Linfoma anaplásico de células grandes asociado a implantes mamarios. Una enfermedad rara y poco conocida / Breast implant-associated anaplastic large cell lymphoma. An uncommon and little-known entity

El objetivo de esta revisión es exponer los conceptos más actualizados sobre el linfoma anaplásico de células grandes asociado a implantes mamarios, debido a que es una condición poco conocida, descrita recientemente, en 1997, y apenas reconocida en 2016 por la Organización Mundial de la Salud como una nueva entidad patológica. Se realizó una búsqueda en Pubmed y Medline de todos los artículos en inglés y español entre los años 2009 y 2019 que incluyeran las palabras clave.Hasta el 6 de junio del 2019 la FDA había recibido 573 reportes de casos con linfoma anaplásico de células grandes asociado a implantes mamarios (LACG-AIM). En 2016 la Organización Mundial de la Salud reconoció el LACG-AIM como una forma única de linfoma anaplásico de células grandes. El LACG-AIM se caracteriza por presentar un crecimiento anormal de las células T (linfocitos T), así como la expresión del receptor de membrana CD30.El desarrollo del LACG-AIM es un proceso complejo que involucra muchos factores de riesgo, incluyendo crecimiento de biofilm bacteriano, la superficie de implantes texturizados, la respuesta inmune y aspectos genéticos del paciente. La presentación clínica más frecuente es un seroma periprotésico tardío. Lo importante en el tratamiento del LACG-AIM es el diagnóstico oportuno y la exéresis quirúrgica completa de las lesiones, lo cual se relaciona con un excelente pronóstico y supervivencia a largo plazo.(AU)

The aim of this review is to describe the most up-to-date concepts regarding breast implant-associated anaplastic large cell lymphoma, because it is a rare condition, recently described in 1997 and barely recognised in 2016 by the World Health Organisation (WHO) as a new pathological entity. A search was conducted in Pubmed and Medline, including all articles in English and Spanish between 2009 and 2019 that included the keywords. Until June 6, 2019, the Food and Drug Administration (FDA) had received 573 case reports of breast implant-associated anaplastic large cell lymphoma (BIA-ALCL). In 2016, the WHO recognised BIA-ALCL as a unique form of anaplastic large cell lymphoma. BIA-ALCL is characterised by abnormal growth of T cells (T lymphocytes), as well as expression of the CD30 membrane receptor. The development of BIA-ALCL is a complex process that involves many risk factors, including the growth of bacterial biofilm, the surface of textured implants, the immune response and the patient's genetic characteristics. The most frequent clinical presentation is a late periprothetic seroma. The most important aspect in the treatment of BIA-ALCL is early diagnosis and complete surgical excision of the lesions, which is related to excellent prognosis and long-term survival.(AU)

Primary cutaneous lymphomas in patients with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL): A series of 12 cases.

BACKGROUND: Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is associated with an increased risk of a second malignancy. METHODS: We conducted a retrospective clinicopathologic review of 12 patients with CLL/SLL who developed a second lymphoma in the skin. Demographic data, clinical information, and histopathology from 31 biopsies were recorded. Cases of secondary cutaneous involvement by CLL/SLL (leukemia cutis) and non-primary cutaneous lymphomas were excluded. RESULTS: A wide variety of primary cutaneous lymphomas was identified, including classic mycosis fungoides (3), cutaneous marginal zone lymphoma (2), primary cutaneous peripheral T-cell lymphoma unspecified (2), folliculotropic mycosis fungoides (1), Sézary syndrome (1), cutaneous gamma-delta T-cell lymphoma (1), cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (1), and cutaneous anaplastic large cell lymphoma (1). A male predominance was observed, and the average age was 74.1 years. In all patients, CLL/SLL predated the development of the second lymphoma, which was aggressive in the majority of cases (58%). Aggressive cytotoxic T-cell lymphomas, generally rare neoplasms, were relatively common (30%). CONCLUSIONS: CLL/SLL patients may develop a second lymphoma in the skin, which may be aggressive. Atypical cutaneous lymphoid infiltrates in this patient population should not be assumed to represent secondary CLL/SLL involvement and require thorough immunohistochemical analysis.

