Year : 2010 | Volume
: 53 | Issue : 1 | Page : 109--111
Malignant hemangiopericytoma of pituitary fossa
Prasenjit Das1, Kunhi P Haresh2, Vaishali Suri1, Mehar Chand Sharma1, Bhawani Shankar Sharma3, Chitra Sarkar1,
1 Department of Pathology, All India Institute of Medical sciences, New Delhi, India
2 Department of Radiotherapy, All India Institute of Medical sciences, New Delhi, India
3 Department of Neurosurgery, All India Institute of Medical sciences, New Delhi, India
Neuropathology laboratory, Department of Pathology, All India Institute of medical Sciences, Academic Building, New Delhi - 110 029
Intracranial hemangiopericytomas are rare tumors with aggressive behavior. Other than the meninges, this lesion has rarely been reported in periventricular and sellar region. We report a case of malignant hemangiopericytoma in sellar region in a 47-year-old male who presented with history of sudden onset of bilateral visual disturbances. To best of our knowledge, this is the second case report of malignant hemangiopericytoma in this location. As this intracranial lesion shows aggressive behavior, in the form of recurrence or extracranial metastasis in comparison to its extracranial counterparts, diagnosis should be made cautiously.
|How to cite this article:|
Das P, Haresh KP, Suri V, Sharma MC, Sharma BS, Sarkar C. Malignant hemangiopericytoma of pituitary fossa.Indian J Pathol Microbiol 2010;53:109-111
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Das P, Haresh KP, Suri V, Sharma MC, Sharma BS, Sarkar C. Malignant hemangiopericytoma of pituitary fossa. Indian J Pathol Microbiol [serial online] 2010 [cited 2021 Jun 20 ];53:109-111
Available from: https://www.ijpmonline.org/text.asp?2010/53/1/109/59196
Hemangiopericytoma (HPC) denotes lesion having typical thin walled branching vascular pattern with pericytic differentiation of the surrounding tumor cells, which can be ascertained by immunohistochemical stains.  This tumor has commonly been described in deep soft tissue, especially in pelvis, retroperitonium and proximal lower limbs. Intracranial HPCs are very rare, accounting for less than 1% of primary central nervous system (CNS) tumors and constitute 2% to 4% of meningeal tumors. , Since the first report of CNS HPC in 1954, most of these have been reported to be originating in meninges and around ventricles. ,, Incidence in pituitary fossa is particularly very rare. ,,,,, In the literature studied, only one case of a hemangiopericytic meningioma found arising in this location is reported.  We describe a primary malignant HPC of pituitary fossa in a 47-year-old male. This is the second case report of primary malignant HPC of pituitary fossa to the best of our knowledge.
A 47-year-old male presented with a history of sudden onset of headache and progressive deterioration of vision in both eyes; left worse than right, of one month duration. There was no history of vomiting, seizures, loss of consciousness, weakness in limbs or urinary disturbance.
On examination, both pupils were normal in size and reacting to light. Examination for vision showed that with the right eye he was able to count fingers at a distance of five feet; while with the left eye he was able to count fingers only at a distance of one foot. There were bitemporal field cuts by confrontation technique. No cranial nerve deficit or motor or sensory deficit was identified.
A routine complete hemogram and biochemical tests including liver and kidney function tests were within normal range. Magnetic resonance imaging (MRI) of the brain was performed, which showed a large well defined lobulated dumb-bell shaped mass of 30x25x22 mm size arising from the pituitary fossa and extending into the suprasellar region. It was hypo-intense on T1 weighted image and hyper-intense on T2 weighted image. The tumor was encasing the optic chiasm [Figure 1]. Cavernous sinus extension was not identified. The tumor showed dense and uniform enhancement with intravenous gadolinium. Radiologically possibility of a malignant pituitary tumor was suggested. A detailed hormonal study was done: T3-10/ 10 HPF] [Figure 2]d. Focal collagen formation was noted [Figure 2]c. The tumor cells were immune-positive for vimentin and CD 34 [Figure 2]e and negative for cytokeratin, epithelial membrane antigen (EMA), bcl2, smooth muscle actin (SMA), chromogranin and synaptophysin. MIB-1 labeling index was high (15-18%) [Figure 2]f.
Post operatively, headache improved but the vision in both eyes deteriorated and the left eye had only perception of light at one meter. Postoperative MRI (15 days post op) revealed residual tumor in the suprasellar region 20x14x11 mm with intra-lesional hemorrhage and perilesional edema [Figure 3]. The patient was taken up for postoperative radiotherapy. He received 60 Gray in 30 fractions over six weeks of external beam radiotherapy by three dimensional conformal techniques with right and left lateral and vertex fields on linear accelerator from October 2007 to November 2007 [Figure 4]. Since then the patient is doing well and showed no recurrence on follow-up.
The term HPC was first used to depict a malignant vascular tumor, arising from pericytes (Zimmerman's' pericytes).  Gradually it became clear that, about 70% (or probably more) of the HPCs are benign (WHO grade II), and the rest behave in malignant fashion. There is variability of the criterions of malignancy. While for some authors tumor size > 5 centimeter, mitotic figures > 4/ 10 HPF, cellular pleomorphism and necrosis, are features indicating malignancy,  in the World Health organization (WHO) book on CNS tumors features of anaplasia (WHO grade III) have been described as mitotic figure of 5/ 10 HPF and/or necrosis along with any two of the following criteria: hemorrhage, moderate to high nuclear atypia and increased cellularity.  According to these criteria our case fits satisfactorily with malignant HPC.
Though it is difficult to differentiate HPCs from its related entities like solitary fibrous tumor (SFT), characteristic histomorphology and immunohistochemical findings are often helpful.  Based on morphological features alone, meningioma, SFT, monophasic synovial sarcoma or infantile fibrosarcomas come into differential diagnosis. Considering the rare location of the present tumor, pituitary adenomas, meningiomas and SFT may be considered as differential diagnosis. In our case, lack of continuity with meningeal surface, whorling and negativity for EMA helped differentiate it from meningioma; absence of stromal hyalinization, variable cellularity, immunonegativity for SMA and bcl2 helped differentiate it from SFT.  Neither was there positivity for neuroendocrine markers to indicate presence of pituitary tissue, nor was there any elevation of serum pituitary hormone levels. Thus the possibility of a dedifferentiated pituitary malignancy was ruled out.
Intracranial HPCs is one of the few primary brain tumors, which metastasize outside the central nervous system.  Local recurrence is not uncommon and has been noted at an average interval of 50 months after application of radiotherapy. Recurrence can be seen as late as nine years (Han et al.  ). In literature, recurrence rates reported at 5, 10 and 15 years were 65%, 76% and 87% respectively. ,, In the index case the patient was being followed up since 11 months with no sign of recurrence.
Reporting HPC in pituitary fossa needs emphasis due to its rarity and the extent to which it may impose difficulty in making a diagnosis either preoperatively or radiologically. Till date, only around five to six cases of HPCs in the sellar region have been reported, with only one example of recurrent angioblastic meningioma in this location [Table 1]. Most of the intracranial HPCs reported till date, are seen arising either from meninges or in and around the ventricles. ,,, While diagnosing an intracranial HPC, special attention should be given to identify the features of malignancy, if any. Our report shows that even malignant HPC can be seen in this site, and in such a case a diligent surgery followed by radiosurgical approach may prove fruitful. 
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