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.
Dendritic Cell Sarcoma, Follicular ; Abdominal Neoplasms ; Drug Therapy ; Bendamustine Hydrochloride ; Prognosis

Humans ; Colon, Descending ; Dendritic Cell Sarcoma, Follicular

Humans ; Dendritic Cell Sarcoma, Follicular/diagnosis* ; Granuloma, Plasma Cell/diagnosis* ; Biopsy, Needle ; Gastrointestinal Neoplasms ; Hepatitis

Colon ; Dendritic Cell Sarcoma, Follicular/surgery* ; Dendritic Cells, Follicular ; Epstein-Barr Virus Infections ; Herpesvirus 4, Human ; Humans

Colon ; Dendritic Cell Sarcoma, Follicular/surgery* ; Dendritic Cells, Follicular ; Granuloma, Plasma Cell/diagnostic imaging* ; Herpesvirus 4, Human ; Humans

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.
Adolescent ; Adult ; Dendritic Cell Sarcoma, Follicular ; Female ; Humans ; In Situ Hybridization ; Liver ; Lymph Nodes ; Male ; Middle Aged ; Retrospective Studies ; Young Adult

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.
Aged ; Dendritic Cell Sarcoma, Follicular ; Dendritic Cells, Follicular ; Electrons ; Female ; Fluorodeoxyglucose F18 ; Follow-Up Studies ; Head ; Humans ; Neck ; Parotid Gland ; Positron-Emission Tomography and Computed Tomography

Castleman Disease ; complications ; Dendritic Cell Sarcoma, Follicular ; complications ; Humans

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.
Aged ; Colonic Polyps* ; Dendritic Cell Sarcoma, Follicular* ; Dendritic Cells, Follicular ; Diagnosis ; Diagnosis, Differential ; Diagnostic Errors ; Female ; Granuloma, Plasma Cell ; Herpesvirus 4, Human ; Humans ; In Situ Hybridization ; Liver ; Pseudolymphoma ; Sarcoma ; Spleen ; Taiwan

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