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

Langerhans cell sarcoma is a rare, proliferative tumor of Langerhans cells, which shows cytologic characteristics and clinical features of malignant tumor. Langerhans cell sarcoma primarily occurs in lymph nodes, skin, lung, liver, and spleen. However, very few cases have been reported in the head and neck region. Because of its rarity, an optimal treatment approach is unknown; however, Langerhans cell sarcoma grows aggressively and shows a poor prognosis, such that a more aggressive and multi-modality treatment approach is necessary. Here, we report the case of a 36-year-old male with Langerhans cell sarcoma, who presented with a mass in the submandibular gland area and was treated with wide excision and postoperative radiotherapy.
Adult ; Head ; Humans ; Langerhans Cell Sarcoma ; Langerhans Cells ; Liver ; Lung ; Lymph Nodes ; Male ; Neck ; Prognosis ; Radiotherapy ; Sarcoma ; Skin ; Spleen ; Submandibular Gland

Histiocytic sarcoma is a rare hematologic malignancy, with very few cases of primary histiocytic sarcoma of the breast described in English scientific literature. Herein, we describe a case of primary histiocytic sarcoma of the breast in a 75-year-old woman, with no clinical history of malignant tumors, who presented with a palpable solitary breast mass. Microscopically, the resected breast mass showed large pleomorphic cells, some multinucleated giant cells, and admixed inflammatory components. The pleomorphic tumor cells further showed a diffuse, noncohesive growth pattern, an abundant eosinophilic cytoplasm, and strong and diffuse immunoreactivity for cluster of differentiation (CD) 68 and CD163. Furthermore, a whole-body positron-emission tomography/computed tomography using deoxy-2-[¹⁸F]fluoro-D-glucose performed after surgery showed no other masses or lesions. After surgical excision, the patient was followed up, and no evidence of tumor recurrence or metastasis was noted.
Aged ; Breast ; Cytoplasm ; Eosinophils ; Female ; Giant Cells ; Hematologic Neoplasms ; Histiocytes ; Histiocytic Sarcoma ; Humans ; Neoplasm Metastasis ; Recurrence

Currently, the World Health Organization classifies Langerhans cell tumors into Langerhans cell histiocytosis and Langerhans cell sarcoma (LCS). LCS is a neoplastic proliferation of Langerhans cells showing malignant cytological features and aggressive clinical behavior with grave prognosis. Only a few cases have been reported in the available literature; therefore, to date, no definitive treatment has been established. A 64-year-old woman presented with a 1-year history of an asymptomatic, slow-growing erythematous nodule measuring 0.7 cm on her scalp. The patient also reported a 3-month history of a painful swelling on the right side of her neck. Histopathological examination of a scalp biopsy specimen revealed sheets of atypical cells with hyperchromatic nucleoli and clear cytoplasm. Immunohistochemical studies revealed malignant cells positive for CD1a, CD31, CD68, and S-100 expression. Additionally, positron emission tomography–computed tomography and fine-needle aspiration revealed LCS of the cervical lymph nodes and surrounding soft tissue. We recommended surgical excision and adjunctive chemotherapy; however, the patient refused treatment and died of the disease 28 months later.
Biopsy ; Biopsy, Fine-Needle ; Cytoplasm ; Drug Therapy ; Electrons ; Female ; Histiocytosis, Langerhans-Cell ; Humans ; Langerhans Cell Sarcoma ; Langerhans Cells ; Lymph Nodes ; Middle Aged ; Neck ; Parotid Gland ; Prognosis ; Scalp ; World Health Organization

Dendritic Cell Sarcoma, Interdigitating ; Dendritic Cells ; Hematopoietic Stem Cell Transplantation ; Humans ; Lymphoma, Non-Hodgkin