Sentinel lymph node biopsy in periocular merkel cell carcinoma: a case report

Filitis et al. BMC Res Notes (2017) 10:490 DOI 10.1186/s13104-017-2746-y BMC Research Notes Open Access CASE REPORT Sentinel lymph node biopsy in periocular merkel cell carcinoma: a case report Dan C. Filitis1* , Gyorgy Paragh2, Faramarz H. Samie3,1 and Nathalie C. Zeitouni4 Abstract Background: The National Comprehensive Cancer Network guidelines for Merkel cell carcinoma recommend performance of the sentinel lymph node biopsy in all patients with clinically negative nodal disease for staging and treatment. Nevertheless, sentinel lymph node biopsy in the periocular region is debated as tumors are typically smaller and lymphatic variability can make performance procedurally problematic. Case presentation: We present a case of a Caucasian patient in their seventies who presented with a 1.0 cm periocular Merkel cell carcinoma, who underwent Mohs surgery with a Tenzel flap repair, that was found to have a positive sentinel lymph node biopsy, but who, despite parotidectomy, selective neck dissection, and radiation, succumbed to the disease. Conclusions: Evidence in both the site-specific and non-specific literature demonstrates: (1) Worsening prognosis with extent of lymph node burden, (2) improvements in our abilities to perform lymphoscintigraphy, (3) locoregional and distant metastatic disease in patients with tumor sizes ≤1 cm, and (4) significant rates of sentinel lymph node positivity in patients with tumor sizes ≤1 cm. Our case supports that sentinel lymph node biopsy should be considered in all clinically nodal negative periocular Merkel cell carcinoma, regardless of size, and despite limited site-specific studies on the subject. Keywords: Merkel cell carcinoma, Periocular, Sentinel lymph node biopsy, Case report Background The sentinel lymph node biopsy (SLNB) plays an important role in the current staging and treatment of Merkel cell carcinoma (MCC) [1]. The National Comprehensive Cancer Network (NCCN) guidelines reflect this, recommending performance of the SLNB in all MCC patients that are clinically negative for nodal disease [2]. Still, whether all MCC patients are candidates for a SLNB, regardless of site of presentation or tumor characteristics, remains investigational and a matter of debate [3]. Periocular MCC represents anywhere from 5 to 20% of head and neck MCC [4, 5]. This is a unique site with a high degree of lymphatic drainage variability where *Correspondence: 1 Department of Dermatology, Columbia University Medical Center, 161 Fort Washington Avenue, 12th Floor, New York, NY 10032, USA Full list of author information is available at the end of the article tumors are often diagnosed earlier at smaller tumor sizes [6, 7]. Herein, we present a case of periocular MCC that highlights the aforementioned factors, and through review of the existing literature, aim to provide context for the decision to pursue SLNB at this distinctive site. Case presentation A Caucasian patient in their seventies with no personal or familial history of cutaneous malignancy presented with a biopsy-proven MCC, with angiolymphatic invasion of the right upper eyelid. Immunohistochemistry was positive for CK20 and negative for TTF-1. The patient underwent Mohs micrographic surgery and SLNB. At this time the small violaceous plaque measured 1.0 × 0.8 cm and clinically and histologically residual MCC was noted in the tumor-debulking specimen. After two stages with negative margins, the final surgical defect measured © The Author(s) 2017. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/ publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Filitis et al. BMC Res Notes (2017) 10:490 Page 2 of 4 2.2 × 0.8 cm. The primary site was reconstructed with a Tenzel flap immediately following Mohs surgery. The SLN was positive for metastatic MCC and the patient thereafter underwent right superficial parotidectomy with facial nerve dissection and right selective neck dissection of levels 1–4. The parotid gland and all lymph nodes removed were negative for lymph node metastasis. Eight months after initial diagnosis the patient presented with multiple subdermal sub-centimeter masses palpable in the soft tissues of the right neck. Fine needle aspiration of a representative lesion was consistent with metastatic MCC. CT of the neck and chest with contrast identified diffuse metastatic disease affecting the lungs, liver, and the adrenals. The patient started palliative radiation for the right neck but succumbed to widespread metastatic disease 1 month later. Conclusions Periocular MCC is rare, and as such, literature and data are scarce. In fact, much of what we believe about the tumor’s behavior and the role for SLNB at this site is extrapolated from MCC at other anatomic locations and data from other eyelid and conjunctival tumors. Due to large variation in lymphatic drainage, higher rates of false negatives have been associated with SLNB in the head and neck region and the periocular region is no exception [7, 8]. In the periocular region, previous false-negative values in studies looking at sebaceous carcinoma and melanoma ranged from 11 to 20% [9]. More recently however, rates of successful identification of SLN, on either pre- or intraoperative lymphoscintigraphy, were found to be as high as 97 and 100%, respectively [10–13]. Site-nonspecific MCC studies report 11–57% SLN positivity [1, 4, 14–25]. SLN positivity showed positive correlation with tumor size, tumor depth, mitotic rate, histological growth pattern, and presence lymphovascular invasion [4, 14–16]. The literature on tumor factors associated with SLN positivity in periocular tumors is limited but trends suggest correlation of nodal metastasis with increased tumor size. In a recent study, 3 of 4 patients presenting with nodal metastasis were found to have >T2b tumors at presentation, suggesting more nodal involvement with larger tumor size as has been demonstrated in the site-nonspecific literature [26]. Still, in comparison to MCC at other sites, for eyelid specific disease, performance of SNLB is seldom reported to date (Table 1). Amato et al. reported on a 61-year-old male with a right upper eyelid lesion that had a positive SLNB. Tumor characteristics were not provided [13]. Maalouf et al. [11] performed SLNB in 4 patients with periocular MCC, all with tumors ≤2 cm, and found 1 of 4 to have a positive SLNB. One of the 3 patients with negative SLNB was defined as having had a falsely negative SLNB as they developed nodal disease 6 months after surgery. (...truncated)