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Giant-cell tumor of the tendon sheath in the external auditory canal.


Giant-cell tumor of the tendon sheath (GCTTS) and pigmented villonodular synovitis belong to the same type of benign proliferative lesions originating in the synovia that usually affect the joints, bursae, and tendon sheaths. They frequently involve the hands, knees, ankles, and feet. We report a case of GCTTS in the external auditory canal in a 53-year-old woman who presented with hearing loss, fullness, and a sessile lesion protruding from the anterior wall of her external ear canal. The 1.5-cm diameter mass was spherical, well encapsulated, firm, and covered with normal skin. The lesion was completely excised, and the patient's symptoms resolved. No recurrence was detected at 2 years of follow-up.


Giant-cell tumor of the tendon sheath (GCTTS) and pigmented villonodular synovitis belong to the same type of benign proliferative lesions originating in the synovia that usually affect the joints, bursae, and tendon sheaths. (1) Tumors with similar histology have been described in the pancreas, ovary, larynx, urinary tract, and salivary glands. (2,3)

Chassaignac first described these benign soft-tissue masses in 1852. (4) Many authors subsequently reported that he "overstated" their biologic potential by calling them "cancers of the tendon sheath."

GCTTSs usually exhibit a slow growth rate, and they are associated with a low incidence of malignant behavior. Three forms have been described: localized, intra-articular, and diffuse. The localized form is the second most common tumor of the hand (simple ganglion cysts are the most common). (5) Localized GCTTS mainly occurs on the volar side of the first three fingers; the second most common site is the ankle/foot area. The intra-articular form occurs almost exclusively in the knee, and the diffuse form usually affects the knee and ankle.

As far as we know, there has been only 1 reported case of GCTTS of the temporomandibular joint (6) and 1 case in the pinna. (7) To the best of our knowledge, no case involving the external ear canal (EAC) has been previously reported. We report such a case.

Case report

A 53-year-old woman was referred to our ENT department for evaluation of a sessile lesion that had protruded from the anterior wall of the EAC. She complained of associated hearing loss and fullness. Physical examination revealed that the 1.5-cm mass was spherical, well encapsulated, firm, and covered with normal skin. The EAC was otherwise normal. Magnetic resonance imaging (MRI) demonstrated an intermediate signal on T1-and T2-weighted spin-echo sequences. No involvement of the cartilaginous layer of the EAC was detected, and the tympanic membrane was intact. Whole-body computed tomography (CT) was performed to look for other lesions, but none was found. The patient's history included removal of a GCTTS from her thumb many years earlier; that tumor had recurred at the same site 1 year later and it, too, was removed.

We performed a complete surgical resection of the lesion. The gross specimen measured 1.3 x 0.8 x 0.4 cm. Light microscopy identified mononuclear cells and scattered multinucleated giant cells in a hyalinized collagen fiber matrix. Immunophenotyping was positive with CD68 and negative with smooth-muscle actin, cytokeratins 5 and 6, p63, and S-100 protein. The histologic specimen exhibited the same features as did the earlier lesions that had been removed from her hand, and it was diagnosed as a typical localized GCTTS.

Wound healing by secondary intention took almost 3 weeks. We did not suture the ear because of the large defect, and this avoided disruption of the temporomandibular joint and allowed the patient to chew without discomfort.

The patient's postoperative course was uneventful, and her symptoms resolved completely. No recurrence was detected at 2 years of clinical follow-up.


No metastasis of a GCTTS has ever been reported. With respect to our case, it is very unusual that two different GCTTSs developed at two different sites. Also, a location in the EAC is unprecedented.

In the EAC, we find many lesions, including osteomas, exostoses, fibromas, angiomas, cholesteatomas, and epidermal and sebaceous cysts. A lesions gross appearance and orientation in the EAC (medial to proximal) provide clues to its nature, as does the number of lesions. Imaging, including a comparison of CT and MRI findings, is important in reviewing the differential diagnosis. Imaging can allow the clinician to avoid performing a biopsy of a vascular lesion.

Radical surgical excision is the treatment of choice, and long-term follow-up is recommended to look for recurrences.


(1.) Ushijima M, Hashimoto H, Tsuneyoshi M, Enjoji M. Giant cell tumor of the tendon sheath (nodular tenosynovitis). A study of 207 cases to compare the large joint group with the common digit group. Cancer 1986;57(4):875-84.

(2.) Behzatoglu K, Durak H, Canberk S, et al. Giant cell tumor-like lesion of the urinary bladder: A report of two cases and literature review; giant cell tumor or undifferentiated carcinoma? Diagn Pathol 2009;4:48.

(3.) Somerhausen NS, Fletcher CD. Diffuse-type giant cell tumor: Clinicopathologic and immunohistochemical analysis of 50 cases with extraarticular disease. Am J Surg Pathol 1999;24(4):479-92.

(4.) Chassaignac CM. Cancer de la game des tendons. Gazette Hopital Civils Militaires 1852;47:185-6.

(5.) Gude W, Morelli V. Ganglion cysts of the wrist: Pathophysiology, clinical picture, and management. Curr Rev Musculoskelet Med 2008;1(3-4):205-11.

(6.) Holscher AH, Rahlf G, Stennert E, Schauer A. Giant cell tumor of tendon sheath in association with synovialchondroma. An unusual tumor-combination at the temporomandibular joint (author's transl) [in German]. Laryngol Rhinol Otol (Stuttg) 1978;57(10):904-7.

(7.) Ismail-Koch H, Ismail A, Heathcote K, et al. Extraosseous giant cell tumor of the pinna presenting in a child: Case report and review of the literature. The Internet Journal of Otorhinolaryngology 2010;12(1). http://ispub.eom/IJORL/12/l/6443. Accessed Aug. 4, 2014.

Margherita Trani, MD; Massimo Zanni, MD; Paolo Gambelli, MD

From the Department of Otolaryngology, Sassuolo Hospital, Sassuolo, Italy.

Corresponding author: Margherita Trani, MD, Nuovo Ospedale Civile di Sassuolo, Via F. Ruini 2, 41049 Sassuolo (MO), Italy. Email: niki.
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Author:Trani, Margherita; Zanni, Massimo; Gambelli, Paolo
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Date:Oct 1, 2014
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