Multifocal Interdigitating Dendritic Cell Sarcoma Demonstrated on FDG PET/CT Imaging.

Interdigitating dendritic cell sarcoma is a very rare malignancy that more commonly occurs in one anatomic location. Surgical resection of the lesion is the mainstay of the therapy. Multiple lesions at different locations are less common at the presentation. We report FDG PET/CT findings of multifocal interdigitating dendritic cell sarcoma in a 30-year-old woman.

A Rare Case of Interdigitating Dendritic Cell Sarcoma of the Rectum: Review of Histopathology and Management Strategy.

Interdigitating dendritic cell sarcoma (IDCS) is a rare neoplasm arising from a subclass of dendritic cells, known for their role in mediating various immunological functions, including T-cell mediated immunity. Although existing literature on IDCS is limited to scattered reports, extranodal manifestation in the gastrointestinal tract, and in particular, the rectum is extremely rare. To our knowledge, we report only the second case of IDCS arising in the rectum in a young 20-year-old man, successfully managed surgically and with a good oncological outcome. Existing literature on the incidence, pathophysiology and treatment strategies is also examined.

Interdigitating dendritic cell sarcoma presenting in the sigmoid colon mesentery: A case report and literature review.

RATIONALE: Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare disease. It commonly occurs in middle-aged males and mainly involves the lymph nodes. Pathological examination plays an important role in differentiating from other tumors, but far less published literature focuses on the imaging characteristics of IDCS. PATIENT CONCERNS: Here, we reported a case of IDCS in a 52-year-old male involving the pelvis with medical imaging and pathologic findings. DIAGNOSES: Preoperative unenhanced CT scan revealed a 6.0 × 6.3 × 8.0 cm mass with density equal to that of adjacent muscle, located in the pelvis. On contrast-enhanced CT images, the tumor presented apparent homogeneous enhancement. CT angiography showed that the tumor was supplied by the branches of inferior mesenteric artery. Pelvic magnetic resonance imaging manifested a lobulated solid mass with low signal on T1-weighted and intermediate to high signal on T2-weighted images. Simultaneously, significantly high signal intensity was exhibited on the diffusion-weighted images. This patient underwent operative resection of the tumor. The pathologic diagnosis was IDCS. INTERVENTIONS: This patient underwent operative resection of the tumor. The resection margins were negative for the neoplastic proliferation and no distant metastases were found. The patient did not receive advanced radiotherapy or chemotherapy. OUTCOMES: Three months after surgery, the follow-up CT scan did not reveal any recurrence or metastases. LESSONS: This case adds to the experience with IDCS by summarizing its characteristics as well as reviewing the literature.

Intracranial Interdigitating Dendritic Cell Sarcoma: First Case Report.

BACKGROUND AND IMPORTANCE: This is the first report of a primarily intracranial interdigitating dendritic cell sarcoma (IDCS). CLINICAL PRESENTATION: A 39-year-old patient with right hemiparesis underwent complete resection of a large parafalcine tumor with subsequent complete recovery of neurological symptoms. Histologically, the tumor was diagnosed as IDCS. Extensive staging did not reveal any extracranial manifestation of this disease. After 1.5 years, the patient remains recurrence free and is being observed closely. CONCLUSION: IDCS are exceedingly rare tumors and so far have not been found intracranially. On the basis of the limited experience with extracranial occurrence, this tumor is best managed by complete resection and careful oncological observation. ABBREVIATIONS: FDCS, follicular dendritic cell sarcomaIDCS, interdigitating dendritic cell sarcomaRTU, ready-to-use kit.

Braf mutation in interdigitating dendritic cell sarcoma: a case report and review of the literature.

Interdigitating dendritic cell sarcoma is an extremely rare tumor. The diagnosis is difficult and is based on clinical, pathological and immunohistochemical evaluation. Differential diagnosis includes melanoma, mesenchymal and hematological malignancies. The mainstay of treatment is surgery for limited disease and different chemotherapy combinations have been tested for advanced disease. No evidence from prospective trials is currently available. We report the case of a 59 year-old male patient who experienced axillary lymphadenopathy with initial diagnosis of large-cell lung cancer on tumor biopsy. He underwent surgical resection with radical intent. Pathological diagnosis of interdigitating dendritic cell sarcoma was obtained on surgical samples. Nine months after radical surgery, he experienced systemic recurrence of disease and underwent chemotherapy with epirubicin and ifosfamide for 4 courses. During chemotherapy, he developed brain disease progression and underwent whole-brain radiotherapy. Systemic progression was then observed and molecular characterization was performed. B-RAF evaluation resulted positive for V600E mutation and the patient was treated with Vemurafenib according to molecular findings. He thus obtained initial clinical benefit but eventually died of brain hemorrhage. In conclusion, we report a case of B-RAF mutation detected in an interdigitating dendritic cell sarcoma patient treated with targeted therapy. B-RAF pathway could have a role in pathogenesis and evolution of this rare disease and could open new perspectives of treatment.

