Head and Neck Extranodal Interdigitating Dendritic Cell Sarcoma: Case Report and Review of the Literature

Abstract

Interdigitating dendritic cell sarcoma (IDCS) is an exceedingly rare neoplasm originating from professional antigen presenting cells normally located in the T zone of the lymph node. The purpose of this report was to describe the first case of the IDCS of the submandibular gland and perform a review of the literature of head and neck IDCS. We present a case of an 81-year-old man with a 5 months history of slowly enlarging painless mass in right submandibular region. Fine needle aspiration cytology was suggestive of squamous cell carcinoma. The patient underwent surgical resection of the right submandibular gland and neck dissection. A malignant spindle cell proliferation involving the submandibular gland and colonizing one laterocervical lymph node was found. Morphology and immunophenotype prompted a differential diagnosis of a metastatic spindle cell melanoma versus an IDCS. Transmission electron microscopy was performed and supported a diagnosis of IDCS. The diagnosis of IDCS is a challenging task and may require a large array of techniques.

Successful Treatment of Interdigitating Dendritic Cell Sarcoma Presenting as Multiple Parotid Tumors

“…However, this neoplasm has been described in extranodal sites, including the liver, skin, lungs, and gastrointestinal tract [3,4]. To date, there were only 4 cases of IDCS in the parotid gland, including the present case (Table 1) [5][6][7][8]. The majority of previously reported cases included the manifestation of a painless lump, but systemic symptoms such as fever, weight loss, and fatigue are not typical [2].…”

Interdigitating dendritic cell sarcoma located in the groin: a case report and literature review

“…Cytokeratins, epithelial membrane antigen (EMA), B-cell markers, T-cell markers, CD30, CD34, ALK, langerin, and myeloperoxidase are negative. 4 – 20 …”

Interdigitating Dendritic Cell Sarcoma

“…Only about 100 cases have been reported in the literature (Saygin C et al, 2013). IDCS is derived from normal interdigitating dendritic cells (IDCs), whose role consists to present antigens to T cells and regulate cellular immune response (Saygin C et al, 2013;Lupato V 2016). An association between IDCSs and solid tumors or others hematologic malignancies (usually B-cell neoplasms) has been reported (Saygin C et al, 2013;Pokuri VK et al, 2015).…”

“…Neoplastic cells typically express, intensively and diffusely, the S100 protein (Fachetti et al, 2017). This neoplasm arises mainly involving lymph nodes (as isolated mass); whereas extranodal sites, like liver, skin, lung or gastrointestinal tract are less common (Lupato V et al, 2016). The clinical course is aggressive; some series described a median survival of 9 months in disseminated cases (Saygin C et al, 2013).…”

“…However, there is no specific marker for IDCS (Xue T et al, 2018). IDCS cells characteristically express S100, vimentin, and CD68 (variable); whereas they are consistently negative for Langerhans cell histiocytic and follicular dendritic cell markers (CD1a, langerin, CD21, CD23, and CD35) (Lupato V et al, 2016). Ki-67 proliferative index usually ranges between 0-70%, with a median value of 10% (Saygin C et al, 2013).…”