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Interdigitating dendritic cell sarcoma of the parotid gland: case report and literature review
Guy Efune1, Baran D Sumer, Venetia R Sarode
1Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, TX 75390-9035, USA.
Insights
Interdigitating dendritic cell sarcoma (IDCS) is a rare cancer of immune cells. This case highlights a challenging IDCS diagnosis in the parotid gland, emphasizing diagnostic markers.
Area of Science:
- Oncology
- Immunology
- Pathology
Background:
- Interdigitating dendritic cell sarcoma (IDCS) is an exceptionally rare neoplasm originating from immune system's antigen-presenting cells.
- Diagnosis of IDCS can be challenging due to its rarity and potential to mimic other tumors.
Observation:
- A 69-year-old male presented with a parotid gland mass initially diagnosed as undifferentiated neoplasm.
- Microscopic examination revealed atypical spindle cells in the parotid gland and a lymph node.
- Immunophenotypic analysis showed positivity for S100, fascin, vimentin, and HLA-II, with negative markers for other cell types.
Findings:
- The features were consistent with Interdigitating dendritic cell sarcoma (IDCS) of the parotid gland.
- This represents one of only three reported cases of IDCS in the parotid gland.
- Morphological similarity to other sarcomas posed a diagnostic challenge.
Implications:
- Increased awareness of IDCS in unusual locations like the parotid gland is crucial for accurate diagnosis.
- Utilizing specific immunophenotypic markers is essential for differentiating IDCS from other neoplasms.
- Prompt diagnosis and appropriate treatment, including surgery and irradiation, are vital for patient outcomes.
Abstract:
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.