RESUMO
BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years. PATIENTS AND METHODS: RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years. RESULTS: Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 [67.8%]), phototherapy (890 patients [44.1%]), surgery (412 patients [20.4%]), and radiotherapy (384 patients [19%]). CONCLUSION: The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data.
Assuntos
Dermatologia , Linfoma Cutâneo de Células T , Micose Fungoide , Neoplasias Cutâneas , Venereologia , Masculino , Humanos , Pessoa de Meia-Idade , Feminino , Linfoma Cutâneo de Células T/diagnóstico , Linfoma Cutâneo de Células T/epidemiologia , Linfoma Cutâneo de Células T/terapia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/terapia , Sistema de Registros , Micose Fungoide/patologiaRESUMO
BACKGROUND: Reliable prognostic factors for patients with primary cutaneous anaplastic large cell lymphoma (PCALCL) are lacking. OBJECTIVE: To identify prognostic factors for specific survival in patients with PCALCL. METHODS: Using the convenience sampling method, patients with PCALCL diagnosed from May 1986 to August 2017 in 16 University Departments were retrospectively reviewed. RESULTS: One hundred eight patients were included (57 males). Median age at diagnosis was 58 years. All of them showed T1-3N0M0 stages. Seventy per cent of the cases presented with a solitary lesion, mostly at the limbs. Complete response rate after first-line treatment was 87%, and no advantage was observed for any of them (surgery, radiotherapy, chemotherapy or other approaches). Nodal and visceral progression rate was 11% and 2%, respectively. 5-year specific survival (SSV) reached 93%; 97% for T1 patients and 84% for T2/T3 patients (P = 0.031). Five-year SSV for patients developing early cutaneous relapse was 64%; for those with late or no relapse, 96% (P = 0.001). Estimated median SSV for patients showing nodal progression was 103 months (95% CI: 51-155 months); for patients without nodal progression, estimated SSV did not reach the median (P < 0.001). Nodal progression was an independent predictive parameter for shorter survival (P = 0.011). CONCLUSION: Multiple cutaneous lesions at presentation, early skin relapse and nodal progression portrait worse prognosis in patients with PCALCL.
Assuntos
Linfoma Anaplásico Cutâneo Primário de Células Grandes/mortalidade , Linfoma Anaplásico Cutâneo Primário de Células Grandes/patologia , Progressão da Doença , Feminino , Humanos , Metástase Linfática , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Prognóstico , Estudos Retrospectivos , Espanha , Taxa de SobrevidaRESUMO
BACKGROUND: Data regarding response to treatment in lymphomatoid papulosis (LyP) are scarce. AIM: To assess the daily clinical practice approach to LyP and the response to first-line treatments. METHODS: This was a retrospective study enrolling 252 patients with LyP. RESULTS: Topical steroids, methotrexate and phototherapy were the most common first-line treatments, prescribed for 35%, 20% and 14% of the patients, respectively. Complete response (CR) was achieved in 48% of treated patients. Eczematous lesions significantly increased relative risk (RR) of not achieving CR (RR = 1.76; 95% CI 1.16-2.11). Overall median time to CR was 10 months (95% CI 6-13 months), and 78% of complete responders showed cutaneous relapse; both results were similar for all treatment groups (P > 0.05). Overall estimated median disease-free survival (DFS) was 11 months (95% CI 9-13 months) but DFS for patients treated with phototherapy was 23 months (95% CI 10-36 months; P < 0.03). Having the Type A LyP variant (RR = 2.04; 95% CI 0.96-4.30) and receiving a first-line treatment other than phototherapy (RR = 5.33; 95% CI 0.84-33.89) were significantly associated with cutaneous early relapse. Of the 252 patients, 31 (13%) had associated mycosis fungoides unrelated to therapeutic approach, type of LyP or T-cell receptor clonality. CONCLUSIONS: Current epidemiological, clinical and pathological data support previous results. Topical steroids, phototherapy and methotrexate are the most frequently prescribed first-line treatments. Although CR and cutaneous relapse rates do not differ between them, phototherapy achieves a longer DFS. Presence of Type A LyP and use of topical steroid or methotrexate were associated with an increased risk of early relapse.
