Customize your JAMA Network experience by selecting one or more topics from the list below.
Pfeiffer ML, Yin VT, Myers J, Esmaeli B. Regional Nodal Recurrence of Sebaceous Carcinoma of the Caruncle 11 Years After Primary Tumor Resection. JAMA Ophthalmol. 2013;131(8):1091–1092. doi:10.1001/jamaophthalmol.2013.321
Sebaceous carcinoma is a rare cancer with a predilection for the periocular region. The vast majority of cases of metastasis of sebaceous carcinoma are detected within 5 years after diagnosis and management of the primary tumor.1,2 Herein, we describe a case of regional nodal recurrence of sebaceous carcinoma of the caruncle 11 years after primary tumor resection.
A 69-year-old man presented to our institution in June 2012. Eleven years earlier, in September 2001, he had been diagnosed as having sebaceous carcinoma of the left caruncle, which was surgically excised by a Mohs surgeon with reportedly negative margins. Since 2001, the patient had undergone ocular examination and magnetic resonance imaging annually and scans with results that had been negative for recurrence. However, examination in March 2012 revealed an enlarged left submandibular lymph node, which was initially treated with oral antibiotics with no improvement. The node was surgically excised, and pathologic examination confirmed metastatic sebaceous carcinoma with extracapsular extension. The patient was referred to our institution for further evaluation and treatment.
At presentation to our institution, the pertinent findings on examination included scarring and webbing of the caruncle, scar tissue along the canaliculi, and a palpable scar at the site of recent lymph node excision. Computed tomography of the head and neck with contrast showed operative changes in the neck but no evidence of tumor recurrence at the left caruncle. Findings on ultrasonography of the neck were negative for lymphadenopathy, and computed tomography of the thorax with contrast showed no evidence of metastatic disease. A completion neck dissection was recommended. In July 2012, the patient underwent neck dissection with concurrent biopsy of the left caruncle including conjunctival map biopsies. Pathologic examination of the excised left caruncular tissue showed scar tissue but no tumor; the conjunctival map biopsy results were negative for tumor. Twenty-five lymph nodes were dissected, and all were free of disease. Given that the previously excised positive lymph node demonstrated extracapsular extension, postoperative adjuvant irradiation of the nodal basins of the neck was recommended.
Clinically evident nodal metastasis developed in this patient 11 years after treatment of primary sebaceous carcinoma of the left caruncle. To our knowledge, regional or distant metastasis of primary sebaceous carcinoma has not previously been reported this late after excision for any primary tumor location.
Review of the literature reveals reported rates of regional nodal metastasis of 4% to 20% for sebaceous carcinoma of the eyelid and conjunctiva.1,3-6 In a recent analysis of 50 patients with eyelid sebaceous carcinoma from our institution, we found that primary tumors classified as T2b or more extensive or at least 10 mm in greatest diameter were associated with an increased risk of regional nodal metastasis.3 On the basis of this observation, we recommended sentinel lymph node biopsy in patients with either of these primary tumor characteristics to detect micrometastasis not detected by palpation or routine imaging of regional lymph nodes.
In another recent report of sebaceous carcinoma from all head and neck sites, higher histologic grade was associated with a higher rate of regional metastasis, and 12 of 79 poorly differentiated eyelid sebaceous carcinomas (15%) metastasized, compared with 0 of 19 well-differentiated cases.6
The standard local treatment for sebaceous carcinoma is complete surgical excision with negative margins. Mohs surgery or similar techniques with frozen-section control of margins are often advocated for skin cancer to achieve negative margins and preserve healthy tissues; however, owing to the propensity for skip lesions (intraepithelial neoplasia) in sebaceous carcinoma, microscopic intraepithelial neoplasia may be present even with negative margins and may lead to local recurrences. It is interesting that our patient developed a late regional nodal metastasis without any evidence of local recurrence. One plausible explanation is that the primary sebaceous carcinoma in the caruncle was a very low-grade, slow-growing carcinoma that gained access to the lymphatic channels at the time of original diagnosis and before surgical removal of the ocular tumor and, because of its slow-growing nature, it took 11 years for the nodal metastasis to reach a size to be detectable on palpation by the patient.
Typical follow-up for a patient with periocular sebaceous carcinoma includes serial examination of the ocular surgical site, palpation of the regional lymph nodes, and imaging (eg, ultrasonography or computed tomography) of the regional lymph nodes for 5 years after resection of the primary tumor. Although it is unrealistic, on the basis of this single case, to recommend surveillance of patients with periocular sebaceous carcinoma beyond 5 years, it is important for clinicians and patients to be aware of the potential for late nodal metastasis of sebaceous carcinoma beyond the initial 5 years after diagnosis and treatment of the ocular tumor. Patients with ocular and periocular sebaceous carcinoma should be educated regarding the possible but rare incidence of late relapse in the regional lymph nodes and the location of lymph nodes at risk in the parotid and submandibular regions.
Corresponding Author: Bita Esmaeli, MD, Section of Ophthalmology and Department of Head and Neck Surgery, Unit 1445, University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030 (firstname.lastname@example.org).
Published Online: June 13, 2013. doi:10.1001/jamaophthalmol.2013.321.
Conflict of Interest Disclosures: None reported.
Funding/Support: The University of Texas MD Anderson Cancer Center is supported in part by Cancer Center Support Grant CA016672 from the National Institutes of Health.
Create a personal account or sign in to: