CLINICAL, IMAGING AND PATHOLOGICAL ASPECTS OF MAMMARY LYMPHOMAS SECONDARY TO MALIGNANT T- CELL CUTANEOUS LYMPHOMAS

2021 ◽  
Vol 2 (1) ◽  
pp. 45-54
Author(s):  
Cristian Lungulescu ◽  
Georgiana-Cristiana Camen ◽  
Raluca-Elena Nica ◽  
Viorel Biciusca ◽  
Teodor-Nicusor Sas

Primary cutaneous lymphomas rank as the second most common clinical form of extranodal non-Hogdkin malignant lymphomas. Among non-Hodgkin malignant skin T-cell lymphomas, Mycosis Fungoides (MF) is the most frequent clinical occurence. The MF lymphoma originates in skin-homing helper T-cells, which express the CD4 + marker, showing chronic evolution, with recurrent lesions. In advanced stages, patients with Mycosis Fungoides may experience severe/extensive skin lesions or extracutaneous localizations of the disease. The secondary breast lymphoma is more common in non-Hodgkin malignant lymphoma than in Hodgkin lymphoma. Among the mammographic characteristics of breast lymphoma we mention: oval or round tumor mass, with well-defined or indistinct margins, absence of intratumoral calcifications, presence of intramammary lymph nodes, supra-adjacent skin thickening and lymphedema that causes diffuse increase in breast density. The ultrasound features of breast lymphoma run as follows: it is oval or round in shape, with well-defined or indistinct margins, which in Doppler ultrasound are identified as hypervascularized masses. The description of the imaging features of mammary lymphomas secondary to cutaneous T-cell lymphomas is required before performing the breast core-needle biopsy.

Blood ◽  
1997 ◽  
Vol 90 (4) ◽  
pp. 1636-1642 ◽  
Author(s):  
J. Marcus Muche ◽  
Ansgar Lukowsky ◽  
Khusru Asadullah ◽  
Sylke Gellrich ◽  
Wolfram Sterry

Abstract Clonal T cells have been demonstrated in skin lesions of all stages of cutaneous T-cell lymphomas (CTCLs). However, there are conflicting data regarding the CTCL stage at which dissemination of clonal cells into peripheral blood occurs. Although the multifocal occurrence of cutaneous CTCL lesions and T-cell recirculation suggest an early appearance of neoplastic cells in the blood, circulating clonal T cells have only been detected in advanced stages. We investigated their occurrence by a highly sensitive polymerase chain reaction (PCR) assay amplifying T-cell receptor γ rearrangements and subsequent heteroduplex temperature gradient gel electrophoresis (HD-TGGE) of the amplification products. Circulating clonal T cells were found in 26 of 45 patients with mycosis fungoides (MF ), six of seven with Sezary's syndrome (SS), 10 of 13 pleomorphic CTCLs, and three of four unclassified CTCLs. Corresponding skin specimens carried clonal T cells in 29 of 40 MF, three of four SS, 12 of 12 pleomorphic, and two of two unclassified CTCL patients. Except for the blood specimen of a psoriatic patient, all samples of 60 controls (psoriasis vulgaris, atopic dermatitis, and healthy volunteers) revealed polyclonal amplification products. In 30 of 32 CTCL patients carrying a clonal rearrangement in blood and skin, identity of both clones was indicated by HD-TGGE and confirmed by sequencing six of these cases. We found an unexpected high frequency of identical clonal T cells in peripheral blood and skin of CTCL patients, including early stages of MF. This supports the concept of an early systemic disease in CTCL and raises new questions concerning the pathogenesis.


2019 ◽  
Vol 22 (3-4) ◽  
pp. 68-72
Author(s):  
Olga Yu. Olisova ◽  
D. R Amshinskaya ◽  
E. M Anpilogova

Cutaneous T cell lymphomas (CTCL) are a clinically and morphologically heterogeneous group of cutaneous malignant tumors, caused by monoclonal proliferation of lymphoid tissue cells in the skin. They are responsible for about 80% of all primary cutaneous lymphomas, while cutaneous lymphomas are responsible for 2% of all dermatological diseases. The incidence of CTCL is now increasing all over the world. For this reason, CTCL has to be diagnosed on the early stage to improve course of the disease. Mycosis fungoides, the most frequent CTCL variant, is usually diagnosed basing on clinical, histological, immunohistochemical and molecular findings. However, it can imitate other chronic dermatoses, which makes it difficult to diagnose. The article presents a review of the current literature data on new diagnostic markers of cutaneous T-cell lymphomas.


Blood ◽  
2006 ◽  
Vol 107 (6) ◽  
pp. 2440-2445 ◽  
Author(s):  
Kei-ichi Yamanaka ◽  
Rachael Clark ◽  
Benjamin Rich ◽  
Rebecca Dowgiert ◽  
Kazuki Hirahara ◽  
...  

