Dendritic Cell Sarcoma, Follicular ; pathology ; Dendritic Cell Sarcoma, Interdigitating ; pathology ; Female ; Humans ; Middle Aged ; Stomach Neoplasms ; pathology

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.
Humans ; Lymphoid Tissue/pathology* ; Soft Tissue Neoplasms/pathology* ; Dendritic Cell Sarcoma, Follicular/pathology* ; Dendritic Cells/pathology* ; World Health Organization

OBJECTIVETo study the pathologic features, diagnosis and differential diagnosis of interdigitating dendritic cell sarcoma (IDCS).

METHODSThe clinical findings, morphologic features and immunophenotype of 3 cases of IDCS were investigated.

RESULTSGross examination showed that IDCS had a greyish-white to greyish-yellow cut surface. The site of occurrence included lung, spleen (with lymph node metastasis) and lymph node. Histologically, the tumor cells were arranged in nests, fascicles and whorls, with intimate admixture of many lymphocytes and plasma cells. They were oval to spindle in shape and contained pale eosinophilic cytoplasm, oval and sometimes grooved nuclei, small distinct nucleoli and ill-defined cell borders. Immunohistochemical study showed that the tumor cells expressed S-100 protein.

CONCLUSIONSIDCS is a rare type of histiocytic and dendritic cell malignancy with distinctive morphologic findings. It needs to be distinguished from follicular dendritic cell sarcoma, inflammatory pseudotumor, Langerhans' cell histiocytosis, malignant melanoma, undifferentiated carcinoma and anaplastic large cell lymphoma. Immunohistochemical staining for S-100 protein is helpful in confirming the diagnosis.

Adolescent ; Carcinoma ; pathology ; Dendritic Cell Sarcoma, Follicular ; pathology ; Dendritic Cell Sarcoma, Interdigitating ; diagnosis ; pathology ; Dendritic Cells ; pathology ; Diagnosis, Differential ; Female ; Humans ; Lymph Nodes ; pathology ; Lymphatic Metastasis ; pathology ; Male ; Middle Aged ; S100 Proteins ; analysis ; immunology ; Young Adult

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.
Aged, 80 and over ; Dendritic Cell Sarcoma, Follicular ; diagnosis ; pathology ; surgery ; Diagnosis, Differential ; Humans ; Immunohistochemistry ; Male ; Tonsillar Neoplasms ; diagnosis ; pathology ; surgery

OBJECTIVETo study the clinicopathologic features of inflammatory pseudotumor-like follicular dendritic cell tumor of spleen.

METHODSOne case of inflammatory pseudotumor-like follicular dendritic cell tumor of spleen was examined macroscopically and microscopically. Immunohistochemical study for CD21, CD23, CD35, clusterin, S-100 protein, vimentin, smooth muscle actin, CD1a, CD68, ALK protein, CD30, CD31, CD34, CD3 and CD20 was performed on formalin-fixed, paraffin-embedded sections by standard EnVision method. In-situ hybridization for Epstein-Barr virus (EBV)-encoded RNA was also carried out.

RESULTSMacroscopically, inflammatory pseudotumor-like follicular dendritic cell tumor was large in size, tan-colored, soft to rubbery in consistance and associated with central hemorrhage and necrosis. Histological examination showed scattered follicular dendritic cells admixed with abundant lymphocytes and plasma cells in the background, simulating inflammatory pseudotumor. On high-power magnification, the follicular dendritic cells possessed a moderate amount of pale to lightly eosinophilic cytoplasm, with indistinct cell borders. The nuclei were ovoid or spindly, with vesicular or stippled chromatin and small distinct, often centrally located, nucleoli. Some of the tumor cells showed nuclear pleomorphism and contained irregular foldings of nuclear membrane, coarse chromatin and prominent eosinophilic nucleoli. Mitotic figures were rarely identified. Immunohistochemical study showed that the tumor cells were positive for vimentin, clusterin, smooth muscle actin and CD68. They were weakly and focally positive for CD35 and S-100 protein, but negative for CD21, CD23, CD1a, ALK protein, CD30, CD31 and CD34. Most of the background lymphocytes were of T-lineage (CD3-positive) ,some were CD20 (B-cell marker)-positive. EBV RNA was demonstrated in the tumor cells by in-situ hybridization analysis.

CONCLUSIONSInflammatory pseudotumor-like follicular dendritic cell tumor is a rarely encountered low-grade malignancy with distinctive morphologic pattern. It is associated with EBV infection.

