The cutaneous monocle tumor- A case of primary periocular signet-ring cell/histiocytoid carcinoma and review of the literature
Authors: Victoria Leung, Marie-Jo Olivier, Isabelle Hardy.
Author Disclosure Block: V. Leung: None. M. Olivier: None. I. Hardy: None.
Abstract Body:Purpose: To describe a case of primary periocular signet-ring cell/histiocytoid carcinoma (PSRCHC), and highlight its unique clinical presentation
Study Design: Case report and literature review
Methods: Chart review, MEDLINE and PubMed literature review
Results: A 78-year-old healthy male presented with a 2-year history of recently progressive right lower eyelid thickening. Examination revealed poorly-defined induration of the eyelid and upper cheek with no associated pain/redness/edema. There was no eyelid margin destruction/madarosis. The epidermis and conjunctiva appeared normal. Visual acuity, pupils, extraocular motility and ocular examination were normal. CT-scanning revealed ill-defined pre-septal infiltration of soft tissues anterior to the inferior orbital rim (5.1x1.6x2.1cm) with no orbital invasion/bony erosion. Incisional biopsy revealed cutaneous PSRCHC. Within 1 month, there was progression to the upper eyelid. Additional mapping biopsies confirmed upper eyelid skin/caruncule/lower bulbar and tarsal conjunctiva involvement. Systemic workup was negative.The patient underwent wide exenteration, elective neck dissection, and parotidectomy. There was tumour infiltration of the eyelids, anterior orbital soft tissues, palpebral and bulbar conjunctiva. There were no nodal/parotid metastases. The patient refused adjuvant radiotherapy for positive cutaneous margins in normal-looking lower cheek and forehead tissue, and thus underwent further surgical excision with free-flap reconstruction. The patient remains disease-free to date, 6 months post-operatively.A literature review revealed 39 cases of periocular PSRCHC. The eyelid (+/-orbit) is the most common site of cutaneous PSRCHC. 34/39 cases affected men (median age=70y, range 33-80y). The characteristic presentation of lower eyelid mass/thickening, which progresses circumferentially to affect the upper eyelid, gives PSRCHC the moniker, “monocle tumor”. However, 4 cases of bilateral involvement have been reported. On histology, there is diffuse infiltration of single cells/cords of cells between collagen bundles within dermis, with well-differentiated signet-ring or poorly-differentiated histiocytoid cells. The epidermis is typically spared. Our case is the first to report tumoral infiltration of clinically normal-looking conjunctiva.
Conclusions: PSRHC is a rare cutaneous tumor predominantly affecting the eyelid. Non-specific, non-inflammatory findings and initially indolent progression often result in delayed diagnosis, and progression to orbital and conjunctival tissues. Histopathologically, PSRCHC may be indistinguishable from metastasis (breast/gastrointestinal), which must be ruled out. Eventual aggressive, infiltrative growth necessitates radical excision with wide margins/exenteration, consideration of lymphadenectomy, and adjuvant radiation/chemotherapy. In our case, positive pre-operative map biopsies, despite normal-looking conjunctiva, rendered exenteration the surgery of choice, and highlights the importance of map conjunctival biopsies to guide surgical planning in PSRHC. Recurrence and metastases are common, but early disease-related mortality appears rare.