Sarcoma folicular de células dendríticas mediastinal: primer reporte de caso en Costa Rica / Mediastinal Follicular Dendritic Cell Sarcoma: First Case Report in Costa Rica

El sarcoma folicular de células dendríticas (SFCD) es una neoplasia maligna rara derivada de las células dendríticas foliculares. Ha sido clasificado, dadas sus características inmunohistoquímicas, como parte del grupo de los sarcomas, donde representa un porcentaje menor al 1%. Actualmente, existen menos de 1.000 reportes en la literatura a nivel mundial, lo cual plantea una dificultad no sólo diagnóstica, siendo confundido frecuentemente con neoplasias de tipo linfoide; sino también terapéutica al no existir un claro consenso sobre su manejo definitivo. Esta revisión de caso clínico describe el primer caso reportado de SFCD en Costa Rica.

Follicular dendritic cell sarcoma (SFCD) is a rare malignant neoplasm derived from follicular dendritic cells, which has been classified, given its immunohistochemical characteristics, as part of the group of sarcomas, where it represents less than 1%. Currently, there are less than 1000 reports in the literature worldwide, which generates a difficulty not only in diagnosis, being frequently confused with lymphoid type neoplasms; but also, as therapeutic as there is no clear consensus on its definitive management. This clinical case review describes the first reported case of SFCD in Costa Rica.

Rare metastatic primary intra-abdominal follicular dendritic cell sarcoma poorly responsive to chemotherapy: A case report

Background@#Follicular dendritic cell sarcoma (FDCS) accounts for about 0.4% of soft tissue sarcomas. Approximately onethird of cases occur in extranodal sites and about 28% of extranodal FDCS may metastasize. Intra-abdominal occurrence is rare and there is limited published data to guide oncologists on how to best treat this malignancy.@*Case Presentation@#This is a case of a 33-year-old female who came in due to incidental finding of a left supraclavicular mass with 2-year history of early satiety. Neck node biopsy revealed a poorly differentiated malignant tumor with positive staining for CD21, CD23, vimentin and S100 consistent with FDCS. PET-CT revealed an intensely FDG-avid large mass in the left upper abdomen with signs of necrosis and mass effect. The patient was given three different chemotherapy regimens that included (1) gemcitabine/docetaxel, (2) single agent doxorubicin and (3) ifosfamide/etoposide, but she progressed on all these. Off-label use of bendamustine was then offered and after just the first cycle, the patient reportedly regained strength and was able to get up from wheelchair with noted interval decrease in size of the cervical mass. Unfortunately, the patient deteriorated and succumbed to infection and multiple pulmonary embolisms.@*Conclusion@#Intra-abdominal FDCS is a rare malignancy with heterogenous outcomes with no uniform treatment strategy at present. Molecular tumor board discussion and multi-disciplinary approach in extranodal FDCS is important in the diagnosis and management. Patients with multiple poor prognostic factors are at risk for tumor recurrence, metastasis, and death.

Interpretation of histiocytic/dendritic cell neoplasms and stromal-derived neoplasms of lymphoid tissues in the 5th edition of WHO classification of haematolymphoid tumors / 中华病理学杂志

The 5th edition of the World Health Organization classification of hematolymphoid tumors (WHO Blue Book) is soon to be published. Significant revisions have been made in the chapters on histiocytic/dendritic cell neoplasms and stroma-derived neoplasms of lymphoid tissues, leading to the reclassification and renaming of specific diseases. This article provides a concise interpretation and summary of these updates, highlighting the differences from the fourth edition. Pertinent changes from clinical pathological diagnosis to treatment and prognosis are explored, with an emphasis on recent advancements in molecular genetics. Newly introduced disease classifications are discussed, and the section on follicular dendritic cell sarcoma contributed by the author is detailed to assist readers in quickly understanding and assimilating the new classification standards.

