Indian Journal of Pathology and Microbiology
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Year : 2008  |  Volume : 51  |  Issue : 1  |  Page : 39-41
Malignant hemangiopericytoma in the gluteal region: A rare entity

1 Department of Pathology, JN Medical College, Aligarh Muslim University, Aligarh, UP, India
2 Department of Orthopedics, JN Medical College, Aligarh Muslim University, Aligarh, UP, India

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We present here a case of malignant hemangiopericytoma in a 40-year-old female who presented with a history of slowly growing mass in left gluteal region for about 1.5 years. She was evaluated and a mass of 10 × 7.5 × 5.5 cm 3 was found on computed tomography. The tumor was resected and was sent for histopathological evaluation. Grossly the tumor was encapsulated and on cut section it was nodular. Microscopy showed spindle cells forming cords and sheets separated by thin delicate stroma consisting largely of thin-walled vessels revealed by reticulin stain. Pleomorphism and mitotic figures were seen. It was diagnosed as a case of malignant hemangiopericytoma of gluteal region. The patient underwent radiotherapy and did apparently well. She is on regular follow-up because long-term follow-up is essential in all cases as recurrence can occur several years after treatment.

Keywords: Malignant hemangiopericytoma, gluteal region, soft tissue tumor

How to cite this article:
Sherwani R K, Rahman K, Akhtar K, Siddiqui F A, Varshney B K, Sherwani M. Malignant hemangiopericytoma in the gluteal region: A rare entity. Indian J Pathol Microbiol 2008;51:39-41

How to cite this URL:
Sherwani R K, Rahman K, Akhtar K, Siddiqui F A, Varshney B K, Sherwani M. Malignant hemangiopericytoma in the gluteal region: A rare entity. Indian J Pathol Microbiol [serial online] 2008 [cited 2022 May 16];51:39-41. Available from: https://www.ijpmonline.org/text.asp?2008/51/1/39/40391

   Introduction Top

Hemangiopericytoma is a rare soft tissue tumor arising from the pericytes of Zimmerman. [1] It may occur anywhere but is most frequently encountered in lower extremities or retroperitoneum. Although it is a disease of middle-aged patients, children and infants could also be affected. [2] Most of the patients present with slowly increasing painless mass that gradually increases in size. The computed tomography (CT) and magnetic resonance imaging (MRI) findings are nonspecific which consist of radio-opaque mass displacing the adjacent organs. [3] Histologically, they are characterized by tightly packed cells around the thin-walled, endothelium-lined vascular channels ranging from capillary-sized vessels to large gaping sinusoidal spaces. [4] They are highly malignant tumors and typically metastasize to lungs, bone, liver, and lymph nodes (Rubin & Farber). Reported metastatic rate ranges from 11.7 to 56.5%. [5]

They vary in size from one centimeter to several centimeters that may be circumscribed or thinly encapsulated.

   Case History Top

We present here a case of a 40-year-old female patient who presented to the orthopedic department with 1.5 years history of swelling over left gluteal region and associated complaint of pain for about 7 months. The swelling was small to start with, but it gradually increased in size with time.

On physical examination, diffuse swelling was noted on the left gluteal region. The margins were not well defined and the temperature above the swelling was slightly raised.

X-ray of the region showed no bony abnormality. The CT findings were suggestive of a well-defined soft tissue lesion with central hypodense area (necrosis). It appeared to arise from left gluteal muscle with invasion into the greater sciatic foramen with sclerosis of the lower aspect of the iliac bone. A diagnosis of malignant soft tissue tumor was given. MRI was suggestive of heterogeneous soft tissue tumor mass lesion involving the left gluteus maximus muscle with ?infiltration into the adjacent iliac blade at sacro-iliac joint likely representing soft tissue sarcoma.

The provisional diagnosis of soft tissue sarcoma of left gluteal region was given. The tumor mass was resected and sent for histopathology.

Pathological findings

Aspiration smear was hemorrhagic and composed of single cells and clusters of spindle cells with elongated nuclei having moderate degree of nuclear pleomorphism. Mitoses were absent. The impression of low-grade sarcoma of fibroblastic or fibrohistiocytic origin was given.

Grossly the tumor was irregular and encapsulated measuring 7 x 5 x 4.5 cm 3 . It was brown in color with attached fat. The surface was congested and was nodular. The cut surface showed nodularity and congestion with some specks of hemorrhage (vascular channels). The orientation of the tumor was not possible.

On histopathology (hematoxylin and eosin) round to spindle-shaped cells forming cords and masses separated by a delicate stroma consisting largely of thin-walled blood vessels, some containing blood but others collapsed and inconspicuous were noted [Figure - 1]. Prominent cellularity and staghorn configuration of dilated vessels was noted [Figure - 2]. There was an increased pleomorphism with high mitotic activity in some areas (>4/10 HPF). Large areas of necrosis and hemorrhage were also in evidence. The reticulin stain showed the tumor cells to be located peripheral to periendothelial ring of reticulin [Figure - 3].

