What causes follicular dendritic cell sarcoma?

We must pay attention to the cause of follicular dendritic cell sarcoma. Only by scientifically understanding these causes can we effectively prevent them in our lives. Common examinations include histopathological examination and electron microscopy. We must take these diseases seriously.

1. Causes

The etiology is unknown, but features of an inflammatory pseudotumor are present in a subset of cases that are recognized to be follicular dendritic cell sarcomas. Such hyperproliferative cases have been shown to be associated with EBV.

2. Clinical manifestations

Follicular dendritic cell sarcoma is a rare tumor with a wide age distribution, mostly adults, and equally common in both sexes. 10% to 20% of tumors may be accompanied by Castleman's disease. The incidence may be increased in patients with schizophrenia who are receiving long-term treatment. Follicular dendritic cell sarcoma occurs in the lymph nodes, accounting for 1/2 to 2/3 of all cases. The cervical lymph nodes are the most common site of occurrence, but it can also occur in multiple other extranodal sites, including the tonsils, spleen, oral cavity, gastrointestinal tract, liver, soft tissue, skin, and even breast. The most common sites of metastasis include lymph nodes, lungs, and liver. Patients often present with a slowly growing, painless mass, but patients with intra-abdominal lesions may have abdominal pain; systemic symptoms are uncommon.

3. Inspection of this section

1. Histopathology

The proliferating spindle and oval cells form bundles and layers of tumor cells. Most of the individual tumor cells have abundant cytoplasm, slight eosinophilia, and clear cytoplasm. The cell nucleus is elongated, the nuclear chromatin is vesicular or dispersed granular, there are small and clear nucleoli, and the nuclear membrane is thin. Occasionally, multilobed cells are seen, sometimes resembling Warthin-Finkeldy giant cells. In addition, intranuclear pseudoinclusions can also be seen. The cytological features are bland in most cases, but significant cellular atypia may be seen in some cases. Undestroyed remnants of lymphoid tissue are often also seen. Tumors arising in the liver and spleen more closely resemble inflammatory pseudotumors in their histologic features.

2. Electron microscopy

The tumor cells have elongated nuclei and often have infolded cytoplasm. The cytoplasm often contains a large number of ribosomes and the most specific feature is the presence of many elongated cytoplasmic processes connected by scattered mature desmosomes, Birbeck granules, and a large number of lysosomes.