Adult ; Antigens, CD ; Antigens, Differentiation, Myelomonocytic ; Dendritic Cell Sarcoma, Follicular ; pathology ; physiopathology ; Dendritic Cells, Follicular ; pathology ; Female ; Granuloma, Plasma Cell ; etiology ; Herpesvirus 4, Human ; genetics ; immunology ; isolation & purification ; Humans ; Splenic Neoplasms ; pathology ; physiopathology

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.
Dendritic Cell Sarcoma, Follicular/pathology/*radiography ; Female ; Humans ; Middle Aged ; Multidetector Computed Tomography/*methods ; Omentum/pathology/*radiography ; Peritoneal Neoplasms/pathology/*radiography

Adult ; Appendiceal Neoplasms ; immunology ; pathology ; Dendritic Cell Sarcoma, Follicular ; immunology ; pathology ; Humans ; Immunohistochemistry ; Male ; Proto-Oncogene Proteins c-kit ; analysis ; Receptors, Complement 3b ; analysis

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.
Child ; Child, Preschool ; Dendritic Cell Sarcoma, Follicular ; pathology ; therapy ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Nasopharyngeal Neoplasms ; pathology ; therapy ; Prognosis

OBJECTIVETo investigate clinicopathologic features, immunophenotypes and differential diagnoses of follicular dendritic cell sarcoma/tumor (FDCS).

METHODSEight cases of FDCS were studied using histological and immunohistochemical examinations and EBER in situ hybridization, with a review of the related literatures.

RESULTSThere were 5 male and 3 female patients with a median age of 50 years. The sites of involvement included lymph node (4 cases), tonsil, nasopharynx, liver, and spleen (1 case each, respectively). The predominant microscopic features histologically included storiform, fascicular, diffuse, whorled and nodular in patterns. The neoplastic cells, dispersed by the infiltrated small lymphocytes, were characterized by abundant eosinophilic or fine granular cytoplasm with indistinct cell borders, and syncytial in appearance. The nuclei of the tumors were ovoid, round to spindled in shape with vesicular or stippled chromatin and small distinct nucleoli. Mitotic figures varied among cases. Pseudovascular spaces and perivascular cuffing were observed in some cases. One case of FDCS involving lesion in liver showed a background of abundant lymphocytes mixing with dispersed spindle or ovoid neoplastic cells having delicate chromatin, mild nuclear atypia, irregular/vesicular nuclei and distinct nucleoli. The neoplastic cells were positive for CD21, CD35, clusterin, and weakly positive for CD68, EMA, S-100 and EGFR. Ki-67 stain showed a variable expression among cases. EBER was positive in 2 cases.

CONCLUSIONSFDCS is a rare malignant tumor with a tendency to relapse and metastasis. Combined morphological and immunophenotypical analysis is necessary to reach a correct diagnosis.

Adult ; Aged ; Antigens, CD ; analysis ; immunology ; Antigens, Differentiation, Myelomonocytic ; analysis ; immunology ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; Dendritic Cells ; pathology ; Female ; Giant Cells ; Humans ; Immunophenotyping ; methods ; Male ; Middle Aged ; Receptors, Complement 3d ; analysis ; immunology ; Treatment Outcome

OBJECTIVETo study the clinicopathologic features of follicular dendritic cell sarcoma (FDCS) and its differential diagnosis.

METHODSTen cases of FDCS were studied by light microscopy, immunohistochemistry and in-situ hybridization. The clinical features and follow-up information were analyzed.

RESULTSAmongst 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).

CONCLUSIONSFDCS 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.

Adult ; Antibodies, Monoclonal, Murine-Derived ; metabolism ; Dendritic Cell Sarcoma, Follicular ; complications ; metabolism ; pathology ; surgery ; Dendritic Cell Sarcoma, Interdigitating ; pathology ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Lymph Node Excision ; Lymph Nodes ; pathology ; surgery ; Male ; Meningioma ; pathology ; Middle Aged ; Nasopharyngeal Neoplasms ; pathology ; Paraneoplastic Syndromes ; complications ; Pemphigus ; complications ; Receptors, Complement 3b ; metabolism ; Receptors, Complement 3d ; metabolism ; Receptors, IgE ; metabolism ; Tonsillar Neoplasms ; metabolism ; pathology ; surgery ; Young Adult