Letter: Primary cutaneous marginal zone B cell lymphoma of the face: A challenging diagnosis.

Feb 2012

Oliveira A, Caetano M, Alves R, Lima M, Selores M.

Source

Department of Dermatology, Centro Hospitalar do Porto - Hospital de Santo António, Porto, Portugal.

Abstract

The primary cutaneous marginal zone B cell lymphoma is a small B cell lymphoma, including cells of the marginal zone, lymphoplasmacytic cells, and plasma cells. Clinically it manifests as erythematous or erythematous-violaceous papules, plaques, or nodules, single or multiple, most often located to the extremities. Its course is usually indolent, with a survival at 5 years of approximately 97 percent. The tumor exhibits a tendency towards local recurrence, but spread to locations outside the skin is extremely rare. We present a case report of a man, 80 years of age, with a primary cutaneous marginal B cell lymphoma of the chin, an atypical location.

Dermatology Online Journal

Cutaneous lymphoma or benign lesion?

Cutaneous lymphoma or benign lesion?

A 66-year-old man presented with an erythematous nodule on the distal ulnar aspect of his forearm, consistent with a pyogenic granuloma on inspection. The lesion had grown in diameter from 5 mm to 30 mm over a

few months, was nonulcerated and expressed a

small amount of serous luid (Figure 1A).

He reported a 30-year history of similar lesions that

had spontaneously resolved. Biopsy results from previous lesions had been consistent with inlamed seborrheic keratosis. Complete excisional biopsy of the current lesion showed a regressing CD30-positive lymphoproliferative disorder with pseudocarcinomatous hyperplasia.

We referred our patient to a lymphoma clinic for

staging and treatment. Bone marrow biopsy and

computed tomography (CT) of his neck, thorax,

abdomen and pelvis were normal. No further

treatment was required after the initial surgical

excision; however, the patient was advised to

return for routine surveillance. A subsequent

biopsy of a new lesion showed similar features

and lymphomatoid papulosis was diagnosed.

Although lymphomatoid papulosis is rare,

with an estimated incidence of one to two

instances per million, dome-shaped papules that occasionally characterize it can mimic numerous common skin conditions, including pyogenic granuloma. Lymphomatoid papulosis may also present with multiple lesions, which may be confused with reactions to insect bites and folliculitis.

These lesions must undergo excisional biopsy for accurate evaluation. Lymphomatoid papulosis is part of a spectrum of CD30-positive lymphoproliferative disorders. It presents as spontaneously regressing papules and nodules that recur over decades. Because lymphoma develops in 10%–19% of patients, regular surveillance is required.

CMAJ

Menus for managing patients with cutaneous T-cell lymphoma.

Mar 2012

Poligone B, Heald P.

Source

Department of Dermatology, University of Rochester School of Medicine Rochester, NY.

Abstract

In the management of patients with cutaneous T-cell lymphoma (CTCL), there are numerous distinct therapy options. Each of these therapies is discussed in terms of when to use it, what factors limit the success of the treatment, and what to expect. A menu is defined as a list of items from which to choose. The treatments for CTCL are presented in various menus where they are options for a particular goal in a particular setting of CTCL. The best recognized clinical scenarios of CTCL are those recognized by the staging system: limited patch plaque (T1), disseminated patch plaque (T2), erythroderma (T4), and tumor (T3). Each phase of the disease will have the menu of therapy options presented for a given goal of management.

Elsevier

Low-Dose Electron Beam Radiation and Romidepsin Therapy for Symptomatic Cutaneous T-CellLymphoma Lesions.

Feb 2012

Akilov OE, Grant C, Frye R, Bates S, Piekarz R, Geskin LJ.

Source

Department of Dermatology, University of Pittsburgh, Pittsburgh, Pennsylvania Medical Oncology Branch, Centre for Cancer Research, National Cancer Institute, Bethesda.