Clinicopathological Features of Follicular Dendritic Cell Sarcoma / 中国医学科学院学报

To explore the clinicopathological and immunohistochemical characteristics of follicular dendritic cell sarcoma(FDCS)and the expressions of IgG and IgG4. We retrospectively analyzed the clinicopathological and immunohistochemical data of 9 pathologically confirmed FDCS cases in Peking Union Medical College Hospital from January 2005 to December 2018.Immunohistochemical staining of IgG and IgG4 were performed,and Epstein-Barr virus(EBV)-encoded RNA(EBER)in situ hybridization were carried out. Nine cases of FDCS included 4 men and 5 women aged 16-53 years [mean(38.2±9.7)years].The clinical manifestations included masses,lymph node enlargement,rash,and fever.The tumors were located in lymph node,retroperitoneal region,adrenal gland,neck,axillary region,and liver,respectively.Ultrasound showed clear boundary cystic or solid mass with maximum diameters of 1.5-15.0 cm.Microscopically,the spindle tumor cells were arranged in solid and storiform patterns with abundant and slightly stained cytoplasm,vacuolated nuclei,and small nucleoli.The mitosis was 1-3/10 high power fields,and necrosis was found in 5 cases.Immunohistochemically,the tumor cells were positive for CD21(6/9),CD35(6/9),and CD23(7/9). FDCS is a rare malignant tumor,which is easy to be missed.The combination of CD21,CD35,and CD23 is helpful for diagnosis.Hyaline-vascular type Castleman's disease may be the precursor of FDCS,and there may be only a small number of IgG4-positive plasma cells in FDCS.Surgical resection remains the main treatment for FDCS.

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.

Análise da concentração de células dendríticas, linfócitos T reguladores e mastócitos em lesões periapicais crônicas / Analysis of Dendritic Cells, T Regulatory Lymphocytes and Mastocytes Concentration in Chronic Apical Periodontitis

As lesões periapicais crônicas estão entre as mais frequentes do complexo maxilofacial, porém o perfil inflamatório dessas lesões é pouco compreendido, tanto do ponto de vista da caracterização celular como da expressão de citocinas. Cistos e granulomas periapicais compõem dois terços dessas lesões inflamatórias em região de mandíbula, onde são mais frequentes. O presente estudo propôs-se a estudar e avaliar a expressão imuno-histoquímica de CD1a+ (marcador de células dendríticas imaturas) e de FoxP3+ (marcador de linfócitos T reguladores) e verificar a presença de mastócitos em granulomas periapicais, cistos radiculares e residuais. Foram selecionados 73 casos, sendo 30 de granulomas periapicais, 29 de cistos radiculares e 14 de cistos residuais, dos arquivos do Serviço de Patologia Cirúrgica Oral e Maxilofacial do Departamento de Estomatologia da Faculdade de Odontologia da Universidade de São Paulo. Todos os grupos foram submetidos a análise morfológica, para classificação do infiltrado inflamatório e espessura epitelial, análise imuno-histoquímica, para detecção e contagem de células dendríticas e linfócitos T reguladores e coloração com azul de toluidina para contagem de mastócitos nas lesões periapicais crônicas. A análise morfológica revelou que a presença de infiltrado inflamatório grau I foi mais comum nos cistos periapicais. A gradação II e III foi mais comumente encontrada em cistos radiculares e granulomas periapicais. A avaliação da espessura epitelial mostrou que os epitélios atrófico e hipertrófico se apresentaram majoritariamente em cistos radiculares. Não houve diferenças estatisticamente significantes em relação ao infiltrado inflamatório e espessura epitelial nas lesões periapicais crônicas estudadas (p>0,05). A avaliação da contagem do número de células dendríticas (CD1a+) apresentou um valor médio maior em cistos radiculares (8,16 células/0,2mm2) (p<0,001) e o número médio de linfócitos T reguladores (FoxP3+) também foi maior em cistos radiculares (5,910 células/0,2mm2) (p<0,05). Na avaliação do número de mastócitos, os cistos radiculares apresentaram maior número médio dessas células do que as outras lesões periapicais (12.68 células/0,2mm2) (p<0,001). A avaliação da correlação entre infiltrado inflamatório e imunomarcação mostrou que houve diferença estatisticamente significante na correlação entre infiltrado inflamatório e células CD1a+ em granulomas periapicais (p<0,001). A medida que a gradação do infiltrado inflamatório aumentou, o número células CD1a+ diminuiu. E a correlação entre espessura epitelial e imunomarcação das células mostrou que a presença de epitélio hipertrófico em cistos radiculares apresentou maior densidade de células CD1a+. Não houve correlação estatisticamente significante da presença de linfócitos Treg e a gradação do infiltrado inflamatório nem da espessura epitelial. Todos esses resultados foram estatisticamente significativos (p<0,05). A concentração de células dendríticas imaturas e linfócitos T reguladores desempenham um papel importante no controle do microambiente inflamatório nos granulomas periapicais e cistos radiculares, respectivamente. A presença de mastócitos nos cistos radiculares pode estar associada à progressão, expansão da lesão e reabsorção óssea.