Assuntos
Antimetabólitos Antineoplásicos/uso terapêutico , Papulose Linfomatoide/tratamento farmacológico , Metotrexato/uso terapêutico , Fototerapia , Neoplasias Cutâneas/tratamento farmacológico , Esteroides/uso terapêutico , Administração Tópica , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Intervalo Livre de Doença , Feminino , Humanos , Lactente , Papulose Linfomatoide/mortalidade , Papulose Linfomatoide/terapia , Masculino , Pessoa de Meia-Idade , Micose Fungoide/mortalidade , Neoplasias Primárias Múltiplas , Receptores de Antígenos de Linfócitos T , Estudos Retrospectivos , Neoplasias Cutâneas/mortalidade , Neoplasias Cutâneas/terapia , Adulto JovemAssuntos
Inibidores da Angiogênese/efeitos adversos , Anticorpos Monoclonais/efeitos adversos , Toxidermias/diagnóstico , Toxidermias/etiologia , Adenocarcinoma/tratamento farmacológico , Adenocarcinoma/secundário , Administração Tópica , Alopurinol/administração & dosagem , Alopurinol/uso terapêutico , Inibidores da Angiogênese/administração & dosagem , Inibidores da Angiogênese/uso terapêutico , Anticorpos Monoclonais/administração & dosagem , Anticorpos Monoclonais/uso terapêutico , Anticorpos Monoclonais Humanizados , Bevacizumab , Neoplasias do Colo/tratamento farmacológico , Neoplasias do Colo/patologia , Toxidermias/tratamento farmacológico , Sequestradores de Radicais Livres/administração & dosagem , Sequestradores de Radicais Livres/uso terapêutico , Humanos , Injeções Intravenosas , Neoplasias Hepáticas/tratamento farmacológico , Neoplasias Hepáticas/secundário , Masculino , Pessoa de Meia-Idade , Pomadas , Resultado do TratamentoRESUMO
BACKGROUND: The presence of a prominent granulomatous tissue reaction in skin biopsies from primary cutaneous or systemic malignant lymphomas with secondary cutaneous involvement is a rare but well-known phenomenon. OBJECTIVE: This paper aims to characterize and study a series of cutaneous lymphomas showing a prominent granulomatous component. PATIENTS AND METHODS: The clinical, histopathological and evolutive features of granulomatous variants of mycosis fungoides (5 patients, 2 of them associating 'granulomatous slack skin' features), Sézary syndrome (1 patient), CD30(+) cutaneous T-cell lymphoma (2 patients), CD4(+) small/medium pleomorphic cutaneous T-cell lymphoma (1 patient), primary cutaneous B-cell lymphoma (3 patients) and peripheral T-cell lymphoma with secondary epithelioid granulomatous cutaneous involvement (4 patients) were reviewed. RESULTS: The observed features were clinically non-distinctive. Only those cases presenting with granulomatous slack skin features were clinically suspected (2 patients). Non-necrotizing granulomata (11 patients) and granuloma annulare-like (4 patients) were the most frequently observed histopathological patterns. In five cases, no diagnostic lymphomatous involvement was initially observed. From our series, no definite conclusions regarding prognosis could be established. CONCLUSION: The diagnosis of cutaneous lymphoma may be difficult when a prominent cutaneous granulomatous inflammatory infiltrate obscures the true neoplastic nature of the condition. However, the presence of concomitant lymphoid atypia may help to suspect the diagnosis. In doubtful cases, the clinical evolution and the demonstration of a monoclonal lymphoid B- or T-cell population may lead to a definite diagnosis.
Assuntos
Linfoma/patologia , Neoplasias Cutâneas/patologia , Adulto , Idoso , Feminino , Humanos , Imunofenotipagem , Linfoma/classificação , Linfoma/imunologia , Masculino , Pessoa de Meia-Idade , Neoplasias Cutâneas/imunologiaRESUMO
BACKGROUND: Smooth-muscle hamartoma is an uncommon, usually congenital cutaneous hyperplasia of the arrectores pilorum muscles. When it is acquired, it may be confused with Becker's nevus with a prominent smooth-muscle component. Both entities can be considered polar forms of a spectrum of dermal smooth muscle hyperplasia. We report three cases of acquired smooth-muscle hamartoma in adult patients. CASE REPORTS: Three healthy women in their fifties presented with adult-onset asymptomatic, multiple small papules grouped on circumscribed areas of the trunk. Results of biopsy specimens showed an excess of haphazardly oriented smooth-muscle bundles in the mid to lower dermis with an unremarkable overlying epidermis. Immunohistochemical studies showed two components in this proliferation: a smooth muscle cell population expressing vimentin and smooth muscle actin, and another population of CD34 positive dendritic cells, interspersed among the former. DISCUSSION: Proliferation of CD34+ dermal dendritic cells has been recently described as an additional component in the proliferation of smooth muscle cells, hair follicles and nerve fascicles that constitute congenital smooth muscle hamartomas. The results of immunohistochemical studies in our three cases extend this observation to the acquired variant of cutaneous smooth muscle hamartoma, which can be included in the expanding spectrum of diseases, recently characterized by the presence of a dermal proliferation of CD34 + cells.
