegian Patient Series

“…Two patients showed oral involvement with infiltration of the tongue. Oral lesions are reported in less than 1% of MF patients, and have been described as a distinct cutaneous lymphoma entity (19). The development of oral lesions in 2 of our FMF patients in our registry is therefore of interest.…”

Mucosal CD30-positive T-cell lymphoproliferations of the head and neck show a clinicopathologic spectrum similar to cutaneous CD30-positive T-cell lymphoproliferative disorders

“…The skin processes were mycosis fungoides (case 8) and primary cutaneous anaplastic large cell lymphoma (case 9), with mucosal disease presenting in the nasal cavity and tongue, respectively. Occasional cases of mycosis fungoides [26][27][28] and lymphomatoid papulosis 16,21,[29][30][31][32] have been reported to involve both cutaneous and mucosal sites, particularly the oral cavity. Some cases of lymphomatoid papulosis with oral involvement also showed genital involvement.…”

“…21,31 Although comparative molecular studies were not performed in most cases, identical clonal rearrangements in the cutaneous and mucosal lesions have been reported in at least two patients. 16,27 Adequate material for comparative studies was not available from the two cases in our series. The existence of patients with mucocutaneous disease suggests mucosal CD30-positive T-cell lymphoproliferations may be biologically similar to cutaneous cases, possibly reflecting molecular features contributing to epitheliotropism of the CD30-positive T cells.…”

The solitary lymphomatous papule, nodule, or tumor

“…5 There may be a subset of cutaneous T-cell lymphomas that, by current diagnostic criteria, would qualify as PTCL-U, but do not follow an aggressive course. 38 MF presenting as a single tumor or in a lymph node may be incorrectly classified as PTCL-U without the feature of epidermotropism. Treatment and prognosis differ as PTCL-U, but not MF, is treated initially with aggressive chemotherapy.…”