Abstract
Histiocytic disorders can be classified according to the distribution pattern of the lesions and the organs involved. Non-Langerhans-cell histiocytosis is a rare group of diseases that have varied clinical presentations ranging from isolated masses to diffuse systemic eruptions. We discuss a patient who initially presented with a vocal cord lesion and was ultimately diagnosed with adult onset xanthogranuloma.
1
Introduction
Histiocytes are mononuclear phagocytic cells that derive from the bone marrow and migrate through the blood stream to various organ systems. Histiocytic disorders involve over proliferation of these cells, leading to organ damage and tumor formation.
These diseases can be separated into distinct groups based on the type of cells involved and the constellation of clinical findings ( Fig. 1 ). The World Health Organization (WHO) proposes to classify histiocytosis into three categories: Langerhans-cell histiocytosis, non-Langerhans-cell histiocytosis, and malignant histiocytosis . Diagnosis requires integration of clinical, pathological, and radiographic evaluation. The disease pattern is not always immediately apparent; for example, isolated lesions may appear first, giving only pathological findings of sinus histiocytosis. Additional work-up is then necessary to identify asymptomatic lesions.
We present a patient who initially presented with a vocal cord mass, which upon removal showed sinus histiocytosis on histological examination. Subsequent work-up and temporal eruption of lesions lead to a diagnosis of adult onset xanthogranuloma.
2
Case presentation
A 56-year-old woman was referred to our clinic for evaluation of a right posterior vocal cord mass discovered incidentally on upper esophagoscopy. The mass was initially biopsied elsewhere and showed squamous mucosa with chronic inflammation, histiocytic reaction and coronal fibrosis. The patient was asymptomatic at this time with no dysphagia, voice changes, or breathing difficulty. Further examination revealed a 2-cm right lower neck mass within the right sternocleidomastoid muscle. A fine needle aspiration was performed given her past history of breast cancer. This showed only scant cells with no evidence of malignancy.
A few months later, she began to have increasing hoarseness and continued enlargement of the neck mass and sought further evaluation in our clinic. Videolaryngoscopy was performed demonstrating a tan colored, well-circumscribed, posterior vocal cord mass ( Fig. 2 ). A CAT scan of the neck was obtained that showed an ill-defined muscle density lesion within the right lower sternocleidomastoid muscle ( Fig. 3 ). The patient was then taken to the operating room for a microlaryngoscopy with excisional biopsy of the vocal cord lesion and an open excisional biopsy of the neck mass. The vocal cord lesion was attached to the posterior vocal ligament with intact overlying vocal cord epithelium. The neck mass was confined to the sternocleideomastoid muscle. Intraoperatively, we found a well-circumscribed mass without any signs of infiltrative growth pattern.
Interestingly, pathological findings in both specimens were identical. The tumors consisted of histiocytic xanthomatous infiltrates with no evidence of malignancy. Immunohistochemical staining was positive for CD68, weakly positive for muramidase, and negative for S100 and CD1a ( Fig. 4 ).
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