ALK-positive anaplastic large T-cell lymphoma presenting primarily as a sinonasal mass with pseudoproptosis: A case report.

We report a case of anaplastic lymphoma kinase-positive anaplastic large T-cell lymphoma (ALK+ALCL) presenting primarily as a sinonasal mass with pseudoproptosis in an 11-year-old boy. The diagnosis was based on histopathological and immunohistochemical (IHC) evaluation, which is indispensable for determining tumor type. On the basis of clinicoradiological findings, provisional differential diagnoses of angiofibroma and rhabdomyosarcoma were made. Upon histopathological examination of the biopsy sent, the diagnosis of lymphoma in the sinonasal region was considered. Upon IHC, the tumor cells showed immunoreactivity for vimentin, CD45, CD30, and ALK. The tumor cells showed focal immunoreactivity for CD3 and CD68. Ki-67 labeling index was 70%. They were nonimmunoreactive for PAN cytokeratin, epithelial membrane antigen, cluster of differentiation (CD) 20, CD15, CD56, S100, smooth muscle actin, and myogenin. The diagnosis of ALK+ALCL was rendered. The studied IHC markers confirmed the histopathological diagnosis and helped in further subtyping. To the best of our knowledge, this is the first case of ALCL presenting primarily as a sinonasal mass with pseudoproptosis.

Anaplastic large cell lymphoma in a patient with MAGIC syndrome: a case and review of the literature.

Mouth and genital ulcer with inflamed cartilage syndrome (MAGIC syndrome) is a rare autoinflammatory disorder with unknown etiology. Except for the common clinical manifestations mimicking Behçet's disease and relapsing polychondritis, some other clinical entities are occasionally observed. In this report, we present a case in which a patient developed anaplastic large cell lymphoma 1 year after the diagnosis of MAGIC syndrome. Additionally, we review the clinical manifestations, management, and prognosis of MAGIC syndrome.

Living-donor liver transplantation providing an adequate chemotherapy for a pediatric patient with anaplastic large cell lymphoma complicated with liver failure due to the aggravation of biliary hepatopathy by secondary hemophagocytic lymphohistiocytosis.

Anaplastic large cell lymphoma (ALCL) accounts for 10-15% of childhood non-Hodgkin lymphoma cases; it is generally chemo-sensitive and is one of the most curable pediatric cancers. We report here a case of pediatric ALCL complicated with acute liver failure due to the aggravation of pre-existing biliary hepatopathy by lymphoma-associated hemophagocytic lymphohistiocytosis (HLH). Although the initial treatment response against ALCL was very good, poor and irreversible liver function due to biliary cirrhosis worsening by lymphoma-associated HLH prevented the patient from receiving further consolidation chemotherapies. To make matters worse, his condition was accompanied with intrahepatic fungal pseudoaneurysm and invasive fungal infection. Thus, we decided to perform an urgent living-donor liver transplantation from his father to correct the patient's liver function and make it possible to proceed with further ALCL therapy. After the living-donor liver transplantation, the patient successfully received consolidation therapy with brentuximab vedotin. To our knowledge, this may be an early reported case of a pediatric patient undergoing liver transplantation during treatment for ALCL. In most patients with HLH-associated ALCL, liver function improves when ALCL is controlled. However, acute liver failure is occasionally observed in HLH cases with pre-existing liver dysfunction. In such cases, liver transplantation should be considered to correct liver dysfunctions if the disease control of HLH is satisfactory.

Brentuximab Vedotin and High-dose Methotrexate Administrated Alternately for Refractory Anaplastic Large-cell Lymphoma With Central Nervous System Disease.

