Follicular dendritic cell sarcoma aggravated by hyaline-vascular Castleman's disease in association with paraneoplastic pemphigus: study of the tumor and successful treatment

Abstract The authors have successfully treated and monitored a case of paraneoplastic pemphigus in association with follicular dendritic cell sarcoma aggravated by hyaline-vascular Castleman's disease. The patient was a 56-year-old female who presented with recalcitrant erosive lichen planus of the oral cavity, tongue, and genital mucosa, along with polymorphous eruptions throughout her body. Histological examination of the cutaneous lesions, indirect immunofluorescence on rat bladder epithelium, and western blot of human keratinocyte proteins identified anti-epidermal antibodies in the patient's serum. Positron emission tomography and computed tomography scans found a mass in her retroperitoneal region. Pathology and immunohistochemistry investigation further corroborated the diagnosis of follicular dendritic cell sarcoma originated from hyaline-vascular Castleman's disease. Complete remission was achieved and the patient has been monitored for four years.

Hiperplasia linfoide ileo-colónica en pacientes pediátricos / Ileo-colonic lymphoid hyperplasia in pediatric patients

Introducción: La Hiperplasia nodular linfoide gastrointestinal constituye una entidad infrecuente con manifestaciones clínicas diversas y con mayor frecuencia en la edad pediátrica. Objetivo: Caracterizar clínica, endoscópica e histológicamente a los pacientes pediátricos con hiperplasia linfoide de colon e íleon terminal diagnosticados en el Instituto de Gastroenterología. Material y Métodos: Se realizó un estudio descriptivo, transversal, en el periodo comprendido entre 2014 y 2016 en el Instituto de Gastroenterología. La muestra estuvo constituida por 50 pacientes, quienes cumplieron los criterios de inclusión y exclusión. Se evaluaron variables demográficas, clínicas, endoscópicas e histológicas, así como el comportamiento de la comorbilidad con enfermedades malignas, enfermedades inflamatorias intestinales, giardiosis, trastornos de la respuesta inmunohumoral y alergias alimentarias. Resultados: El sexo masculino, entre 7-10 años y el color de la piel blanca fueron los más frecuentes. El sangrado rectal fue el síntoma principal (62 por ciento) y la localización a nivel del íleon terminal en 69 por ciento, no se relacionó con enfermedad maligna, hubo tres pacientes con diagnóstico de hiperplasia linfoide de íleon terminal y colitis ulcerosa. El 74 por ciento presentó aspecto nodular por histología y 60 por ciento se le diagnosticó Giardia lamblia, en la evaluación inmunohumoral predominó los pacientes sin inmunocompromiso (78 por ciento), el Prick Test fue positivo en 60 por ciento, sobre todo, a la leche de vaca. Conclusiones: La manifestación clínica que predominó fue el sangrado rectal, endoscópicamente la localización en íleon y la forma nodular por histología. No encontramos comorbilidades con enfermedades malignas y fueron más frecuentes los trastornos alérgicos y parasitarios que las alteraciones inmunológicas(AU)

Introduction: Lymphoid nodular hyperplasia of the gastrointestinal tract is an uncommon entity with diverse clinical manifestations, which is more frequent in the pediatric age. Objective: To characterize clinically, endoscopically, and histologically, those pediatric patients with lymphoid hyperplasia of the colon and terminal ileum diagnosed in the Institute of Gastroenterology. Material and Methods: A descriptive, cross-sectional study was carried out during the period between 2014 and 2016 in the Institute of Gastroenterology. The sample consisted of 50 patients who met the inclusion and exclusion criteria. Demographic, clinical, endoscopic and histological variables were evaluated, as well as the behavior of comorbidity with malignant diseases, inflammatory bowel diseases, giardiasis, disorders of the humoral immune response and food allergies. Results: The male sex, the age group between 7-10 years, and the white skin color were the most frequent. Rectal bleeding was the main symptom (62 percent), and the location of the lesions in the terminal ileum was observed in 69 percent of the patients. There was no relationship between lymphoid hyperplasia and malignant disease, but three patients were diagnosed with lymphoid hyperplasia of the terminal ileum, and ulcerative colitis. 74 percent of the biopsies presented a nodular variety, and 60 percent of the patients were diagnosed with Giardia lamblia; the cases that were not immunocompromised prevailed in the evaluation of humoral immune response (78 percent); the Prick Test was positive in 60 percent of children, especially to cow's milk. Conclusions: The predominant clinical manifestation was rectal bleeding; the localization was in the ileum, which was seen by endoscopic procedure; and the nodular form was demonstrated by histology. We did not find comorbidities with malignant diseases, and allergic and parasitic disorders were more frequently diagnosed than immunological alterations(AU)