Recurrent Follicular Dendritic Cell Sarcoma of the Parotid Gland Imaged with 18F-FDG PET/CT / 대한핵의학회잡지

Follicular dendritic cell sarcoma (FDCS) is an extremely rare tumor with only 67 cases of head and neck FDCS reported in the literature. A 65-year-old female had a 6-cm follicular dendritic cell sarcoma resected from the left parotid gland with close margins. It recurred 1 year later as a 5-cm mass that was intensely [18F] fluoro-2-deoxy-D-glucose (18F-FDG) avid on positron emission tomography/computed tomography (PET/CT) and was re-excised. A follow-up PET/CT did not show any metastatic disease. The use of 18F-FDG PET/CT in the management of FDCS warrants further research. We present the 18F-FDG PET/CT imaging findings of this rare tumor.

Interdigitating dendritic cell sarcoma/tumor: a clinicopathologic study / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic characteristics and diagnostic criteria of interdigitating dendritic cell sarcoma/tumor (IDCS/T).</p><p><b>METHODS</b>The clinical features, histologic findings and results of immunohistochemical study in six cases of IDCS/T were analyzed, with review of literature.</p><p><b>RESULTS</b>The age of patients ranged from 20 to 68 years. The sites of involvement included lymph node, tonsil and soft tissue. Histologically, the tumor cells were arranged in sheets, fascicles or whorls and intimately admixed with abundant lymphocytes and plasma cells. They were oval to spindly in shape and contained pale eosinophilic cytoplasm, oval nuclei and distinct nucleoli.Immunohistochemical study showed that the tumor cells were positive for S-100 protein and CD68.</p><p><b>CONCLUSIONS</b>IDCS/T is a rare malignant tumor with poor prognosis. It carries distinctive histologic pattern and immunophenotype. The entity needs to be distinguished from follicular dendritic cell sarcoma/tumor, anaplastic large cell lymphoma and other spindle cell sarcomas in occurring soft tissue.</p>

Diagnosis and management of nasal and pharyngeal dendritic cell tumor-case report and literature review / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To investigate the clinical manifestation, pathological characteristics, treatment and prognosis of dendritic cell tumor.@*METHOD@#Four cases of nasal and pharyngeal dendritic cell tumor were described, including two cases of follicular dendritic cell sarcoma (FDCS), one case of Langerhans cell histiocytosis (LCH) and one case of Langer hans cell sarcoma (LCS). One of the patients with FDCS received multimodality therapy (surgery combined with chemotherapy), and the other patient only received chemotherapy and radiotherapy. The patients with LCH or LSC were treated by surgery.@*RESULT@#Of the two FDCS patients, one achieved complete remission after treatment by surgery combined with four cycles of CHOP chemotherapy regimen and concurrent radiotherapy (50 Gy), and the other who only received chemotherapy and radiotherapy survived with tumor for more than seven months of follow up. The patient of LCH was followed up for more than 2 years after surgery without recurrence or metastasis. The patient of LCS did not undergo radiotherapy or chemotherapy after surgery and died after 10 months of follow up.@*CONCLUSION@#Dendritic cell tumor is a group of very rare tumor and can be easily misdiagnosed in clinic, the confirmed diagnosis of which relies on histopathological features, immunohistochemistry combined with electron microscopy. FDCS, LCH and LCS have different pathological features, immunophenotypes and prognosis.

