Spindle Cell Oncocytoma of the Adenohypophysis With Marked Hypervascularity

Abstract

A 68-year-old male presented with a very rare case of spindle cell oncocytoma (SCO), a recently identified very rare neoplasm of the anterior pituitary, manifesting as panhypopituitarism and visual field defect. The pituitary tumor with suprasellar extension was only partially resected via transsphenoidal surgery because of the tumor consistency and bleeding. Histological diagnosis was consistent with schwannoma. The tumor regrew and angiography revealed hypervascularity, so a transcranial approach was employed for the re-operation which only achieved partial resection because of intraoperative extensive bleeding. The tumor cells showed similar histological and immunohistochemical profiles to the previous specimen, but electron microscopy demonstrated that cytoplasm abundantly filled with mitochondria. The final diagnosis of SCO was established and the patient received postoperative conventional radiation therapy of 50 Gy. Only 15 cases of SCO have been reported, and the diagnosis was mistaken in many cases as schwannoma, oncocytic pituitary adenoma, or craniopharyngioma, and multiple surgeries followed by radiation therapy were required.

Update on Hypophysitis and TTF‐1 Expressing Sellar Region Masses

“…However, case reports emerging since WHO 2007 indicate that recurrences may be even more frequent than with subtotally resected pituicytomas, although the tendency to report adverse or unusual outcomes obviously can skew the literature. Cases reported by Demssie et al , Coiré et al , Borota et al , Kloub et al , Matyja et al , Fujisawa et al and Borges et al have all had recurrences, some within 5 , 9 or 18 months' time, but most over several years , or even as long as after a 16‐year interval . Whether this suggests that spindle cell oncocytoma might deserve a higher WHO grade than I is conjectural at this point.…”

“…Several of the uncommon clinical and biological features mentioned earlier for pituicytoma are, however, shared with spindle cell oncocytoma, including occasional association with other endocrine abnormalities in the same patient and tendency for bleeding or high vascularity . One of the original five patients reported by Roncaroli et al had an oncocytic thyroid adenoma , and the case reported by Borges et al had thyroid disease .…”

“…It had first been reported as a distinct entity by Roncaroli in 2002, in five patients culled from four different institutions, reflecting the rarity of the entity (90). Even with these five initial examples, only approximately 20 total cases have been published (2,8,26,37,56,75,88,102,117,118). All have occurred in adults thus far, with a mean of 56 years (38).…”

Spindle cell oncocytoma of adenohypophysis: Review of literature and report of another recurrent case

“…SCO is a rare entity, described first in 2002 with few subsequent case reports to date . As described first by Roncaroli et al , SCO occurs most commonly in middle and older age groups with mean age of 61.6 years .…”

An Unusual Oncocytic Sellar Neoplasm in a Patient with Birt-Hogg-Dubé Syndrome

“…Spindle cell oncocytoma of the adenohypophysis is a rare and diagnostically challenging entity with only twenty-four cases reported in the English literature since the first account in 2002 by Roncaroli et al, (Alexandrescu, Brown, Tandon, & Bhattacharjee, 2012;Borges, Lillehei, & KleinschmidtDeMasters, 2011;Borota et al, 2009;Coire, Horvath, Smyth, & Kovacs, 2009;Dahiya et al, 2005;Demssie et al, 2011;Farooq, Bhatt, & Chang, 2008;Fujisawa et al, 2012;Fuller, Scheithauer, Roncaroli, & Wesseling P, 2007;Kloub, Perry, Tu, Lipper, & Lopes, 2005;Matyja et al, 2010;Mlika et al, 2011;Ogiwara, Dubner, Shafizadeh, Raizer, & Chandler, 2011;Romero-Rojas et al, 2011;Roncaroli et al, 2002;Singh et al, 2012;Vajtai, Sahli, & Kappeler, 2006). The classical histologic description of SCO is that of a fascicular neoplasm arising from the adenohypophysis and composed of spindled and epithelial oncocytic cells with immunohistochemical positivity for S100, vimentin, EMA, galectin-3, and antimitochondrial antibodies (Roncaroli et al, 2002).…”