OBJECTIVETo study the clinicopathologic characteristics and diagnostic criteria of interdigitating dendritic cell sarcoma/tumor (IDCS/T).

METHODSThe clinical features, histologic findings and results of immunohistochemical study in six cases of IDCS/T were analyzed, with review of literature.

RESULTSThe 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.

CONCLUSIONSIDCS/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.

Adult ; Aged ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; Dendritic Cell Sarcoma, Interdigitating ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Histiocytoma, Malignant Fibrous ; metabolism ; pathology ; Histiocytosis, Langerhans-Cell ; metabolism ; pathology ; Humans ; Lymph Nodes ; pathology ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Male ; Middle Aged ; Neck ; S100 Proteins ; metabolism ; Sarcoma ; pathology ; Soft Tissue Neoplasms ; metabolism ; pathology ; Thigh ; Tonsillar Neoplasms ; metabolism ; pathology ; Vimentin ; metabolism ; Young Adult

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

Abdominal Neoplasms ; complications ; metabolism ; pathology ; surgery ; Adult ; Dendritic Cell Sarcoma, Follicular ; complications ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Female ; Humans ; Ki-1 Antigen ; metabolism ; Leukocytosis ; complications ; metabolism ; pathology ; surgery ; Receptors, Complement 3b ; metabolism ; Receptors, Complement 3d ; metabolism ; Young Adult

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.
Abdomen/ultrasonography ; Abdominal Neoplasms/*diagnosis/etiology/pathology ; Dendritic Cell Sarcoma, Follicular/*diagnosis/etiology/pathology ; Female ; Giant Lymph Node Hyperplasia/complications/*diagnosis ; Humans ; Middle Aged ; Positron-Emission Tomography ; Tomography, X-Ray Computed

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

OBJECTIVETo study the clinicopathologic features, immunophenotype, molecular genetics and differential diagnosis of solid variant of angiomatoid fibrous histocytoma.

METHODSThe clinicopathologic features of 3 cases of solid variant of angiomatoid fibrous histocytoma were analyzed and the literature was reviewed.

RESULTSThere 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.

CONCLUSIONSSolid 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.

Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Back ; Calmodulin-Binding Proteins ; genetics ; Child ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Forearm ; Histiocytoma, Malignant Fibrous ; genetics ; metabolism ; pathology ; surgery ; Humans ; Knee ; Male ; Neoplasms, Muscle Tissue ; pathology ; Neurilemmoma ; metabolism ; pathology ; RNA-Binding Protein EWS ; RNA-Binding Proteins ; genetics ; Soft Tissue Neoplasms ; genetics ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Carcinoma ; metabolism ; pathology ; Cyclophosphamide ; therapeutic use ; Dendritic Cell Sarcoma, Follicular ; drug therapy ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Head and Neck Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Lung Neoplasms ; drug therapy ; secondary ; Male ; Melanoma ; metabolism ; pathology ; Middle Aged ; Neoplasm Recurrence, Local ; Prednisone ; therapeutic use ; Receptors, Complement 3b ; metabolism ; Receptors, Complement 3d ; metabolism ; Vincristine ; therapeutic use

Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; Dendritic Cell Sarcoma, Interdigitating ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Granuloma ; metabolism ; pathology ; surgery ; Head and Neck Neoplasms ; metabolism ; pathology ; surgery ; Humans ; Leukocyte Common Antigens ; metabolism ; Lymph Nodes ; metabolism ; pathology ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Lymphoma, T-Cell ; metabolism ; pathology ; Male ; Middle Aged ; Neoplasms, Multiple Primary ; metabolism ; pathology ; surgery ; Receptors, Cell Surface ; metabolism ; S100 Proteins ; metabolism ; Tonsillar Neoplasms ; metabolism ; pathology ; secondary

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.
Adult ; Dendritic Cell Sarcoma, Follicular/pathology/*radiography ; Diagnosis, Differential ; Female ; Gastrointestinal Neoplasms/radiography ; Head and Neck Neoplasms/pathology/radiography ; Humans ; Male ; Mediastinal Neoplasms/radiography ; Middle Aged ; Tomography, X-Ray Computed

Abdominal Cavity ; Adult ; Anemia, Aplastic ; therapy ; Antigens, CD34 ; metabolism ; Carcinoma ; metabolism ; pathology ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; Diagnosis, Differential ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Ki-67 Antigen ; metabolism ; Lymph Nodes ; pathology ; Male ; Neoplasms, Muscle Tissue ; metabolism ; pathology ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Sarcoma, Kaposi ; etiology ; metabolism ; pathology ; Viral Proteins ; metabolism