Linfoma de células grandes B originado en enfermedad de Castleman: reporte de un caso y revisión de la literatura / Large B-cell lymphoma coexisting with Castleman's disease: report of one case

We report a 73-year-old female patient with Castleman's disease coexistent with large B cell type non-Hodgkin's lymphoma in a right axillary lymphadenopathy. An excisional biopsy was performed: microscopically, the lymph node revealed the presence of numerous plasma cells and small lymphoid cells characteristic of Castleman's disease. An analysis of another portion of the specimen revealed lymphoid cells with large abnormal nuclei gathered locally that were CDD 79+, CD 38+ and MUM-1+ as well as positive for Kaposi sarcoma-associated herpesvirus and negative for Epstein Barr virus encoded RNA-1 (EBER).

A case of Paraneoplastic Pemphigus associated with Castleman's disease / Um caso de Penfigo Paraneoplastico (PNP) associado a doenca de Castleman

We present a case of PNP associated with Castleman's Disease. We have also reviewed the literature and described the characteristics of the two associated diseases. Gene clonal rearrangement was done to help diagnosis. We used, in addition, stereotactic radiosurgery which, as far as we know, has never before been employed to treat PNP associated with Castleman's Disease. This produced a good response, suggesting that it might be a good alternative treatment for PNP associated with Castleman's Disease when it is too difficult to operate.

Apresentamos um caso de PNP associada à doença de Castleman.Também revisamos a literatura, e referenciamos as características das duas doenças associadas. Um rearranjo genético clonal foi feito para ajudar o diagnóstico. Além disso, usamos a radiocirurgia que até então nunca havia sido utilizada para tratar PNP associada à doença de Castleman. Esta produziu uma boa resposta, sugerindo que pode ser uma boa alternativa para o tratamento de PNP associada com a doença de Castleman quando é muito difícil fazer uma cirugia convencional.

Síndrome de POEMS associada à doença de Castleman: relato de caso e revisão da literatura / POEMS syndrome associated with Castleman disease: case report and literature review

JUSTIFICATIVA E OBJETIVOS: A síndrome de POEMS é uma entidade rara e pouco diagnosticada devido a sua variedade de manifestações clínicas, a maior parte delas inespecíficas. Pode estar associada à doença de Castleman em até 25% dos casos, uma entidade histopatológica também pouco comum. O diagnóstico da síndrome de POEMS é clínico, baseado em critérios definidos pela Mayo Clinic e seu tratamento ainda é um desafio. O objetivo deste estudo foi relatar um caso de síndrome de POEMS associada à doença de Castleman. RELATO DO CASO: Paciente do sexo masculino, 52 anos, foi encaminhado ao serviço com quadro de seis meses de evolução de emagrecimento, ginecomastia, hiperpigmentação de face e extremidades e parestesia distal em membros inferiores. Ao exame físico, notava-se também linfonodomegalia difusa. Exames laboratoriais mostravam eritrocitose, trombocitose, hipotireoidismo subclínico, testosterona no limite inferior da normalidade e hiperproteinorraquia. Exames de imagem para detecção de neoplasia foram todos normais. A biópsia de linfonodo supraclavicular esquerdo demonstrou doença de Castleman e a imunofixação sérica evidenciou componente monoclonal de cadeia leve, o que levou ao diagnóstico de síndrome de POEMS associada à doençade Castleman. CONCLUSÃO: A síndrome de POEMS deve ser lembrada em pacientes com quadro neurológico associado à doença monoclonal e manifestações clínicas diversas, enquanto a doença de Castleman deve fazer parte do diagnóstico diferencial de linfonodomegalia. A associação das duas entidades, apesar de rara, pode estar presente.

