Contributed by Henry Armah, MD, PhD and Raja Seethala, MD
Published on line in October 2006
The patient is an 83-year old male with a past medical history of hypertension. He presented with an enlarging right supraorbital mass. Physical examination revealed a right supraorbital mass causing proptosis.
CT and MRI scans demonstrated a bulging right supraorbital mass displacing the globe medially and inferiorly, and invading the frontal sinus or arising from the frontal sinus.
The patient underwent a biopsy of the right supraorbital mass.
The biopsy specimen consisted of three fragments of firm gray tan tissue (0.3 x 0.3 x 0.5 cm). Microscopic examination revealed a poorly differentiated carcinoma with foamy to focally clear cytoplasm [Fig. 1] with rare mucin vacuoles highlighted by a mucicarmine and PAS after diastase treatment [Fig. 2]. The immunoprofile was as follows: epithelial membrane antigen (EMA) positive [Fig. 3], p63 positive [Fig. 4], androgen receptor negative, prostate specific antigen (PSA) negative (PSA), and thyroid transcription factor-1 (TTF-1) negative. The typing of this tumor was deferred to definitive resection.
The patient subsequently underwent a right anterior orbitotomy and right frontotemporal craniotomy with excision of tumor en bloc with part of the superior orbital rim and the anterior cranium at the level of the frontal sinus.
The tumor consisted of a portion of supraorbital bony resection (4.6 x 2.0 x 1.1 cm) with a red-tan, firm tumor (3.7 x 2.6 x 2.5 cm) extending through the cortex and protruding anteriorly into the periorbital soft tissue [Fig. 5] showing a homogenous tan cut surface [Fig. 6]. The tumor was grossly present at the deep inferior bony edge, 0.1 cm from the posterolateral bony margin, and 1.2 cm from the anteromedial bony margin.
The tumor consisted of two components: The inner component consisted mainly of a proliferation of multilayered oncocytic columnar epithelium that showed both a papillary exophytic and inverted growth patterns [Fig. 7]. Numerous mucinous microcysts, some filled with neutrophils were present. This component transitioned into the second component, a poorly differentiated nested carcinoma with oncocytic to clear cytoplasm similar to that seen on biopsy [Fig. 8]. This carcinomatous component extended through bone into surrounding soft tissue [Fig. 9] and showed focal angiolymphatic invasion [Fig. 10].
A Mucicarmine stain again showed rare positive mucin globules in the invasive tumor [Fig. 11]. A p63 immunostain highlighted the transition, staining only basal layer in the first component, but staining almost all cells of the second component [Fig. 12].