Assuntos
Hamartoma/patologia , Músculo Liso/patologia , Feminino , Humanos , Pessoa de Meia-Idade , Doenças Musculares/patologiaRESUMO
BACKGROUND: Multiple facial angiofibromas are considered a characteristic and pathognomonic feature of tuberous sclerosis. In contrast, the observation of localized multiple angiofibromas limited to one side of the face is a uncommon. OBSERVATIONS: We cared for two patients with multiple clustered unilateral angiofibromas (although one patient also presented a few angiofibromas on the other side) without other features associated with the sclerosis tuberous complex. DISCUSSION: The possibility that these patients presented an independent disorder or a minor form of tuberous sclerosis is discussed.
Assuntos
Angiofibroma/diagnóstico , Neoplasias Faciais/diagnóstico , Neoplasias Primárias Múltiplas/diagnóstico , Neoplasias Cutâneas/diagnóstico , Esclerose Tuberosa/diagnóstico , Angiofibroma/patologia , Criança , Neoplasias Faciais/patologia , Feminino , Humanos , Masculino , Neoplasias Primárias Múltiplas/patologia , Pele/patologia , Neoplasias Cutâneas/patologiaRESUMO
INTRODUCTION: The classification of Langerhans' cell histiocytosis into 5 forms does not cover all types of clinical presentations. We observed a patient with inborn Langerhans' cell histiocytosis involving the skin and bone tissue. The clinical course was benign during the first two years of the patient's life. CASE REPORT: Skin lesions noted at birth resolved spontaneously but recurred twice in a more benign form. Histology examination showed "band" infiltration of the papillary derma composed of Langerhans' cells in direct contact with the basal epidermal layer and a few eosinophils. Immunolabelling was intensely positive for protein S100 confirming the diagnosis of Langerhans' cell histiocytosis. Extension was limited to a lytic lesion in the lower part of the left tibia which was treated by curettage. DISCUSSION: In our opinion, Langerhans' cell histiocytosis covers a wide range of clinical presentations. Our case was remarkable because of the benign course despite its congenital nature and dissemination to skin and bone. Prolonged surveillance of Langerhans' cell histiocytosis is required for patients without any apparent prognosis factors.
Assuntos
Doenças Ósseas/congênito , Granuloma Eosinófilo/congênito , Histiocitose de Células de Langerhans/congênito , Dermatopatias/congênito , Doenças Ósseas/diagnóstico por imagem , Feminino , Histiocitose de Células de Langerhans/patologia , Humanos , Recém-Nascido , Radiografia , Remissão Espontânea , Dermatopatias/patologia , TíbiaRESUMO
We report a 42-year-old white bisexual man with disseminated Kaposi's sarcoma limited to the skin and gastrointestinal tract. Results of several serum tests for human immunodeficiency virus (HIV) antibodies have been negative. The CD4/CD8 ratio has remained normal, and his Kaposi's sarcoma has had a benign clinical course during 30 months of follow-up. Similar reports of disseminated Kaposi's sarcoma with a benign clinical course in homosexual or bisexual men without demonstrable HIV infection are reviewed. Some cellular immune impairment that might be more prevalent in the homosexual population may be implicated in the pathogenesis of this type of Kaposi's sarcoma.
Assuntos
Bissexualidade , Sarcoma de Kaposi/patologia , Neoplasias Cutâneas/patologia , Síndrome da Imunodeficiência Adquirida/complicações , Adulto , Antígenos CD/análise , Humanos , Ativação Linfocitária , Masculino , Neoplasias Penianas/complicações , Neoplasias Penianas/imunologia , Neoplasias Penianas/patologia , Sarcoma de Kaposi/complicações , Sarcoma de Kaposi/imunologia , Neoplasias Cutâneas/complicações , Neoplasias Cutâneas/imunologiaRESUMO
We report a large family with leiomyomatosis cutis et uteri. Sixty-four percent of the females were involved; 18% had only uterine myomas, 10% had only cutaneous piloleiomyomas, and 36% had both. Five patients (45%) had to have an hysterectomy before age 35. Management of female patients having leiomyomatosis cutis should include a periodical gynecological examination in order to rule out the presence of uterine myomas.