BACKGROUND AND OBJECTIVES: POEMS syndrome is a rare, often poorly diagnosed entity because of its variety of clinical manifestations, the majority of them being unspecific. It can be associated with Castleman disease, also a rare histopathological entity, in up to 25% of cases. The diagnosis of POEMS syndrome is a clinical one, based on Mayo Clinic criteria, and its management is still a challenge. The objective of this article was to report a case of POEMS syndrome associated with Castleman disease. CASE REPORT: Male patient, 52-year-old was referred to our service due to weight loss, gynecomastia, face and extremities hyperpigmentation and distal paresthesia of lower limbs that have been progressing for 6 months. At clinical examination, diffuse lymphadenopathy was also noted. Laboratory tests demonstrated polycythemia, thrombocytosis, subclinical hypothyroidism, testosterone at the lower limit of normal, and elevated cerebrospinal fluid protein. Images to detect tumors were all normal. Aleft supraclavicular lymph node biopsy revealed Castleman disease and serum immunoelectrophoresis demonstrated monoclonal light chain. With these findings, the diagnosis of POEMS syndrome associated with Castleman disease was established. CONCLUSION: POEMS syndrome should be considered in patients with a neurological condition associated with a monoclonal disease and many diverse clinical manifestations, while Castleman disease might be part of differential diagnosis of lymphadenomegaly. The association of the two entities, though rare,can be present.

Transformation of Castleman's Disease into Follicular Dendritic Cell Sarcoma, Presenting as an Asymptomatic Intra-abdominal Mass / 대한소화기학회지

Follicular dendritic cell (FDC) sarcoma is an extremely rare malignant neoplasm arising from FDCs. The exact origin of FDCs remains unclear; both a hematopoietic lineage origin and a stromal cell derivation have been proposed. Proliferation of FDCs can lead to benign reactive lesions or generate neoplastic conditions. The lesions are most commonly found in lymph nodes and usually involve the head and neck area. Castleman's disease is a rare non-neoplasitic lymphoproliferative disorder. Rare cases of hyaline-vascular Castleman's disease have been associated with FDC sarcoma, but a clonal relationship has not been convincingly demonstrated. A pathway toward tumor evolution, beginning with hyperplasia and dysplasia of FDCs, has been proposed. Despite this known association between Castleman's disease and FDC sarcoma, there have only been few reported cases of sarcoma arising as a complication of pre-existing Castleman's disease, especially in abdominal lesions. We describe here a 51-year-old female with an FDC sarcoma arising from unicentric, hyaline-vascular type Castleman's disease in an intra-abdominal mass. Pathologically, the lesion showed a series of changes during the process of transformation from Castleman's disease to FDC sarcoma.

A Case of Castleman's Disease Mimicking a Hepatocellular Carcinoma: A Case Report and Review of Literature / 대한소화기학회지

Castleman's disease is a rare disease characterized by lymph node hyperplasia. Although Castleman's disease can occur wherever lymphoid tissue is found, it rarely appears in the abdominal cavity, and is especially rare adjacent to the liver. Here, we report a rare case of Castleman's disease in the portal area that mimicked a hepatocellular carcinoma (HCC) in a chronic hepatitis B patient. A 40 year-old woman with chronic hepatitis B presented with right upper quadrant discomfort. Computed tomography and magnetic resonance imaging results showed a 2.2 cm-sized, exophytic hypervascular mass in the portal area. HCC was suspected. However, histologic examination revealed Castleman's disease. We suggest that Castleman's disease should be included as a rare differential diagnosis of a hypervascular mass in the portal area, even in patients with chronic hepatitis B.

