Interdigitating Dendritic Cell Sarcoma of the Eyelid With a Rapidly Fatal Course

Interdigitating dendritic cells participate in the immune system as antigen-presenting cells, stimulating T lymphocytes. Interdigitating dendritic cells normally are localized in the T-cell–rich areas of lymph nodes and are believed to be derived from hematopoietic precursors and to belong to the mononuclear phagocytic system. Interdigitating dendritic cells sarcoma is an extremely rare malignancy derived from these antigen-presenting cells normally localized in lymphoid organs. Only 45 cases have been reported in the literature to date.^1,2 We are unaware of previous reports of this sarcoma in the eyelid and could not find any reference to it in a MEDLINE search.

A 72-year-old man who had an unknown recurrent lesion in his lower right eyelid for 8 months was referred to our department. Two prior biopsies revealed a nevoid lesion and dermatofibroma. The patient was referred to our clinic when the tumor recurred. His best-corrected visual acuity was 0.8 OD. A 20-mm × 6-mm multinodular, ulcerated tumor was present on his right lower eyelid (Figure 1). The anterior chamber and the vitreous were free of inflammation and the lens revealed a mild senile cataract. Thepatient underwent sufficient surgical resection with a 4-mm–wide tumor-free margin and reconstruction using a tarsoconjunctival flap combined with a skin graft. Microscopically, the tumor consisted of medium to large spindle-shaped cells with hyperchromatic nuclei. The cells were arranged in fascicles and formed whorls. The mitotic count was high (Figure 2). The final diagnosis was interdigitating dendritic cell sarcoma. The tumor cells strongly expressed S-100 protein, and many tumor cells showed reactivity with antibodies to lysozyme and CD68 as well as CD45 (Figure 3). Numerous small, reactive CD3-positive T lymphocytes were mixed. Some tumor cells were positive for CD83 and fascin. No reactivity was seen with antibodies to cytokeratins, HMB-45, Melan-A, or CD1a or as markers for follicular dendritic cells, such as CD21, CD23, and CD35. The tumor staging revealed no metastatic disease. After careful deliberation of treatment options with his oncologist, the patient declined chemotherapy and local radiation therapy. Two years later, the patient had a local recurrence and metastatic lung and liver disease. A biopsy of a presumed metastasis in the lung confirmed the diagnosis. His clinical condition deteriorated and he died rapidly.

Interdigitating dendritic cell sarcoma is anextremely rare malignancy derived from antigen-presenting cells. Monocytes and related cells can be divided into 2 major categories: phagocytes and dendritic cells. The dendritic cells include (1) the follicular dendritic cell, (2) the Langerhans cell, (3) the intestinal dendritic cell, (4) the indeterminate cell, and (5) the interdigitating dendritic cell. Special immunohistochemical markers help to classify these dendritic cell neoplasms. The immunohistochemical diagnosis in this case was based on the World Health Organization classification for hematopoietic and lymphoid tissues.³ Patients with interdigitating dendritic cell sarcoma usually have lymph-node enlargement at initial examination, though they rarely have extranodal disease. A solitary skin tumor similar to our patient's was reported by Miracco et al.⁴ This sarcoma has a high potential of local recurrence and systemic disease. Of only 45 cases that have been reported in the world literature, the median overall survival (Kaplan-Meier method) from the time of diagnosis is reported to be 15 months.⁵ There is no consensus on a standard chemotherapy or radiation therapy regimen for interdigitating dendritic cell sarcoma. Chemotherapy regimens used in malignant lymphoma showed variable degrees of remission; field radiation seems to be the best treatment of localized disease.⁶

To our knowledge, interdigitating dendritic cell sarcoma has not involved the eyelid. Although optimal therapy for this tumor is yet to be determined, adjuvant chemotherapy or radiation therapy may be reasonable given its highly malignant course.

Correspondence: Dr Boldin, Department of Ophthalmology, Medical University Graz, Auenbruggerplatz 4, A-8036 Graz, Austria (ingrid.boldin@yahoo.de).

Financial Disclosure: None reported.