Review mycosis fungoides in children and adolescents. Figure 6 skin specimen from patient 6 shows typical histopathologic features of mycosis fungoides. Mycosis fungoides mf is a lowgrade cutaneous lymphoma accounting for more than half of primary cutaneous tcell lymphomas ctcls. Mycosis fungoides, also known as alibertbazin syndrome or granuloma fungoides, is the most common form of cutaneous tcell lymphoma. Symptoms include rash, tumors, skin lesions, and itchy skin. Epidemiology, etiology, genetics, and cytogenetic abnormalities. Due to the rarity of ctcl, randomized studies are lacking, and treatment is based mainly on the recent published european organisation for research and treatment of cancer guidelines. Mycosis fungoides and ctcl are often used interchangeably, which is imprecise, as mycosis fungoides is just one type of ctcl. Pmid 15128898, 2004 the pathogenesis of mycosis fungoides.
As an epidermotropic primary cutaneous t lymphoma ctcl, it may comprise of miniature or medium sized lymphocytes containing cerebriform nuclei and a t helper cell immune phenotype, although variants of a cytotoxic t lymphocyte ctl component may commonly arise. Staging guidelines from the mycosis fungoides cooperative group are presented in table 346. Atypical lymphocytes with indented cerebriform nuclei infiltrate the. Mycosis fungoides mf is well known but relatively rare tcell lymphoma of the skin. Mycosis fungoides is a rare form of cancer, but it is considered to be the most frequent form of cutaneous lymphoma. Mycosis fungoides including sezary syndrome treatment pdq. What are the histologic findings of mycosis fungoides in. Mycosis fungoides, which accounts for almost 50% of all primary cutaneous lymphomas, is an epidermotropic ctcl that is characterized by a proliferation of small to mediumsized t lymphocytes with cerebriform nuclei. Mycosis fungoides is rarely cured, but some people stay in remission for a long time. Learn more about how it is diagnosed, treated and its prognosis. Mycosis fungoides pictures, staging, prognosis, symptoms. In mycosis fungoides, the histopathology is characterised by infiltrates of malignant t cells. Mycosis fungoides mf is a rare, albeit commonest form of pctcl.
These are small, medium in size, and characteristically have irregular cerebriform nuclei. In individuals with mycosis fungoides, the skin becomes infiltrated with plaques and nodules that are composed of lymphocytes. Mycosis fungoides mf is the most common primary cutaneous t cell lymphoma, which is characterised in its early stages by epidermotropism of small to mediumsized t lymphocytes with cerebriform nuclei. Mycosis fungoides variantsclinicopathologic features. In early stages, its often treated with medicines or therapies that target just your skin. Dense dermal infiltrates of atypical t cells with cerebriform nuclei. Primary cutaneous t cell lymphoma pctcl is a spectrum of diseases composed of malignant clonal helper t lymphocytes. Mycosis fungoides symptoms, diagnosis, treatments and causes. The estimated annual incidence rate in the united states is only approximately 0. Related features include severe pruritus, alopecia, palmoplantar hyperkeratosis, and bacterial superinfection. Mycosis fungoides is a rare form of tcell lymphoma of the skin. The disease is typically slowly progressive and chronic.
Dermal infiltrate of atypical t cells with cerebriform nuclei alone or clustered in epidermis and in small sheets in dermis. Histopathologic staging of lymph nodes in mycosis fungoides and sezary syndrome a. Typical cells are lymphocytes with cerebriform and sometimes hyperchromatic nuclei, and mostly confined to the epidermis. Mycosis fungoides nord national organization for rare.
It is notable for highly symptomatic progressive skin lesions, including patches, plaques, tumors, and erytheroderma, and has a poorer prognosis at later stages. Nests of medium to large sized neoplastic lymphocytes with pleomorphic and cerebriform nuclei are observed within the epidermis pautrier microabscesses and adjacent superficial dermis h and e. Mf is defined as an epidermotropic, primary cutaneous tcell lymphoma ctcl characterized by infiltrates of small to mediumsized t lymphocytes with cerebriform nuclei. Ctcl is more common than cutaneous bcell lymphoma cbcl.
Mf d emblee is an uncommon variant of mf, which is characterised by rapid development of tumors without a patch or plaque stage disease. Mycosis fungoides mf is a clinical diagnosis that requires strong correlation with histopathologic and sometimes molecular findings to exclude benign inflammatory diseases, more aggressive primary cutaneous lymphomas, and extracutaneous lymphomas that can involve the skin. The clinical stages patch, plaque, tumour correlate with the progressive density of malignant t cells. Oct 01, 2007 the diagnosis of mycosis fungoides is based on the clinical and biopsy findings. As an epidermotropic primary cutaneous t lymphoma ctcl, it may comprise of miniature or medium sized lymphocytes containing cerebriform nuclei and a t helper cell immune phenotype. This condition may usually affect adults who are over 50 years of age but cases of children being affected have been reported 1, 2. Early stage patients present with patches and plaques in the skin and the disease may progress slowly over many years 1015 years. Diagnosis and management of mycosis fungoides cancer network. In advanced cases, ulcerated tumors and infiltration of lymph nodes by diseased cells. Most common form of cutaneous lymphoma is mycosis fungoides mf, a cutaneous tcell lymphoma, which is categorized as patch, plaque, or tumor stage.