Follicular Dendritic Cell Sarcoma of the Inflammatory Pseudotumor-like Variant Presenting as a Colonic Polyp

Follicular dendritic cell (FDC) sarcoma is rare and is classified either as conventional type or inflammatory pseudotumor (IPT)-like variant. Extranodal presentation is uncommon and nearly all gastrointestinal FDC tumors are of the conventional type. IPT-like variant tumors occur almost exclusively in the liver and spleen and are consistently associated with Epstein-Barr virus (EBV). Here we report the case of a 78-year-old woman with an IPT-like FDC sarcoma presenting as a pedunculated colonic polyp. Histologically, scanty atypical ovoid to spindle cells were mixed with a background of florid lymphoplasmacytic infiltrate, which led to an initial misdiagnosis of pseudolymphoma. These atypical cells expressed CD21, CD23, CD35, and D2-40, and were positive for EBV by in situ hybridization, confirming the diagnosis. The patient was free of disease five months after polypectomy without adjuvant therapy. Although extremely rare, the differential diagnosis for colonic polyp should include FDC sarcoma to avoid an erroneous diagnosis. A review of the 24 cases of IPT-like FDC sarcoma reported in the literature reveal that this tumor occurs predominantly in females with a predilection for liver and spleen, and has a strong association with EBV.

Solid variant of angiomatoid fibrous histocytoma:report of 3 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features, immunophenotype, molecular genetics and differential diagnosis of solid variant of angiomatoid fibrous histocytoma.</p><p><b>METHODS</b>The clinicopathologic features of 3 cases of solid variant of angiomatoid fibrous histocytoma were analyzed and the literature was reviewed.</p><p><b>RESULTS</b>There were a total of 2 males and 1 female. The age of patients ranged from 9 to 12 years. The patients presented with a painless mass located in left forearm, left knee or back. The lesions were treated by complete surgical resection. On gross examination, the tumors varied from 1.6 cm to 4.5 cm in greatest dimension. They were well-circumscribed and had pale yellow to grayish-red solid cut surface. Histologically, the tumor was composed of histocytoid cells arranged in sheet-like pattern. A fibrous pseudocapsule surrounded by lymphocytes and plasma cells was identified. Immunohistochemical study showed that the tumor cells in all cases were positive for vimentin and CD68. They were negative for S100 protein, cytokeratin, CD34, CD31, smooth muscle actin, CD35, CD21 and CD30. Two cases also expressed CD99 and one of them was positive for desmin and epithelial membrane antigen. Fluorescence in-situ hybridization was positive for EWSR1 gene.</p><p><b>CONCLUSIONS</b>Solid type represents a variant of angiomatoid fibrous histocytoma and is considered as tumor of borderline malignant potential. Definitive diagnosis requires thorough histologic examination and clinical correlation. Immunohistochemistry and EWSR1 gene study are helpful in further delineation and differential diagnosis. Complete resection or wide local excision with post-operative follow up is the main modality of treatment.</p>

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.

Follicular dendritic cell sarcoma of soft plate: one case report / 临床耳鼻咽喉头颈外科杂志

Follicular dendritic cell sarcoma is a rare low-grade malignant tumor. At present, only twenty ca ses was discovered all over the world. This paper reports a case treated in our hospital, explores the clinical manifestations, pathological diagnosis and treatment to provide certain help to clinical doctor in diagnosis and treatment to reduce the misdiagnosis of the disease.

Follicular Dendritic Cell Sarcoma of the Omentum: Multidetector Computed Tomography Findings

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.

Follicular dendritic cell sarcoma: a case report and review of literature / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To report a case of follicular dendritic cell sarcoma (FDCS) of tonsil,analyze its clinical and pathological features, as well as the diagnosis and differential diagnosis.@*METHOD@#Tonsillectomy of low temperature coblation were done with general anesthesia. Histopathology, immunohistochemistry, electron microscope were used to analyzed the features of FDCS. The clinical character and treatment were reported.@*RESULT@#There was no evidence of recurrence in two years.@*CONCLUSION@#A correct diagnosis of FDCS was difficult to make , and immunohistochemical and ultrastructural studies are useful to FDCS's diagnosis. Low temperature coblation used in FDCS need more experience.