   Discussion Top

Malignant hemangiopericytoma is a rare soft tissue tumor of perivascular origin arising from the pericytes, which are special cell types first described by Zimmerman. [6] But in some studies the actin subset pattern of pericytes differed from that of hemangiopericytoma, indicating a possible nonpericytic origin of these tumors. [7] Anatomically it is common in the lower extremities, followed by retroperitoneum and other sites. [2] In another series, the leading site was that of thigh and inguinal region. [8]

In spite of a number of literatures, the histological diagnosis causes a considerable problem and in many cases it remains a diagnosis of exclusion.

The findings in our case were similar to those of the others. On the basis of the clinical findings the provisional diagnosis of soft tissue sarcoma was put forward. The aspiration showed hemorrhagic smear and the diagnosis of low-grade sarcoma of fibroblastic or fibrohistiocytic origin was given. The tumor was excised and the specimen was sent for histopathological evaluation. Grossly the tumor was encapsulated and the surface was hemorrhagic. On histology, based on the nuclear pleomorphism, necrosis, and mitotic figures, the final diagnosis of malignant hemangiopericytoma was given.

The behavior of this tumor is based on the presence of hemorrhage, necrosis, and the mitotic figures. In benign lesion, there are fewer than 2-3 mitotic figures per 10 high power fields. In contrast to it, the malignancy is recognizable by the presence of a higher mitotic rate (4 or more per 10 HPF), presence of greater degree of cellurarity, immature and pleomorphic tumor cells, and foci of hemorrhage and necrosis. [4]

In another study, malignant nature was labeled based on the presence of mild degree of cellular anaplasia and 1 mitotic figure/10 HPF or moderate degree of anaplasia and 1 mitotic figure/20 HPF. [8] Taking any of the above studies into account, this case was frankly of the malignant variety.

Immunohistochemically, the use of factor VIII-related antigens could differentiate between endothelial cells and surrounding pericytes by beautifully staining the endothelial cells to a variable degree and leaving the pericytes unstained. In addition to it pericytes are actin and vimentin positive but do not stain for desmin and myoglobin. [9]

There is a high rate of metastasis and recurrence in malignant hemangiopericytoma. But fortunately in this case there was no matastatic focus at the time of presentation.

As with any other soft tissue tumor, radiotherapy or chemotherapy is required for the treatment of malignant hemangiopericytoma. [10] The patient was referred to the radiotherapy department for the radiation. She received 30 cycles of therapy and presently doing fine, showing the usefulness of early management of the tumor. She is on regular follow-up as the metastasis is common in this tumor even several years after presentation.

   References Top

1.Stout AP, Murray MR. Hemangiopericytoma. A vascular tumor featuring Zimmerman's pericytes. Ann surg 1942;116:26.  Back to cited text no. 1    
2.Enzinger FM. Smith BH. Hemangiopericytoma. An analysis of 106 cases. Hum Pathol 1976;7:61.  Back to cited text no. 2    
3.De Villierws DR. Farman J, Campbell JAH: Pelvic hemangiopericytoma: Preoperative arteriographic demonstration. Clin Radiol 1967;18:318.  Back to cited text no. 3    
4.Enzinger FM, Weiss SW. Soft tissue tumours, 3rd ed. St. Louis: CV Mosby; 1995. p. 713-29.  Back to cited text no. 4    
5.O'Brien P, Brasfield RD. Haemangioperictytoma. Cancer 1965;18:249.  Back to cited text no. 5    
6.Zimmerman KW. Der feinere bau der Blutcapillaren. Z Anat Entwicklungsg 1923;68:29.  Back to cited text no. 6    
7.Schürch W, Skalli O, Lagace R, Seemayer TA, Giulio G. Intermediate filament protein and actin isoforms as a marker of soft tissue tumor differentiation and origin III. Hemangiopericytoma and glomus tumor. Am J Pathol 1990;136:771.  Back to cited text no. 7    
8.McMaster M, Soule E, Ivins J. Hemangiopericytoma-a clinicopathological study and long term follow up of 60 patients. Cancer 1975;36:2232.  Back to cited text no. 8    
9.Mittal KR, Gerald W, True LD. Hemangiopericytoma of breast. Report of a case with ultrastructural and immunohistochemical staining. Hum Pathol 1986;17:1181.  Back to cited text no. 9    
10.Dube VE, Parlson JF. Metastatic hemangiopericytoma cured by radiotherapy. J Bone joint Surg 1974;56A:833.  Back to cited text no. 10    

Correspondence Address:
R K Sherwani
Department of Pathology, JN Medical College, Aligarh Muslim University, Aligarh, UP
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.40391

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  [Figure - 1], [Figure - 2], [Figure - 3]


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