Pediatric anaplastic large-cell lymphoma (ALCL), which is characterized by strong expression of CD30, is usually responsive to multidrug chemotherapy. Brentuximab vedotin (BV) which is an anti-CD30 antibody-drug conjugate is a promising drug with effects on relapsing or refractory ALCL. However, its effects may not be sufficient for the central nervous system disease. The authors herein reported an 11-year-old boy with ALCL that progressed as central nervous system disease receiving intensive induction chemotherapy has achieved and maintained remission by BV and high-dose methotrexate administrated alternately. Alternate therapy with high-dose methotrexate may complement these shortcomings of BV to provide safe treatment without worsening adverse events.

[Acute intestinal intussusception revealing intestinal T-cell lymphoma in adults]. / Invagination intestinale aiguë révélant un lymphome T digestif chez l'adulte: à propos d'un cas.

Acute intestinal intussusception occurs primarily in infants and young children. It uncommonly occurs in adults. It can have various causes. In the vast majority of cases, it is secondary to a benign or malignant tumor. Intestinal T-cell lymphoma revealed by intestinal intussusception is very rare. We report the case of a 43-year old patient admitted with intestinal occlusion to the Emergency Department at the University Hospital Hassan II, Fes, Morocco. Abdominal CT scan showed acute intestinal intussusception associated with incarcerated bowel loop with bowel wall thickening. Treatment was based on open carcinological resection. Anatomopathological and immunohistochemical examination of the surgical specimen showed large T-cell anaplastic lymphoma. After surgery, chemotherapy was indicated to improve prognosis and to avoid a potential relapse. Intestinal intussusception rarely occurs in adults. It most often leads to the detection of an organic cause such as a tumor. This study and literature review aim to highlight the clinical and diagnostic features as well as the therapeutic approaches for this rare disease.

Linfoma anaplásico de células grandes asociado a implantes mamarios. Cuatro casos diagnosticados en la provincia de Alicante, España / Breast implant associated anaplastic large cell lymphoma. Four cases diagnosed in the province of Alicante, Spain

Introducción y Objetivo. El linfoma anaplásico de células grandes asociado a implantes mamarios es un subtipo de linfoma de células T periférico que se puede encontrar en la cápsula periprotésica, el fluido entre implante y cápsula en forma de seroma o en casos avanzados como enfermedad metastásica. Aportamos 4 casos clínicos de este tipo de linfoma diagnosticados en la provincia de Alicante, España, siendo de interés por ser escasa la incidencia reportada y pocos los trabajos científicos publicados a nivel nacional sobre esta patología. Material y método. Describimos estos 4 casos registrando: edad, antecedentes, indicación del implante, detalles de la cirugía previa, tipo de superficie y marca del implante, presentación clínica del cuadro y tratamiento administrado. Resultados. Dos casos correspondieron a cirugía estética y 2 a cirugía reconstructiva, la presentación clínica en 3 casos fue como seroma tardío y en 1 como linfadenopatía. Tres implantes eran de superficie texturizada y 1 de poliuretano. En todos los casos se realizó capsulectomía bilateral. Conclusiones. La aportación de nuestra serie contribuye a destacar la importancia de reportar los casos diagnosticados dada la escasa incidencia de la enfermedad y el reducido número de publicaciones sobre la misma en el ámbito iberolatinoamericano

Background and Objective. The anaplastic large cell lymphoma associated with breast implants is a subtype of peripheral T cell lymphoma that can be found in the periprosthetic capsule, the fluid between the implant and the capsule in the form of a seroma, or in advanced cases such as metastatic disease. We provide 4 clinical cases diagnosed in the province of Alicante, Spain, being of interest because of the low incidence reported and few published scientific papers nationwide. Methods. We describe these 4 clinical cases and recorded: age, history, implant indication, previous surgery details, implant type of surface and brand, clinical presentation of the pathology and treatment. Results. Two cases corresponded to cosmetic surgery and 2 to reconstructive surgery; clinical presentation in 3 cases was late seroma and lymphadenopathy in 1. Three implants had textured surface and one was of polyurethane. Bilateral capsulectomy was performed in all cases. Conclusions. The main contribution of our cases is to highlight the importance of informing about diagnosed cases, because of the rare incidence of the disease and the lack of publications about this theme in the Ibero-Latinamerican environment

