Follicular dendritic cell sarcoma of the tonsil: a case report and literature review.Abstract
We describe a case of follicular dendritic cell follicular dendritic cell
Any of the cells present in aggregates of B cells that trap antigen-antibody complexes on their dendritic processes. Also called dendritic cell. sarcoma (FDCS) of the tonsil tonsil
Small mass of lymphoid tissue in the wall of the pharynx. The term usually refers to the palatine tonsils on each side of the oropharynx. They are thought to produce antibodies to help prevent respiratory and digestive tract infection but often become infected in a 59-year-old woman. She was successfully treated with excision of the mass and postoperative radiation therapy. According to our review of the literature, only 25 cases of extranodal FDCS in the head and neck have been previously reported, including only 10 cases that involved a tonsil. We briefly review these earlier reports, and we discuss the diagnosis and management of FDCS.
Follicular dendritic cells are nonlymphoid, nonphagocytic accessory cells of the lymphoid system. Their function is to capture and present antigens and immune complexes to B cells. (1) Follicular dendritic cell sarcoma (FDCS) is a rare entity, as evidenced by the small number of cases reported in the literature. Only 25 cases of extranodal FDCS in the head and neck have been published, (2) and only 10 of these have involved the tonsil. (2-10) In this article, we describe the eleventh such case, and we discuss the diagnostic and treatment considerations.
A 59-year-old woman presented to the otolaryngology clinic with a 2-year history of a right tonsil mass that had been found by a physician at another institution. The patient had a history of obstructive sleep apnea Obstructive sleep apnea (OSA)
A potentially life-threatening condition characterized by episodes of breathing cessation during sleep alternating with snoring or disordered breathing. with snoring. She denied dysphagia, odynophagia, weight loss, and otalgia otalgia /otal·gia/ (o-tal´jah) pain in the ear; earache.
Pain in the ear; earache.
Examination confirmed the presence of a 4-cm exophytic mass involving the right tonsil. No other masses or lymphadenopathies were noted. Computed tomography (CT) of the neck with contrast demonstrated a right oropharyngeal oropharyngeal /oro·pha·ryn·ge·al/ (-fah-rin´je-al)
1. pertaining to the mouth and pharynx.
2. pertaining to the oropharynx. mass with no extension into the parapharyngeal space (figure 1).
[FIGURE 1 OMITTED]
The patient was brought to the operating room for endoscopy and biopsy. Histopathologic analysis of the specimen identified FDCS. Hematoxylin and eosin (H&E) stains identified spindle- and oval-shaped cells forming whorls with frequent multinucleated multinucleated
characterized by having more than one nucleus per cell.
multinucleated giant cell
see giant cell. giant cells (figure 2). Immunohistochemical stains were positive for CD21, CD23, and CD35 and negative for CDlp, S-100, CD20, CD3, CD31, CD34, and cytokeratin.
[FIGURE 2 OMITTED]
The mass was excised (figure 3) via wide local excision of the right tonsil with primary closure. The patient had no evidence of regional or distant metastasis. She underwent postoperative radiation therapy, receiving 60 and 50 Gy to the oropharynx oropharynx /oro·phar·ynx/ (-far´inks) the part of the pharynx between the soft palate and the upper edge of the epiglottis.
n. and neck, respectively. Eighteen months after the cessation of treatment, she exhibited no evidence of recurrence.
[FIGURE 3 OMITTED]
Our analysis of the 10 previously reported cases of FDCS of the tonsil revealed a 1:1 distribution of men and women and an age range of 18 to 77 years (mean: 45). (2-10) Except for 1 patient who received preoperative radiation, all patients were treated primarily with tonsillectomy tonsillectomy /ton·sil·lec·to·my/ (ton?si-lek´tah-me) excision of a tonsil.
Surgical removal of tonsils or a tonsil. . Radical neck dissection Radical Neck Dissection Definition
Radical neck dissection is an operation used to remove cancerous tissue in the head and neck.
