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Delayed lymph node metastasis of excised caruncular sebaceous carcinoma in an atypical demographic

      Sebaceous cell carcinoma is a rare tumour that accounts for 1% to 5% of malignant eyelid tumours in white populations.
      • Valenzuela-Flores G.
      • Mozas-Dávila D.
      • Rodríguez-Reyes A.A.
      • Gómez-Leal A.
      Sebaceous gland carcinoma of the eyelids.
      However, in East and South Asian populations in which basal cell carcinoma is less frequent, sebaceous cell carcinoma is more frequent. A recent review of malignant eyelid tumours in Western Kowloon, Hong Kong, reported that 11.1% of their tumours were sebaceous cell carcinoma.
      • Mak S.T.
      • Wong A.C.
      • Io I.Y.
      • Tse R.K.
      Malignant eyelid tumours in Hong Kong 1997-2009.
      It originates in meibomian glands and glands of Zeis.
      • Shields J.A.
      • Demirci H.
      • Marr B.P.
      • Eagle R.C.
      • Shields C.L.
      Sebaceous carcinoma of the eyelids: personal experience with 60 cases.
      Most commonly found on the upper and lower eyelids, it can masquerade as chronic unilateral blepharitis, recurrent chalazia, basal cell carcinoma, or sebaceous cell carcinoma.
      • Shields J.A.
      • Demirci H.
      • Marr B.P.
      • Eagle R.C.
      • Shields C.L.
      Sebaceous carcinoma of the eyelids: personal experience with 60 cases.
      This often causes delays in diagnosis. This disease rarely occurs on the caruncle. Sebaceous cell carcinoma predominantly occurs among older adults, and it is more common among females. In 1 large case report of 60 consecutive cases of periorbital sebaceous cell carcinoma, the median age was 72 years, 73% of patients were female, and only one tumour originated from the caruncle.
      • Shields J.A.
      • Demirci H.
      • Marr B.P.
      • Eagle R.C.
      • Shields C.L.
      Sebaceous carcinoma of the eyelids: personal experience with 60 cases.
      In this article, we describe a case of lymph node metastasis of caruncular sebaceous cell carcinoma in a relatively younger male six months after the original lesion was surgically excised.
      A 49-year-old male was referred to our ophthalmic plastic surgery clinic from the medical oncology team for follow-up of a right caruncular mass that was excised overseas about six months previously. The pathology report was not available, but the patient informed us that the lesion was a sebaceous cell carcinoma. At presentation, he denied any local or constitutional symptoms suggestive of residual or metastatic disease. Medical history consisted of posttraumatic stress disorder, which was being treated pharmacologically. Examination showed a completely excised right caruncle, with minimal postoperative scar tissue formation. Otherwise, the medial canthal angle was unremarkable, as was the rest of the ocular examination. On family history, his mother had benign colorectal tumours. Three maternal great uncles and one maternal great grandfather had colorectal cancer. His more immediate relatives including siblings were healthy.
      Because of the lack of a pathology specimen from overseas, the bed of the caruncle was re-excised at our institution. This, however, showed no carcinoma on pathological examination. Further investigations included magnetic resonance imaging and computed tomography (CT) of the head and neck, as well as a CT scan of the chest and abdomen. No abnormalities were reported, except for a nonspecific 1.4-cm nodule of the right posterior renal fascia.
      Two months later, he presented with a swollen right preauricular lymph node, and a fine needle biopsy revealed metastatic sebaceous cell carcinoma. A repeat head and neck CT scan showed an encapsulated right parotid gland mass measuring 2.1 cm. Surgical removal of the right parotid and salivary gland, and right neck lymph node dissection was undertaken by the otolaryngology service. Pathologic examination of the surgical specimens showed metastatic sebaceous cell carcinoma of the parotid gland, but no involvement in any of the 11 dissected lymph nodes. There was no evidence of perineural or lymphovascular invasion. Postoperatively, he received adjuvant radiation therapy to the area (6600 Gy in 33 fractions). A second re-excision of the original caruncular area revealed a squamous papilloma, but no evidence of sebaceous cell carcinoma.