Coexistencia de linfoma plasmablástico, sarcoma de Kaposi y enfermedad de Castleman en un paciente con infección por virus de inmunodeficiencia humana / Coexistence of plasmablastic lymphoma, Kaposi sarcoma and Castleman disease in a patient with HIV infection

El linfoma plasmablástico (LP) es un linfoma de células B poco común que está fuertemente asociado con la infección por el virus de inmunodeficiencia humana (VIH), y muestra una afinidad característica de presentación extra-ganglionar en la cavidad oral. Informamos el caso de un LP afectando el estómago en un paciente masculino de 36 años de edad con infección por VIH, asociado con sarcoma de Kaposi (SK) en áreas adyacentes al linfoma. Tenía el antecedente de enfermedad de Castleman y SK en una biopsia de ganglio linfático.

Plasmablastic lymphoma (PL) is an uncommon B-cell lymphoma that is strongly associated with human immunodeficiency virus (HIV) infection, and displays distinctive affinity for extranodal presentation in the oral cavity. We report the case of a PL involving the stomach in a 36 year-old man HIV+ patient, associated with Kaposi sarcoma (KS) in sections adjacent to lymphoma. He had a positive history of Castleman disease and KS in a lymphoid node biopsy.

Doença de Castleman associada a sarcoma de células dendríticas foliculares e miastenia gravis / Castleman's disease associated with follicular dendritic cell sarcoma and myasthenia gravis

A doença de Castleman é um distúrbio linfoproliferativo atípico, de etiologia desconhecida, que pode estar associada a uma série de condições clínicas, inclusive doenças de caráter autoimune e neoplasias malignas. No presente relato, uma paciente de 72 anos foi encaminhada ao serviço de cirurgia torácica do Hospital Universitário Getúlio Vargas, localizado na cidade de Manaus (AM) para a ressecção de um tumor de mediastino posterior. Três meses antes, havia sido internada em UTI com um quadro de dispneia intensa, ocasião na qual foi diagnosticada miastenia gravis. Após a ressecção da massa mediastinal, a análise histopatológica revelou doença de Castleman hialino-vascular complicada por sarcoma de células dendríticas foliculares. Até o momento da redação deste estudo, a paciente utilizava um anticolinesterásico e corticoides para o controle da miastenia gravis.

Castleman's disease is an atypical lymphoproliferative disorder of unknown etiology, which might be associated with various clinical conditions, including autoimmune diseases and malignant neoplasms. We report the case of a 72-year-old female patient who was referred to the thoracic surgery department of Getúlio Vargas University Hospital, in the city of Manaus, Brazil, for the resection of a posterior mediastinal tumor. Three months prior, the patient had been admitted to the ICU with signs of severe dyspnea, at which time she was diagnosed with myasthenia gravis. After the resection of the mediastinal tumor, the histopathological examination revealed hyaline vascular-type Castleman's disease, complicated by follicular dendritic cell sarcoma. At this writing, the patient was being treated with an anticholinesterase agent and corticosteroids for the control of myasthenia gravis.

Castleman's Disease Presenting with Uveal Effusion Syndrome

We report a rare case of multicentric Castleman's disease that presented with ophthalmic involvement, along with a review of the literature. A 63-year-old male presented with decreased visual acuity in both eyes. Both eyes had serous elevations of the retinas with shifting subretinal fluid and annular choroidal detachment. No retinal breaks were found. Laboratory tests revealed pancytopenia, hypergammaglobulinemia, and an increased erythrocyte sedimentation rate. Chest and abdominal computed tomographies showed multiple lymphadenopathies in the mediastinum, abdomen, and in both inguinal areas. Histological examination of the inguinal lymph node biopsy was consistent with Castleman's disease. After combination chemotherapy, the serous elevations of both retinas and the annular choroidal detachments of both eyes disappeared. Ophthalmic involvement in Castleman's disease is very rare, and to the authors' knowledge, this is the first report of ophthalmic involvement of Castlemans's disease in Korea.

