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Sellar Glomangioma

Article in Endocrine Pathology September 2011


DOI: 10.1007/s12022-011-9179-2 Source: PubMed

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Endocr Pathol (2011) 22:218221
DOI 10.1007/s12022-011-9179-2

Sellar Glomangioma
Julius O. Ebinu & Mehdi Shahideh &
George M. Ibrahim & Allan Vescan & Fred Gentili &
Rowena Ridout & Sylvia Asa & Gelareh Zadeh

Published online: 13 September 2011


# Springer Science+Business Media, LLC 2011

Abstract Glomus tumors of the sellar region are exceed- Introduction


ingly rare with only two reported cases in the literature. A
case of a sellar glomangioma in a 72-year-old man is Glomus tumors are benign mesenchymal neoplasms com-
described. The tumor had the radiographic and gross posed of cells resembling modified smooth muscle cells of the
appearance of a pituitary adenoma. Microscopically, the normal glomus body [1]. These benign neoplasms typically
tumor was composed of epithelioid cells surrounding occur in the dermis or subcutis of the extremities, and are
prominent small vascular channels. The tumor cells had commonly found in the subungual region of the fingers, the
indistinct cell borders and monotonous nuclei. Histological palm, wrist, forearm, and foot [2]. They may also occur in
and immunohistochemical analysis identified the sellar locations in which glomus bodies are sparse or absent. Such
tumor as a glomangioma. Here, we report the clinicopath- sites include the gastrointestinal tract, nerves, bone, nose,
ological features of a third case of a sellar glomus tumor and the sella turcica [1, 2]. Glomus tumors of the sellar
and review the literature. region are exceptionally rare; at present, there are only two
reported cases [3, 4]. Herein, we describe the clinicopatho-
Keywords Pituitary . Glomangioma . Glomus tumor logical features of another case of glomangioma occurring in
the sella turcica and review the literature.

Case Report
J. O. Ebinu : M. Shahideh : G. M. Ibrahim : F. Gentili :
G. Zadeh (*) A 72-year-old man with a history of chronic sinusitis,
Department of Surgery, Division of Neurosurgery,
hypertension, and hypercholesterolemia presented to medical
Toronto Western Hospital,
399 Bathurst Street, 4W-439, attention following a routine ophthalmological examination in
Toronto, ON M5T 2S8, Canada January 2010. At the time, he admitted to having frequent
e-mail: Gelareh.Zadeh@uhn.on.ca headaches secondary to his chronic sinus problems but was
otherwise well. His ophthalmological exam revealed a
A. Vescan
Department of Otolaryngology Head and Neck Surgery, bitemporal hemianopsia. The patient was apparently unaware
Mount Sinai Hospital, of his visual field deficit. Pertinent investigations included a
Toronto, Canada computed tomographic (CT) scan of the head that showed a
homogenously enhancing 23-cm intrasellar mass with
R. Ridout
Division of Endocrinology, Toronto Western Hospital, evidence of suprasellar extension (Fig. 1a). Magnetic reso-
Toronto, Canada nance imaging (MRI) confirmed the presence of an intra-
sellar mass lesion with an extensive suprasellar component,
S. Asa
and lateral extension into bilateral cavernous sinuses
Department of Pathology,
University Health Network, University of Toronto, (Fig. 1bd). Minimal extension into the roof of the sphenoid
Toronto, ON, Canada sinus on the right was also noted. While these findings were
Endocr Pathol (2011) 22:218221 219

Fig. 1 Radiographic appearance


of a sellar glomangioma. a Axial
non-contrast CT scan demon-
strating a hyperdense suprasellar
lesion. MRI images of the sellar
glomangioma. b Axial T2-
weighted MRI. T1-weighted
MRI with gadolinium in c sag-
ittal and d coronal views