      He tolerated all his treatments well. Moderate keratoconjunctivitis after radiation treatment was managed successfully with short-term mild topical steroids. Between the major surgery and radiation therapy, he was treated for pathologically confirmed actinic keratosis in the pretibial regions bilaterally. A right posterior auricular cyst was drained, and pathology revealed inflammatory cells with rare nonspecific atypical cells. He is currently being followed closely by medical oncology, otolaryngology, our ophthalmic plastic surgery clinic, and the familial oncology clinic. The latter clinic ruled out Muir-Torre syndrome and hereditary nonpolyposis colorectal cancer syndrome. Sixteen months after his initial presentation to our institution, he remains recurrence-free.
      Sebaceous cell carcinomas can be locally invasive and can metastasize to lymph nodes and distant organs. There are a few reports in the literature of eyelid sebaceous cell carcinomas producing lymph node metastases after the original tumour was excised.
      • Shields J.A.
      • Demirci H.
      • Marr B.P.
      • Eagle R.C.
      • Shields C.L.
      Sebaceous carcinoma of the eyelids: personal experience with 60 cases.
      • Warnecke K.K.
      • Sieg P.
      Sebaceous carcinoma of the eyelids—case reports and review of the literature.
      • Ho V.H.
      • Ross M.I.
      • Prieto V.G.
      • Khaleeq A.
      • Kim S.
      • Esmaeli B.
      Sentinel lymph node biopsy for sebaceous cell carcinoma and melanoma of the ocular adnexa.
      Where reported, the time span between excision and metastasis detection ranged from 17 months to 7 years.
      • Shields J.A.
      • Demirci H.
      • Marr B.P.
      • Eagle R.C.
      • Shields C.L.
      Sebaceous carcinoma of the eyelids: personal experience with 60 cases.
      • Warnecke K.K.
      • Sieg P.
      Sebaceous carcinoma of the eyelids—case reports and review of the literature.
      In Shields et al.’s3 article, it is unclear whether their one case of caruncular sebaceous cell carcinoma had a metastasis. Therefore, to our knowledge, this is the first reported case of a caruncular sebaceous cell carcinoma presenting with lymph node metastasis after surgical excision of the original lesion. Moreover, there were only six months between the initial excision of the caruncular mass and the appearance of the lymph node metastasis, which is a significantly shorter period than what is reported in the literature. This can be partially explained by the fact that sebaceous cell carcinomas are known to masquerade as more benign lesions, thus evading timely diagnosis and excision.
      Sebaceous cell carcinoma is primarily a disease of older adults. Shields et al.
      • Shields J.A.
      • Demirci H.
      • Marr B.P.
      • Eagle R.C.
      • Shields C.L.
      Sebaceous carcinoma of the eyelids: personal experience with 60 cases.
      reported a median age at presentation of 72 years. When sebaceous cell carcinoma occurs in much younger patients, there is typically a history of irradiation for a childhood malignancy, especially retinoblastoma.
      • Howrey R.P.
      • Lipham W.J.
      • Schultz W.H.
      • et al.
      Sebaceous gland carcinoma: a subtle second malignancy following radiation therapy in patients with bilateral retinoblastoma.
      However, our patient has no such history, making his case even more unique.
      Given that sebaceous cell carcinoma can invade the surrounding conjunctiva and even cornea via pagetoid spread, some authors recommend map biopsies of the surrounding conjunctiva even if obvious invasion is not visible.
      • Shields J.A.
      • Demirci H.
      • Marr B.P.
      • Eagle R.C.
      • Shields C.L.
      Sebaceous carcinoma of the eyelids: personal experience with 60 cases.
      We offered this to our patient at the last visit and are waiting to hear back from him about whether he is interested in pursuing this option.
      Because there is a 5-year mortality rate of 50% to 67% for patients with lymph node metastases,
      • Rao N.A.
      • McLean I.W.
      • Zimmerman L.E.
      Sebaceous carcinoma of the eyelid and caruncle: correlation of clinicopathologic features with prognosis.
      this patient will be followed closely into the foreseeable future.

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