Optic Neuropathy Associated with Castleman Disease

A 44-year-old woman with Castleman disease presented with acute visual loss in the left eye. A full ophthalmologic examination and imaging were performed. Visual acuity was 20/20 in the right eye and 20/100 in the left eye. Total dyschromatopsia, a relative afferent pupillary defect, and a cecocentral scotoma were observed in the left eye. Mild disc edema, without leaking during fluorescein angiography, was also observed. Magnetic resonance imaging revealed a small cystic epidermoid-like lesion in the right prepontine and suprasellar cistern. Her visual acuity did not improve and deteriorated to 20/200 in the left eye at 22 months after the initial visual loss. Optic neuropathy may rarely be associated with Castleman disease and suggests a poor prognosis.

Kaposi Sarcoma Herpes Virus-associated Hemophagocytic Syndrome Complicated by Multicentric Castleman Disease and Kaposi Sarcoma in a HIV-negative Immunocompetent Patient: An Autopsy Case

Kaposi sarcoma herpes virus (KSHV), also known as human herpesvirus-8, plays an important role in the pathogenesis of Kaposi sarcoma (KS), multicentric Castleman disease (MCD) of the plasma cell type, and primary effusion lymphoma. KSHV is rarely associated with the hemophagocytic syndrome (HPS), but when it does occur, it most occurs in immunocompromised patients. We report herein an unusual case of KSHV-associated HPS in an immunocompetent patient. A previously healthy 62-yr-old male was referred for evaluation of leukocytopenia and multiple lymphadenopathies. After a lymph node biopsy, he was diagnosed with MCD of the plasma cell type. KSHV DNA was detected in the lymph node tissue by polymerase chain reaction. Following a short-term response of the leukocytopenia to prednisolone, mental change, left side weakness, fever, thrombocytopenia, hemolytic anemia, and renal failure developed. Despite intravenous immunoglobulin therapy and plasmapheresis, he expired. The lymph nodes were infiltrated by hemophagocytic histiocytes in the sinuses. Pulmonary nodules and gastric erosions were shown to be KS. KSHV DNA was detected in the stomach, lung, and liver. This is the first case of multiple KSHV associated diseases including MCD and KS with KSHV-associated hemophagocytic syndrome in an HIV-negative, non-transplant, immunocompetent patient.

Doença de Castleman associada a derrame pleural / Castleman's disease accompanied by pleural effusion

A doença de Castleman é uma rara afecção do tecido linfóide. Relatamos o caso de uma paciente do sexo feminino com otosclerose bilateral, sem sintomas respiratórios e com achado incidental de derrame pleural esquerdo em uma radiografia de tórax. A tomografia computadorizada de tórax revelou uma massa mediastinal. A biópsia demonstrou tratar-se de variante plasmocitária da doença de Castleman. A paciente foi submetida à ressecção da massa mediastinal. Houve regressão do derrame, o qual persistiu como pequena loculação no espaço pleural esquerdo.

Castleman's disease is a rare disorder of the lymphoid tissue. We report the case of a female patient with bilateral otosclerosis, no respiratory symptoms, and pleural effusion discovered as an incidental finding on a chest X-ray. Computed tomography of the chest revealed a mediastinal mass. The biopsy findings demonstrated that it was a plasmacytic variant of Castleman's disease. The patient underwent mediastinal mass resection. This resulted in near-total resolution of the effusion, which remained as a small loculation within the left pleural space.

Multiple capillary hemangiomas: a distinctive lesion of multicentric Castleman's disease and POEMS syndrome.

A diagnosed case of Castleman's disease, proven by biopsy from enlarged inguinal lymph nodes, presented with multiple, asymptomatic, erythematous papules and nodules prevalent since nine years over the trunk and extremities. The lesions had been gradually increasing in number and size. The patient had had plasmacytoma of the lower thoracic vertebra 12 years ago, for which he was adequately treated with chemotherapy and local radiotherapy. Dermatological examination revealed erythematous papules and nodules on the face, trunk, and extremities that were diagnostic of capillary hemangiomas. Histopathology of the erythematous, soft papule was suggestive of capillary hemangioma. Contrast-enhanced computerized tomography of the abdomen and pelvis showed multiple retroperitoneal nodes suggestive of Castleman's disease along with multiple osteolytic lesions in the pelvic girdle and vertebrae. The patient was treated with injection rituximab and is currently under follow-up. We report this case to highlight a rare association between Castleman's disease and POEMS syndrome.