A series of cytoarchitectural features was assessed, and differences in the distribution of. Peripheral blood involvement with mycosis fungoides or sezary syndrome mfss cells is correlated with more advanced skin stage, lymph node and visceral involvement, and shortened survival. Sezary cells are enlarged atypical lymphocytes with convoluted nuclei. Mycosis fungoides is the most common form of cutaneous tcell lymphoma ctcl. Tthe conundrum of parapsoriasis versus patch stage he. Mycosis fungoides is a frequent cutaneous lymphoma contributing to an estimated half 50% of the emerging dermal lymphomas. Cerebriform definition of cerebriform by medical dictionary. Mycosis fungoides mf is the most common form of primary cutaneous tcell lymphoma ctcl characterized by epidermotropic smalltomediumsized t lymphocytes with cerebriform nuclei. Efficacy of histologic criteria for diagnosing early mycosis. Histologically, the pattern of lymphoid infiltration in lpp is similar to spp figure 3, but the infiltrates often contain lymphocytes with cerebriform nuclei called lutzner cells similar to that seen in mf. Mf is a difficult disease to treat, which is incurable and refractory to multiple treatments.
Diagnosis remains difficult owing to mfs nonspecific skin presentation. Mycosis fungoides american journal of clinical pathology. Mycosis fungoides is the most common example of cutaneous t cell lymphoma. Mycosis fungoides is an epidermotropic primary cutaneous tcell lymphoma characterized by infiltrates of small to mediumsized t lymphocytes with cerebriform nuclei. It generally affects the skin, but may progress internally over time. Mycosis fungoides and sezary syndrome also have a formal staging system proposed by the international society for cutaneous lymphomas iscl and the european organization of research and treatment of cancer eortc. It has indolent behavior, characterized by an evolution of skin patches and plagues, and later progressing to tumorous lesions over a course of many years. Management of mycosis fungoidestype cutaneous tcell. Stage information for mycosis fungoides including sezary syndrome the stages that follow are defined by tnm classification. Mycosis fungoides mf is the most common type of cutaneous tcell lymphoma ctcl and represents nearly 50% of all primary cutaneous lymphomas. Nests in the epidermis known as pautrier microabscesses. Apr 12, 2020 in the early stages of mycosis fungoides, the histopathology is nonspecific,ref81ref82ref83ref84 and the condition is often misdiagnosed as an inflammatory disorder. Mycosis fungoides mf, the most common subtype of cutaneous t cell lymphoma ctcl, has been referred to as one of the great imitators due to the heterogeneity of its cutaneous manifestations. Mycosis fungoides mf is the most prevalent cutaneous lymphoma.
Mycosis fungoides mf is the most common variant of cutaneous t cell lymphoma and frequently presents as earlystage disease with skin patches and plaques with an indolent course, but patients experience significant morbidity from itch and disfigurement. Fine needle aspiration cytology of unilesional mycosis. The pathology of cutaneous tcell lymphoma cancer network. Wang, md key facts terminology primary cutaneous tcell lymphoma characterized by epidermotropism clinical course showing stepwise evolution of patches, plaques, and tumors clinical issues overall indolent clinical course clinical stage is most important predictor of prognosis microscopic pathology skin. Cerebriform nuclei an overview sciencedirect topics.
The term mycosis fungoides should be used only for classic cases, characterized by the evolution of patches, plaques, and tumors, or for variants with a similar clinical course. Mycosis fungoides mf is the most common primary cutaneous tcell lymphoma. Sarveswari and yesudian parapsoriasis versus mycosis fungoides are common in the indian subcontinent figure 2a. Nonmalignant inflammatory cells are usually found among the malignant cells. Mycosis fungoides is a rare form of tcell lymphoma of the skin cutaneous. Patch, plaque, tumour mycosis fungoides insight medical. Radiation oncologymycosis fungoides wikibooks, open books. Conventional mf begins as eczematoid or psoriasiform patches and. Histopathological findings include a bandlike infiltrate that involves the papillary dermis and consists of mononuclear cells with hyperchromatic, cerebriform nuclei without spongiosis. It exhibits a protracted clinical course with slow progression from slightly scaly skin lesions patches to infiltrated plaques and tumors. More detailed information about the symptoms, causes, and treatments of mycosis fungoides is available below.1239 655 1223 951 1043 1323 246 883 1431 544 226 1046 687 392 1617 364 560 1221 287 211 921 174 944 383 1199 323 1044 1403 477 945 538 277 1434 1044 259 993 1085 1361 823 1281 586 236 1337 569