Imaging Findings of Follicular Dendritic Cell Sarcoma: Report of Four Cases

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.

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.

Clinicopathological study on the follicular dendritic cell sarcoma / 中华肿瘤杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathologic features and differential diagnostic methods for follicular dendritic cell sarcoma.</p><p><b>METHODS</b>Histological and immunohistochemical examinations and EBER in situ hybridization were used to investigate the pathological features of 5 cases of follicular dendritic cell sarcoma, and related literature was reviewed.</p><p><b>RESULTS</b>There were 3 males and 2 females with a median age of 54 years (range, 28 - 75 years). The location of lesions included lymph node (2 cases), tonsil (1 case), stomach (1 case), and liver (1 case). The growth patterns were fascicular or whorls and/or diffuse. The neoplastic cells were spindle or ovoid in shape with indistinct border and slightly eosinophilic cytoplasm. The nuclei were round, oval or spindle in shape with small distinct nucleoli. Warthin-Finkeldey-like multinucleated giant cells were detected in two cases. Mitotic figures were found in 1-22/10 HPF. Immunohistochemical staining showed that CD21 and CD23 (3 of 5), CD35 (4 of 5), D2-40 (4 of 4), and CXCL13 (3 of 4) were positive in neoplastic cells. EBER was detected in one of five cases by in situ hybridization. Four cases were followed-up for 6 approximately 25 months and no recurrence or death was observed yet.</p><p><b>CONCLUSION</b>Follicular dendritic cell sarcoma is an extremely rare and should be considered as a moderately malignant tumor, and may present histological polymorphism with certain distinctive features. Immunohistochemistry is necessary in differential diagnosis to distinguish from other tumors.</p>

Follicular dendritic cell sarcoma: a clinicopathologic analysis of ten cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features of follicular dendritic cell sarcoma (FDCS) and its differential diagnosis.</p><p><b>METHODS</b>Ten cases of FDCS were studied by light microscopy, immunohistochemistry and in-situ hybridization. The clinical features and follow-up information were analyzed.</p><p><b>RESULTS</b>Amongst the 10 cases of FDCS studied, the male-to-female ratio was 1:1. The mean age of the patients was 42 years. Six of them were located in cervical and peritoneal lymph nodes and four in extranodal sites (including tonsil, pelvic cavity, tail of pancreas and spleen). Histologically, the tumor cells had whorled, storiform or diffuse growth patterns. They were spindle in shape and contained syncytial eosinophilic cytoplasm, with round or oval nuclei, vesicular chromatin, distinct nucleoli and a variable number of mitotic figures. Multinucleated tumor giant cells and intranuclear pseudoinclusions were occasionally seen. There was a sprinkling of small lymphocytes and neutrophils within the tumor as well as in the perivascular region. Immunohistochemical study showed that the tumor cells were diffusely or focally positive for CD21, CD23, CD35 and D2-40, but negative for LCA, CD20, CD3, CD1a, HMB45 and CK. Some of them showed EMA, CD68 and S-100 reactivity. In-situ hybridization for Epstein-Barr virus-encoded RNA (EBER) showed positive signals in only one case (which was diagnosed as inflammatory pseudotumor-like FDCS). Of the 7 patients with follow-up information available (duration: 2 months to 39 months; mean: 14 months), 2 cases with paraneoplastic pemphigus died of pulmonary infection at 5 and 7 months respectively. The remaining 5 patients were alive and disease-free after surgical excision (+/- chemotherapy and radiotherapy).</p><p><b>CONCLUSIONS</b>FDCS is a rare low to intermediate-grade malignant tumor. Appropriate application of FDC markers, such as CD21, CD35 and D2-40, would be helpful for arriving at a correct diagnosis. Most cases are associated with good prognosis after surgical treatment, with or without chemotherapy and radiotherapy. Patients with paraneoplastic pemphigus carry a less favorable prognosis.</p>