Primary intratesticular spindle cell tumors: interdigitating dendritic cell tumor and inflammatory myofibroblastic tumor.

Spindle cell neoplasms arising in the testis are uncommon; most cases belong to the category of gonadal stromal tumors, and the presence of distinctive clinical and pathological features usually lead to a definitive diagnosis. In some instances, however, the diagnosis of these tumors can be challenging and special techniques are needed. The present study reports 2 unusual cases of primary intratesticular tumors showing a striking morphology, characterized by spindle to stellate cells in a lymphoid background. The diagnosis of interdigitating dendritic cell tumor and inflammatory myofibroblastic tumor was established in the 2 cases, and their probable origin in cells of the accessory immune system is discussed. Although both tumors share similar histological features, their immunohistochemical profiles were decisive for a definitive diagnosis.

Interdigitating dendritic cell sarcoma of lymph node mimicking granuloma: a case report and review of the literature.

Interdigitating dendritic cell sarcoma (IDCS), is an extremely rare neoplasm. We report a case of a 77-year-old man presented with gradual lymph nodes enlargement in inguina, neck and axilla for 6 months. Biopsy revealed that part of the lymph node was replaced by several large granuloma-like nodules composed of mild atypical tumor cells, resembling epithelioid cells. Mitotic figures were hardly found. Immunohistochemistry showed that tumor cells were positive for S-100, CD68 and CD45. Ki-67 labeling index was 5%. To the best of our knowledge, this is the first case of IDCS showing granuloma-like growth pattern with mild atypical tumor cells.

Interdigitating dendritic cell sarcoma of the ileum recurred in multiple lymph nodes and duodenum three years after operation without chemotherapy.

Neoplasms derived from interdigitating dendritic cell are extremely rare. Here we describe a case of a 47-year-old man with interdigitating dendritic cell sarcoma (IDCS) in the ileum. He was admitted to a hospital due to ileus. The ileal tumor, measuring 2cm, was detected and resected with regional lymphadenectomy. At that time, a pathologic diagnosis of malignant peripheral nerve sheath tumor was made. The patient, who was not treated with chemotherapy, showed no signs of recurrence. After three years, we detected cervical lymphadenopathy and multiple duodenal masses in the patient in our hospital. Oval to spindle-shaped atypical cells, which resembled ileal tumor cells, infiltrated into the lymph node and duodenum. Immunohistochemical staining of these three lesions revealed positivity of S100 protein and several macrophage-related antigens. Based on the histologic and immunohistochemical analysis, the histopathologic diagnosis of IDCS was confirmed. To our knowledge, five cases of IDCS arising in the intestinal tract have been reported to date, and only one case, treated with both surgery and chemotherapy, led to remission. This is the first case that has a comparatively favorable prognosis without chemotherapy after surgery.

Interdigitating dendritic cell sarcoma of the parotid gland: case report and literature review.

Interdigitating dendritic cell sarcoma (IDCS) is an exceedingly rare neoplasm arising from the antigen-presenting cells of the immune system. We report a case of IDCS occurring in a 69-year-old man who presented to an outside institution with a painless mass in his right parotid gland for several months. He presented to our institution after undergoing a superficial parotidectomy. A diagnosis of undifferentiated neoplasm, favoring poorly differentiated carcinoma, was made at that time. He underwent a total parotidectomy and neck dissection at our institution. Microscopically, the tumor was composed of atypical spindle cells involving the parotid gland and an ipsilateral level III lymph node. Immunophenotypic analysis demonstrated positive staining for S100, fascin, vimentin, and HLA-II. Follicular dendritic cell, lymphoid, epithelial, myoepithelial, and melanoma markers were negative. Taken together, the above features were consistent with IDCS. An IDCS of the parotid gland is extremely rare, with only 2 cases reported in the literature. The unusual location and morphological similarity to follicular dendritic sarcoma and other types of soft tissue sarcomas can be a diagnostic challenge. Awareness of this tumor and the use of appropriate markers are crucial in making the diagnosis. The patient did well postoperatively, and he underwent a complete course of postoperative irradiation to the right parotid and neck.