Oral Soft Tissue Sarcomas Overview

Study Notes

Characterized by alternating hypercellular and hypocellular areas with myxoid collagenous stroma
Densely cellular subepithelial zone composed of primitive small cells with hyperchromatic nuclei and mitotic activity
Rhabdomyoblasts are typically seen in the hypocellular foci
Resembles stages of skeletal muscle embryogenesis
Rhabdomyoblasts are plump cells with eccentric nuclei and dense eosinophilic cytoplasm
Elongated strap cells display eosinophilic cytoplasm and cross striations
Tadpole cells (unipolar) are also observed

Features nests and sheets of primitive small round cells with abundant eosinophilic cytoplasm
Noncohesive cells floating in empty spaces create a characteristic alveolar pattern

Treatment usually involves local surgical excision followed by multiagent chemotherapy (vincristine, actinomycin D, and cyclophosphamide)
5-year survival rates vary depending on the subtype:
- Embryonal NOS: 66-72%
- Botryoid (type of embryonal): 95%
- Alveolar: 44-53%

Almost always begins in lymph nodes, any group can be affected
Most commonly presents in cervical and supraclavicular nodes
Shows a male predilection and a bimodal age distribution, peaking between 15-35 years and after 50 years
Typically presents as enlarging, nontender, discrete masses in one lymph node region
If untreated, it spreads to other lymph node groups and eventually involves the spleen and extralymphatic tissues (bone, liver, lung)
Oral involvement is rare
Systemic symptoms (weight loss, fever, night sweats, and generalized pruritus) may occur in 30-40% of patients
The absence of these symptoms is associated with a better prognosis

Normal nodal architecture is disrupted by a diffuse infiltrate of large, atypical neoplastic lymphoid cells
These atypical cells are known as Reed-Sternberg cells
Reed-Sternberg cells are typically binucleated (“owl-eye” nuclei) or multinucleated (“pennies on a plate”) with prominent nucleoli

Classified primarily as B cell lymphomas (more common) or T cell lymphomas
Typically develops in lymph nodes, but 40-50% arise in extranodal sites, including the oral cavity
Oral involvement is often an extranodal manifestation

Characterized by proliferation of lymphocytic-appearing cells with varying degrees of differentiation, depending on the specific lymphoma type

The tumor can develop in the oral soft tissues or centrally within the jaws
Soft tissue lesions appear as nontender, diffuse swellings, affecting the buccal vestibule, posterior hard palate, or gingiva
Lesions can be erythematous or purplish, and may or may not be ulcerated