Castleman's disease in a patient with celiac disease.

A 72-year-old man with celiac disease was doing well on gluten-free diet. Five years later, he developed dyspepsia, fever and weight loss. CT scan and laparoscopic biopsies of small mesenteric lymph nodes clinched the diagnosis of Castleman's disease. He was started on chlorambucil; unfortunately he died few months after the diagnosis was made.

Castleman disease presenting as obstructive jaundice

A 48-year-old Saudi male was admitted with features of obstructive jaundice. Endoscopic retrograde cholangiopancreatography showed stricture in distal common bile duct CBD. Computed tomography scan revealed lymphadenopathy along CBD and in porta hepatis. Cholangiocarcinoma, lymphoma or metastatic deposits were suspected but no definite preoperative diagnosis could be established. Laparotomy disclosed lymph node enlargement in porta hepatis and along the CBD and lesser curvature of the stomach. Triple bypass procedure was performed to relieve the obstruction. Pathological examination of the lymph nodes showed Castleman disease of hyaline vascular type

Diffuse large B-cell lymphoma arising from a multicentric mixed variant of Castleman's disease.

This case report describes a patient with multicentric mixed type Castleman's disease and concomitant non-Hodgkin's lymphoma of diffuse large B cell type in the neck. Multicentric CD is a systemic illness with disseminated lymphadenopathy; its aggressive and usually fatal course is associated with infectious complications and risk for malignant tumors, such as lymphoma or Kaposi sarcoma.

Anemia hemolítica autoinmune en paciente infectado por virus de inmunodeficiencia humana y enfermedad de Castleman / Autoimmnune hemolytic anemia in AIDS-HIV patient

We present a case of a 41 years old C3 phase AIDS patient on antiretroviral therapy for 5 years, who developed a severe hemolytic anemia refractory to usual treatment, which progressed to death. Anemia in AIDS patients is a common finding (60-80%); however hemolytic anemia is unusual and when it is present, it is generally mild; severe anemia is associated with bad prognosis. The etiology of hemolytic anemia in HIV patients is due to multiple causes, the one mediated by autoantibodies against the red cell, usually IgG, being most common. The antibodies most frequently detected are against U or i antigen. Drug-related hemolysis, in glucose 6-phosphate dehydrogenase (G-6PD)-deficient patients, may also occur. A third mechanism of hemolysis is related to microangiopathy secondary to bacterial sepsis or hemolytic uremic syndrome (HUS). Some reports associate haemolytic anemia and Castleman´s disease, a lymphoproliferative disease with localized widespread adenopathy, fever, autoimmune manifestations and recurring infections.

Se presenta un paciente con SIDA etapa C3, 41 años de edad, en tratamiento antiretroviral durante 5 años, que cursó con linfoadenopatías generalizadas y anemia hemolítica severa refractaria a tratamiento, con progresión hacia la muerte. La anemia en pacientes con SIDA es un hallazgo común (60-80%). Sin embargo, la anemia hemolítica es poco frecuente y generalmente de escasa magnitud, pero si es severa se relaciona con mal pronóstico. La etiología de la anemia hemolítica en pacientes VIH es múltiple siendo más común la mediada por autoanticuerpos contra antígenos del eritrocito, en su mayoría IgG. Los más frecuentes son dirigidos contra antígeno U e i. Otra causa frecuente es la inducida por fármacos, en pacientes con déficit de G-6PD. Un tercer mecanismo de hemólisis es el producido por microangiopatía secundaria a sepsis bacteriana o síndrome hemolítico urémico. Algunas publicaciones asocian anemia hemolítica y enfermedad de Castleman, desorden linfoproliferativo que se puede presentar clínicamente como una masa localizada o una alteración sistémica con linfoadenopatías, fiebre, manifestaciones autoinmunes e infecciones recurrentes.