suggestive of a pituitary macroadenoma, the differential MRI revealed the presence of minimal residual tumor. The
diagnosis of tumors arising in the sellar region includes patient underwent full CT examination of chest, abdomen,
craniopharyngioma, tumors of the hypothalamus and neuro- and pelvis, and no other pathology was identified.
hypophysis such as glioma, meningioma, and others, germ
cell tumors, hematologic tumors, mesenchymal and vascular
lesions, and metastatic tumor [5]. A full endocrine work-up Methods and Results
did not show any evidence of endocrinopathy, suggesting
that this was a nonsecretory mass lesion. The patient Histologic Examination
underwent transsphenoidal resection of the pituitary mass.
Intraoperatively, mucosal injection with an irregular surface The tumor was composed of epithelioid cells arranged in an
contour was noted on the posterior aspect of the nasal organoid fashion around prominent small, thin-walled vascu-
septum bilaterally. This involved approximately 1 cm of the lar channels (Fig. 2a). The tumor cells had indistinct cell
posterior septum with extension on to the anterior aspect of borders and relatively monotonous round to oval nuclei. The
the sphenoid face. A full thickness resection of the posterior GordonSweet reticulin stain demonstrated a complex
septum was performed in order to rule out a sinonasal pattern of reticulin deposition around individual tumor cells.
malignancy. Intraoperative frozen section was negative for
malignancy, and we therefore proceeded with resection of the Immunohistochemistry
sellar lesion.
Postoperatively, the patient did well, and a repeat Morphologically, the tumor cells exhibited properties of a
ophthalmological assessment demonstrated a significant spindle cell neoplasm such as spindle cell oncocytoma,
improvement of his visual fields. While gross total pituicytoma, and meningioma. By immunohistochemistry,
resection was thought to have been achieved, postoperative the tumor cells were immunoreactive for smooth muscle
220 Endocr Pathol (2011) 22:218221

Fig. 2 Sellar glomangioma immunohistological pattern. a Hematox- The tumor cells of sellar glomangioma are strongly and diffusely
ylin and eosin (H&E) staining of the tumor. The tumor is composed of positive for smooth muscle actin (SMA) and vimentin. d CD34
epithelioid cells surrounding prominent small vascular channels. The identifies prominent vascular channels
tumor cells have indistinct cell borders and monotonous nuclei. b, c

actin (SMA) and were strongly and diffusely positive for the normal glomus body [1]. Glomus bodies are associated
vimentin (Fig. 2b, c). CD34 identified prominent vascular with arteriovenous anastomoses and are thought to function
channels (Fig. 2d). The tumor cells were negative for as thermoregulators [2, 6]. Glomus tumors typically occur
keratins, chromogranin, GFAP, tryosine hydroxylase, EMA, in sites where glomus bodies are present, such as in the
desmin, and galectin-3 as well as ER, PR, and TTF-1. skin, and predominantly in the nail beds and fingertips
Stains for S100 and CD68 revealed scattered positive small where they contribute to thermal regulation [2]. While these
cells that were not considered to be neoplastic. The MIB-1 neoplasms are thought to be associated with glomus bodies,
labeling index was >10%. they have also been described in sites where glomus bodies
are absent [1, 7].
Electron Microscopy Many tumors previously reported as glomangiomas have
now been reclassified as solitary fibrous tumors. However,
By electron microscopy, the tumor cells exhibited smooth in the sellar lesions, the presence of smooth muscle actin in
muscle differentiation and formed basement membrane tumor cells supports proposed myoid differentiation of
surrounding individual cells. Myofibrils within the cyto- pericytic cells and distinguishes these lesions from solitary
plasm showed focal condensation into dense bodies. fibrous tumors that also occur in this region [8, 9]. It is
important to note that glomus tumors should not be
confused with glomus jugulare tumors or paragangliomas,
Discussion which are neuroendocrine tumors that originate from
chromaffin cells in paraganglia or chromaffin-negative
Glomus tumors are benign mesenchymal neoplasms com- glomus cells derived from the embryonic neural crest
posed of cells resembling modified smooth muscle cells of [10]. Sellar glomangiomas are considered to be true
Endocr Pathol (2011) 22:218221 221

pericytic tumors, and they have been postulated to arise In light of the limited literature surrounding sellar
from the gomitoli of the posterior lobe of the pituitary gland glomangiomas, we report another case of a sellar gloman-
[3]. The gomitoli are unique vascular structures in the upper gioma. Although rare, these lesions have the potential to
infundibular stem of the pituitary; they are composed of a recur locally with malignant progression [4], thereby
central muscular artery surrounded by a spiral of capillaries. underscoring the importance of following these patients
They are implicated in regulation of the rate of pituitary closely over a long period of time.
portal blood flow that transports hypothalamic regulatory
hormones to the adenohypophysis. Thus, their control of
blood flow is analogous to that of the thermoregulatory References
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