AbstractCutaneous T-cell lymphomas (CTCLs) are malignancies of T cells that have a special affinity for the skin. We have previously reported that much of the T-cell receptor repertoire is altered in CTCL, and both malignant and nonmalignant clones are numerically expanded, presumably in response to T-cell trophic cytokines. We therefore examined levels of the T-cell trophic cytokines IL-2, IL-4, IL-7, IL-12, IL-13, and IL-15 in plasma in 93 CTCL patients and healthy controls. Only IL-7 levels were elevated in CTCL. We next looked at lesional skin from patients with CTCL and found elevated levels of IL-7 mRNA. Explant cultures of normal and lesional CTCL skin biopsies revealed significantly more IL-7 protein production in CTCL skin. Additionally, cultures of CTCL skin released greater numbers of T cells than normal skin; this was blocked by the addition of an IL-7 neutralizing antibody. Finally, these cultures induced proliferation of normal peripheral skin-homing T cells that were added to the cultures. These observations led us to postulate that IL-7 produced by skin cells contributes to the survival and proliferation of T cells within skin lesions and is likely the source of elevated circulating IL-7 in CTCL. (Blood. 2006;107:2440-2445)


Blood ◽  
2009 ◽  
Vol 113 (1) ◽  
pp. 127-136 ◽  
Author(s):  
Remco van Doorn ◽  
Marloes S. van Kester ◽  
Remco Dijkman ◽  
Maarten H. Vermeer ◽  
Aat A. Mulder ◽  
...  

Abstract Mycosis fungoides (MF), the most common cutaneous T-cell lymphoma, is a malignancy of mature, skin-homing T cells. Sézary syndrome (Sz) is often considered to represent a leukemic phase of MF. In this study, the pattern of numerical chromosomal alterations in MF tumor samples was defined using array-based comparative genomic hybridization (CGH); simultaneously, gene expression was analyzed using microarrays. Highly recurrent chromosomal alterations in MF include gain of 7q36, 7q21-7q22 and loss of 5q13 and 9p21. The pattern characteristic of MF differs markedly from chromosomal alterations observed in Sz. Integration of data from array-based CGH and gene-expression analysis yielded several candidate genes with potential relevance in the pathogenesis of MF. We confirmed that the FASTK and SKAP1 genes, residing in loci with recurrent gain, demonstrated increased expression. The RB1 and DLEU1 tumor suppressor genes showed diminished expression associated with loss. In addition, it was found that the presence of chromosomal alterations on 9p21, 8q24, and 1q21-1q22 was associated with poor prognosis in patients with MF. This study provides novel insight into genetic alterations underlying MF. Furthermore, our analysis uncovered genomic differences between MF and Sz, which suggest that the molecular pathogenesis and therefore therapeutic requirements of these cutaneous T-cell lymphomas may be distinct.


Blood ◽  
1997 ◽  
Vol 90 (4) ◽  
pp. 1636-1642 ◽  
Author(s):  
J. Marcus Muche ◽  
Ansgar Lukowsky ◽  
Khusru Asadullah ◽  
Sylke Gellrich ◽  
Wolfram Sterry

Clonal T cells have been demonstrated in skin lesions of all stages of cutaneous T-cell lymphomas (CTCLs). However, there are conflicting data regarding the CTCL stage at which dissemination of clonal cells into peripheral blood occurs. Although the multifocal occurrence of cutaneous CTCL lesions and T-cell recirculation suggest an early appearance of neoplastic cells in the blood, circulating clonal T cells have only been detected in advanced stages. We investigated their occurrence by a highly sensitive polymerase chain reaction (PCR) assay amplifying T-cell receptor γ rearrangements and subsequent heteroduplex temperature gradient gel electrophoresis (HD-TGGE) of the amplification products. Circulating clonal T cells were found in 26 of 45 patients with mycosis fungoides (MF ), six of seven with Sezary's syndrome (SS), 10 of 13 pleomorphic CTCLs, and three of four unclassified CTCLs. Corresponding skin specimens carried clonal T cells in 29 of 40 MF, three of four SS, 12 of 12 pleomorphic, and two of two unclassified CTCL patients. Except for the blood specimen of a psoriatic patient, all samples of 60 controls (psoriasis vulgaris, atopic dermatitis, and healthy volunteers) revealed polyclonal amplification products. In 30 of 32 CTCL patients carrying a clonal rearrangement in blood and skin, identity of both clones was indicated by HD-TGGE and confirmed by sequencing six of these cases. We found an unexpected high frequency of identical clonal T cells in peripheral blood and skin of CTCL patients, including early stages of MF. This supports the concept of an early systemic disease in CTCL and raises new questions concerning the pathogenesis.


