ABSTRACT
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.
Subject(s)
Humans , Female , Middle Aged , Castleman Disease/complications , Castleman Disease/pathology , Pemphigus/etiology , Pemphigus/pathology , Dendritic Cell Sarcoma, Follicular/etiology , Dendritic Cell Sarcoma, Follicular/pathology , Biopsy , Tomography, X-Ray Computed , Blotting, Western , Treatment Outcome , Fluorescent Antibody Technique, Indirect , Positron-Emission Tomography , Dendritic Cell Sarcoma, Follicular/surgery , HyalinABSTRACT
We report computed tomography (CT) findings for a rare case of follicular dendritic cell sarcoma of the greater omentum from a 47-year-old female patient. The tumor presented ash a palpable mass lesion in the umbilical region for the last two months. Multidetector CT scan of the abdomen showed a 14-cm soft-tissue mass with calcification and necrosis within the greater omentum. As a result, a follicular dendritic cell sarcoma should be considered in the differential diagnosis of a solitary omentum mass, especially one with coarse and chunk-like calcifications.
Subject(s)
Female , Humans , Middle Aged , Dendritic Cell Sarcoma, Follicular/pathology , Multidetector Computed Tomography/methods , Omentum/pathology , Peritoneal Neoplasms/pathologyABSTRACT
Follicular dendritic cell sarcoma is a rare malignant neoplasm and little is known about its radiological features. We present here four cases of follicular dendritic cell sarcomas and we provide the image characteristics of these tumors to help radiologists recognize this entity when making a diagnosis.
Subject(s)
Adult , Female , Humans , Male , Middle Aged , Dendritic Cell Sarcoma, Follicular/pathology , Diagnosis, Differential , Gastrointestinal Neoplasms/diagnostic imaging , Head and Neck Neoplasms/pathology , Mediastinal Neoplasms/diagnostic imaging , Tomography, X-Ray ComputedABSTRACT
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.
Subject(s)
Aged , Female , Humans , Dendritic Cell Sarcoma, Follicular/complications , Castleman Disease/complications , Myasthenia Gravis/diagnosis , Diagnosis, Differential , Dendritic Cell Sarcoma, Follicular/pathology , Castleman Disease/classificationABSTRACT
Dendritic cells (DC) are an essential component of the nonlymphoid, nonphagocytic immunoaccessory reticulum cells of the peripheral lymphoid tissue. Follicular dendritic cells (FDC) are one type of DC in the lymphoid follicle associated with B lymphocytes. They play an important role in the uptake and presentation of antigen generation and regulation of immune complexes. FDC can be recognized histologically by their oval to triangular nucleus, delicate basophilic nuclear membrane, almost empty nucleoplasm, small but distinct central nucleolus, and indistinct cellular outline; some cells can be binucleated or multinucleated. Ultrastructurally, they possess delicate interwoven cell processes connected by desmosomes. Immunohistochemically, they can be highlighted by staining with CD21, CD35, R4/23, Ki-M4, CNA.42 and CD68 (Kp-1) (l-4). FDC sarcoma is rare. FDC sarcomas affected predominantly lymph nodes with occasional extranodal involvement. Many cases of FDC sarcomas are probably misdiagnosed as other tumors such as large cell lymphoma, sarcomatoid carcinomas, fusocellular sarcomas or melanomas. We present five cases of FDC sarcomas and discuss the salient clinical, pathological and immunohistochemical features of these tumors.