Purpose was included in 4 of these cases. Only 1 instance of cervical metastasis was found during the initial workup. Postoperative radiation was performed in 3 cases, with chemotherapy added in 2 of them. Follow-up, which ranged from 3 months to 8 years, yielded 2 cases of recurrence. One case involved a local recurrence with cervical metastasis at 4.5 years, and the other involved metastasis to the lung.
The neoplastic neoplastic /neo·plas·tic/ (ne?o-plas´tik)
1. pertaining to a neoplasm.
2. pertaining to neoplasia.
pertaining to neoplasia or a neoplasm. process associated with follicular dendritic cells was first described by Monda et al in 1986. (11) The diagnosis of FDCS is based on morphology, immunohistochemistry, and electron microscopy. (10) FDCS cells are spindle- and oval-shaped with plump cytoplasm arranged in whorl whorl
1. A form that coils or spirals; a curl or swirl.
2. A turn of the cochlea or of the ethmoidal crest.
3. An area of hair growing in a radial manner.
4. and storiform patterns. The cytoplasm is eosinophilic eosinophilic /eo·sin·o·phil·ic/ (-fil´ik)
1. readily stainable with eosin.
2. pertaining to eosinophils.
3. pertaining to or characterized by eosinophilia. and fibrillar fi·bril·lar or fi·bril·lar·y
1. Relating to a fibril.
2. Relating to the fine rapid contractions or twitchings of fibers or of small groups of fibers in skeletal or cardiac muscle. . The nuclei are also spindle- and oval-shaped within nuclear membranes. There are variable degrees of mitotic activity. (12)
FDCS stains positive for CD21, CD23, and CD35. (13) Monoclonal FDCS-specific markers include R4/23, KiM4, KiM4p, and Ki-FDC1p. (10) The reported specificity of the FDCS tumor markers ranges from 63 to 94%. (14) The diagnosis of FDCS can be elucidated if the cell is immunoreactive immunoreactive
exhibiting immunoreactivity. to one or more of the above-mentioned FDCS markers.
The differential diagnosis for FDCS includes ectopic ectopic /ec·top·ic/ (ek-top´ik)
1. pertaining to ectopia.
2. located away from normal position.
3. arising from an abnormal site or tissue.
adj. meningioma meningioma /me·nin·gi·o·ma/ (me-nin?je-o´mah) a benign, slow-growing tumor of the meninges, usually next to the dura mater, which may invade the skull or cause hyperostosis, and often causes increased intracranial pressure; it is usually , ectopic thymoma Thymoma Definition
Thymomas are the most common tumor of the thymus.
The thymus is located in the upper chest just below the neck. , metastatic carcinoma, malignant melanoma, malignant fibrous histiocytoma malignant fibrous histiocytoma
A deeply situated tumor, especially on the extremities of adults, frequently recurring after surgery and metastasizing to the lungs. , and large-cell lymphoma. (12) These entities differ from FDCS structurally and immunohistochemically.
The behavior of FDCS is similar to that of low- and intermediate-grade malignant neoplasms and much like that of soft-tissue sarcoma. The similar properties include an increased incidence of local recurrence and occasional regional and distant metastases. (12)
The optimal treatment of FDCS has not been determined because of the rarity of this tumor. Surgical excision has been the most common treatment. Postoperative radiation and even chemotherapy have been tried, but their survival benefit is unknown. (12)
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Chad McDuffie, MD; Timothy S. Lian, MD; Joel Thibodeaux, MD
From the Department of Otolaryngology-Head and Neck Surgery (Dr. McDuffie and Dr. Lian) and the Department of Pathology (Dr. Thibodeaux), Louisiana State University Health Sciences Center, Shreveport.
Reprint requests: Timothy S. Lian, MD, Department of Otolaryngology-HNS, Louisiana State University Health Sciences Center, 1501 Kings Hwy., Shreveport, LA 71103. Phone: (318) 675-6262; fax: (318) 675-6260; e-mail: firstname.lastname@example.org
The information in the article was originally presented as a poster at the Southern Section meeting of the Triological Society; Jan. 13-15, 2005; Miami.