Abstract

Background: 

Romidepsin is a structurally unique histone deacetylase inhibitor FDA-approved for therapy of relapsed or refractory cutaneous T-cell lymphoma (CTCL). Localized electron beam radiation therapy (LEBT) is standard practice in the care of patients with chronically traumatized and painful lesions. Combinational therapy of those two modalities may be beneficial for the therapy of CTCL.

Objectives: 

To report observations on supportive LEBT utilized for isolated refractory lesions in patients on romidepsin.

Methods:  Observations during a phase 2 clinical trial sponsored by the National Cancer Institute (NCI 1312) examining the efficacy of romidepsin for patients with relapsed, refractory, or advanced CTCL, stage IB-IVA mycosis fungoides (MF) or Sézary syndrome. Skin responses were assessed by evaluation of five target lesions only. Patients with objective clinical responses in target lesions who had symptomatic non-target lesions were allowed limited localized radiation to isolated lesions for symptomatic relief. Patients who received localized radiation were not considered complete responders at any point.

Results:  Five patients with advanced MF (3 had stage IIB and 2 had stage IVA2) received localized electron beam radiation to symptomatic non-target lesions while on a protocol with romidepsin. None of these patients experienced additional or unexpected toxicity. Four of the five patients demonstrated fast and durable responses. We noted that significantly lower than standard doses of electron beam radiation effectively treated symptomatic lesions in these patients.

Conclusions:  Electron beam therapy demonstrated significant responses at very low doses without additional toxicity in patients on protocol treatment with the histone deacetylase inhibitor romidepsin. This merits formal investigation in a clinical trial for potential synergy in patients with cutaneous T-cell lymphoma.

British Association of Dermatologists.

Wiley OnLine

MDM2 Inhibitor Nutlin-3a Induces Apoptosis and Senescence in Cutaneous T-Cell Lymphoma: Role of p53. MDM2 Inhibitor Nutlin-3a Induces Apoptosis and S

MDM2 Inhibitor Nutlin-3a Induces Apoptosis and Senescence in Cutaneous T-Cell Lymphoma: Role of p53.

Mar 2012

Manfé V, Biskup E, Johansen P, Kamstrup MR, Krejsgaard TF, Morling N, Wulf HC, Gniadecki R.

Source

Department of Dermatology, Bispebjerg Hospital, Copenhagen, Denmark.

Abstract

P53 is rarely mutated in cutaneous T-cell lymphoma (CTCL) and is therefore a promising target for innovative therapeutic approaches. Nutlin-3a is an inhibitor of MDM2 (human homolog of murine double minute 2), which disrupts its interaction with p53, leading to the stabilization and activation of p53. To investigate the potential therapeutic use of nutlin-3a in CTCL, we screened CTCL lines Hut-78, SeAx, MyLa2000, Mac1, and Mac2a by measuring p53 levels after nutlin-3a treatment. In MyLa2000, Mac1, and Mac2a, we observed the increase in p53, indicating the fully functional p53. In the remaining cell lines, P53 mutation analysis identified a homozygous nonsense mutation (R196Stop in Hut-78) and a homozygous missense mutation (G245S in SeAx). In MyLa2000, Mac1, and Mac2a carrying wild-type P53, nutlin-3a induced apoptosis and senescence demonstrated by permanent G0/G1 cell-cycle block and expression of the senescence-associated β-galactosidase. This effect was abolished in cells in which p53 was silenced by small interfering RNA. Sézary cells lack functional p53 and were resistant to nutlin-3a. However, nutlin-3a potentiated the efficacy of conventional chemotherapeutics not only in cells with intact p53 but also in Hut-78, SeAx, and Sézary cells. Thus, targeting p53 by nutlin-3a may constitute a therapeutic approach in CTCL because of increased apoptosis and senescence of tumor cells.Journal of Investigative Dermatology advance online publication, 1 March 2012; doi:10.1038/jid.2012.10.

PubMed