An 82-year-old male consulted MDACC with a right infraorbital ulcerated subcutaneous nodule and a right nontender neck mass. He had history of SRCC arising in the right orbit diagnosed 13 years prior to presentation at MDACC, followed by radiation therapy, chemotherapy, and right orbital exenteration. His family history revealed lung cancer in one brother, and one sister died of stomach cancer. A CT scan of the maxillofacial area and neck confirmed soft tissue thickening along the right infraorbital region () and metastases to the right intraparotid and right level II lymph nodes. A CT scan of the chest, abdomen, and pelvis showed no evidence of possible second primary disease or metastases. Revision of the right orbital exenteration, parotidectomy, and right neck dissection were performed, and positive resection margins were identified by intraoperative frozen section analysis. The indurated mass involving periorbital tissue and right upper cheek measured 5.3 × 4.5 cm. Microscopically, multifocal cords of signet ring cells arising in close proximity to eccrine dermal ducts were observed (), extending into soft tissue and surgical margins of resection. The patient underwent chemoradiation and passed away 5 years after the final treatment (18 years from initial presentation). The case was previously reported by Requena et al. [].
A 40-year-old male consulted MDACC with a history of thickening of the left medial lower eyelid and enlargement of a caruncle. A CT scan of orbits and head and neck soft tissue demonstrated an enhancing and infiltrative mass along the inferomedial aspect of the left anterior orbit () and no evidence of facial or cervical lymphadenopathy. PET/CT scan performed in an outside facility did not detect second primary malignancy or metastatic disease. Radical surgical resection of the left orbital, eyelid, and lacrimal sac mass with intraoperative assessment of margins was performed. Grossly, the specimen measured 4.0 × 3.5 cm. Histopathologically, neoplastic cells forming a mass lesion adjacent to the punctum and infiltrating caruncle was seen (). Cords of pleomorphic cells with histiocytoid appearance infiltrating connective tissue and depicting vesicular nuclei, large nucleoli, and eosinophilic cytoplasm with intracytoplasmic vacuolization were identified ( inset). Perineural invasion was present. The patient underwent proton therapy and chemotherapy. Thirteen months after treatment, the patient had a left neck dissection with metastatic adenocarcinoma to two of six lymph nodes at level III.