2018 ◽  
Vol 142 (1) ◽  
pp. 139-142 ◽  
Author(s):  
Lhara Sumarriva Lezama ◽  
Dita Gratzinger

Primary cutaneous lymphomas are defined as non-Hodgkin lymphomas that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. Mycosis fungoides is the most common type of primary cutaneous T-cell lymphoma, representing almost 50% of primary cutaneous T-cell lymphomas, and primary cutaneous CD30+ T-cell lymphoproliferative disorders are the second most common group (30%). Transformed mycosis fungoides is usually CD30+ and can involve multiple nodal sites; other primary cutaneous CD30+ T-cell lymphoproliferative disorders can also involve draining regional nodes. Nodal involvement by CD30+ T-cell lymphoproliferative disorders can mimic classical Hodgkin lymphoma, which can aberrantly express T-cell antigens. The aim of this article is to briefly review salient clinical, histologic, immunophenotypic, and molecular features that can be used to distinguish lymph node involvement by CD30+ cutaneous T-cell lymphomas and lymphoproliferative disorders from classical Hodgkin lymphoma, a clinically important differential diagnosis that represents a challenging task for the pathologist.


2022 ◽  
Vol 13 (1) ◽  
pp. 116-117
Author(s):  
Fatima Azzahra El Gaitibi ◽  
Sara Oulad Ali ◽  
Jihane Belcadi ◽  
Kaoutar Znati ◽  
Mariame Meziane ◽  
...  

Sir, Mycosis fungoides is a primary cutaneous T–cell lymphoma, secondary clonal proliferation of mature skin-homing T cells, mostly CD4-positive, with a predilection for involving the epidermis. It is an indolent lymphoma that progresses over several years and represents 50% of primary cutaneous T-cell lymphomas [1]. Its clinical presentation is variable, thus leading to several clinical variants. Herein, we describe a rare variant of mycosis fungoides: pityriasis lichenoid-like mycosis fungoides. A 45-year-old female was referred to our department with a papular rash evolving for the last year without regression. The patient had a history of breast carcinoma in complete remission for two years. A clinical examination revealed erythematous, scaly, non-itchy papules covering the entire body but sparing the face (Figs. 1 and 2). There was no scalp involvement or associated lymphadenopathy. Based on the clinical presentation, the suggested diagnosis was pityriasis lichenoid. A histological examination revealed Pautrier’s microabscesses, atypical lymphocyte infiltration along the basal layer and papillary dermis, and prominent epidermotropism (Fig. 3). There was pilotropism without mucin. Besides, hyperkeratosis with focal parakeratosis and perivascular infiltrate were noted. An immunohistochemical analysis revealed infiltrates of T cells expressing CD3, CD2, CD5, and a predominance of CD4-positive T cells in the epidermis compared to CD8-positive T cells. CD7 and CD30 were, however, negative. These findings were consistent with pityriasis lichenoid-like mycosis fungoides. The patient was classified as a IB stage and received UVB phototherapy with good progress.


Hematology ◽  
2006 ◽  
Vol 2006 (1) ◽  
pp. 323-330 ◽  
Author(s):  
Steven T. Rosen ◽  
Christiane Querfeld

Abstract Primary cutaneous T-cell lymphomas (CTCLs) encompass a clinically and biologically heterogeneous group of non-Hodgkin lymphomas (NHLs) defined by clonal proliferation of skin-homing malignant T lymphocytes and natural killer cells. They account for up to 75% to 80% of all cutaneous lymphomas. The current WHO-EORTC classification of cutaneous lymphomas with primary cutaneous manifestations lists 13 entities. The most common subtypes—mycosis fungoides, Sézary syndrome, primary cutaneous anaplastic large cell lymphoma, and lymphomatoid papulosis—which represent approximately 95% of CTCLs, will be discussed in the following review. Each entity has unique biological characteristics and clinical course. Topical and/or systemic therapies are employed based on the stage of the disease and the tempo of progression.


2020 ◽  
Vol 10 (1) ◽  
pp. 17
Author(s):  
Iris Wohlmuth-Wieser ◽  
Joel M. Ramjist ◽  
Neil Shear ◽  
Raed Alhusayen

The diagnosis of cutaneous T-cell lymphomas (CTCL) is frequently delayed by a median of three years and requires the clinical evaluation of an experienced dermatologist and a confirmatory skin biopsy. Dermoscopy and high-frequency ultrasound (HFUS) represent two non-invasive diagnostic tools. While dermoscopy is inexpensive and widely used for the diagnosis of melanoma and non-melanoma skin cancers, HFUS of skin lymphomas represents a novel diagnostic approach that is not yet implemented in the routine dermatologic practice. The aim of our study was to prospectively assess skin lesions of patients with either CTCL patches or plaques with dermoscopy and HFUS and to compare the findings with atopic dermatitis (AD) and psoriasis. Thirteen patients with an established diagnosis of CTCL, psoriasis, or AD were studied: Dermoscopy features including spermatozoa-like structures and the presence of white scales could assist in differentiating between early-stage CTCL and AD. HFUS measurements of the skin thickness indicated increased epidermal-, thickness in CTCL, and psoriasis compared with AD. Our results support the use of dermoscopy as a useful tool to diagnose CTCL. HFUS could augment the dermatologic assessment, but further studies will be needed to define standardized parameters.


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