Primer caso en la Región de Murcia (España) de linfoma anaplásico de células grandes asociado a implantes mamarios / First case in region of Murcia (Spain) of breast implant-associated anaplastic large cell lymphoma

El linfoma anaplásico de células gigantes asociado a implantes mamarios es una entidad extremadamente rara de la que, hasta enero de 2019, han sido diagnosticados 656 casos en 34 países con 17 muertes comunicadas. Se postula que su etiología es el resultado de una reacción a un cuerpo extraño como la prótesis y la inflamación crónica persistente que se deriva. Presentamos el primer caso de esta patología diagnosticado y tratado en la Región de Murcia, España. Se trata de una paciente con seroma crónico recidivante que aparece a los 5 años de la colocación de implantes mamarios tras diagnóstico y tratamiento por cáncer de mama. El diagnóstico definitivo se obtuvo en el estudio anatomopatológico e inmunohistoquímico de la cápsula periprotésica tras cirugía de retirada de implantes y capsulectomía completa bilateral. Se administró quimioterapia postoperatoria por recomendación del Comité de Tumores hospitalario

Breast implant-associated large cell anaplastic lymphoma is an extremely rare entity of which, until January 2019, 656 cases have been diagnosed in 34 countries with 17 reported deaths. It's etiology is postulated as the result of a foreign body reaction to the implant and the resultant chronic and persistent inflammation. We present the first case of this pathology diagnosed and treated in the Region of Murcia, Spain. A patient with chronic relapsing seroma that appears 5 years after breast implant placement after diagnosis and treatment for breast cancer. The definitive diagnosis was obtained in the pathological and immunohistochemical study of the periprosthetic capsule after implant removal surgery and bilateral complete capsulectomy. Postoperative chemotherapy was administered on the recommendation of the hospital Tumor Committee

Anaplastic large cell lymphoma, with 1,25(OH)2D3-mediated hypercalcemia: A case report.

Hypercalcemia due to malignant tumors including malignant lymphomas is relatively common. Among cancer patients with hypercalcemia, humoral hypercalcemia of malignancy is the most common and accounts for about 80% of all cases with hypercalcemia. 1,25-dihydroxyvitamin D3(1,25(OH)2D3)-mediated hypercalcemia is relatively rare. Although malignant lymphoma has been also reported to cause 1,25(OH)2D3-mediated hypercalcemia, it is not known whether there is any association between 1,25(OH)2D3-mediated hypercalcemia and any specific histological type of malignant lymphoma. We herein report a case of an anaplastic large cell lymphoma (ALCL), anaplastic lymphoma kinase (ALK) -negative with 1,25(OH)2D3-mediated hypercalcemia, which has never been previously reported. An 80-year-old Japanese man was admitted to our department due to acute exacerbation of hypercalcemia. He was diagnosed with ALCL, ALK-negative. Serum 1,25(OH)2D3 level was high and seemed to be associated with the lymphoma because the serum calcium and 1,25(OH)2D3 levels improved in response to chemotherapy. Histological findings showed that many CD68 positive macrophages were observed in the microenvironment of tumor cells. Lymphoma cells or tumor microenvironmental cells may produce 1,25(OH)2D3 because several previous reports showed the source of 1,25(OH)2D3 can be either lymphoma or tumor microenvironmental cells. Moreover, because 1,25(OH)2D3-mediated hypercalcemia has been reported regardless of the specific histological type of lymphoma, tumor microenvironmental cells may be involved in this condition. However, we could not identify the source of 1,25(OH)2D3 in this case. The association between 1,25(OH)2D3 production and prognosis in malignant lymphomas is yet unknown; further studies are needed to elucidate the clinical characteristics of malignant lymphoma with 1,25(OH)2D3-mediated hypercalcemia.