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ABDOMINAL RADIOLOGY Table of Contents   
Year : 2006  |  Volume : 16  |  Issue : 3  |  Page : 369-370
Hemangiopericytoma of the retroperitoneum


Department of Radiodiagnosis, Dyanand Medical College & Hospital, Ludhiana - 141001, India

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Keywords: Hemangiopericytoma, retroperstoneum

How to cite this article:
Kalia V, Saggar K, Ahluwalia A, Sandhu P, Vibhuti. Hemangiopericytoma of the retroperitoneum. Indian J Radiol Imaging 2006;16:369-70

How to cite this URL:
Kalia V, Saggar K, Ahluwalia A, Sandhu P, Vibhuti. Hemangiopericytoma of the retroperitoneum. Indian J Radiol Imaging [serial online] 2006 [cited 2020 Feb 26];16:369-70. Available from: http://www.ijri.org/text.asp?2006/16/3/369/29020
Hemangiopericytomas are rare tumors, representing approximately 1% of all vascular neoplasms. They are believed to originate from the pericytes of zimmerman which surround the capillaries[1]. These tumors occur at all ages with a predilection to early middle age and with equal sex distribution. They present as large, well encapsulated retroperitoneal masses displacing the gastrointestinal tract. The neoplasm must be considered in the differential diagnosis of pelvic and retroperitoneal soft tissue mass despite its relative rarity as compared with other retroperitoneal masses of mesenchymal origin (liposarcoma, malignant fibrous histocytioma or leiomyosarcoma)[2].

A 64 years old female presented with abdominal pain and distension of two months duration. No other relevant history was there. On per abdomen examination, there was a large mass palpable in the umblical, hypogastric and iliac regions. The mass was non tender, firm and dull on percussion. There was no ascites and bowel sounds were present. Routine lab tests and chest radiograph were normal. Ultrasonography showed a large heterogenous predominantly cystic mass with multiple septations in the pelvis reaching till epigastrium [Figure - 1]. Uterus was normal, ovaries could not be visualized. Rest of the abdomen was normal. Computed tomography revealed a large heterogenous mixed density mass with enhancing septae in the pelvis extending superiorly till the epigastric region. No areas of fat attenuation were identified. Ovaries were not separately visualized [Figure - 2]. On Laparotomy, a large retroperitoneal cystic multiloculated mass was excised with hemorrhagic fluid in it. Both ovaries and uterus were separately seen. Histopathology showed presence of a cellular tumor with tumor cells arranged in diffuse sheets and present around vascular channels. The individual tumor cells showed round to oval vesicular nuclei, inconspicuous nucleoli and exhibited mild nuclear pleomorphism. Areas of hyaline, cystic and myxoid change were seen with foci of necrosis. Occasional mitotic figures were also seen suggestive of malignant mesenchymal tumor of vascular origin i.e. hemangiopericytoma [Figure - 3]. Immunohistochemistry showed reactivity for CD 34 confirming it to be hemangiopericytoma.

Hemangiopericytomas are rare tumors, representing approximately 1% of all vascular neoplasms[1]. They present initially as masses of varying size, ranging from 1-20 cms in diameter, which usually enlarge and are painless. Other symptoms are due to pressure effect related to the size and site of origin of the tumor. Both sexes are equally affected [1],[2],[3]. The retroperitoneum is the second most common site of hemangiopericytoma after the upper and lower extremities especially the thigh [3],[4].They can also arise from the central nervous system, bones, from paranasal sinuses, omentum and skin or there can be intravascular or lipomatous hemangiopericytoma. Central nervous system tumors can arise from the meninges or can be intraventricular and has to be differentiated from meningiomas. This discrimination can be done with magnetic resonance spectroscopy which shows large peak at 3.56 parts per million in hemangiopericytoma due to higher levels of myoinositol[5]. Sinonasal hemangiopericytomas are subdivided into soft tissue type and true hemangiopericytomas and they should be differentiated from one another as soft tissue type are highly aggressive whereas true hemangiopericytomas show localized benign behaviour[6]. In retroperitoneum, these tumor occur less frequently in pelvic retroperitoneal space than in abdominal retroperitoneum [3]. Pathological diagnosis of this tumor is based on the presence of large branched, sinusoidal, vascular channels which can be elicited radiologically and by angiography. There is no definite pathological method (i.e. grading system) of determining the potential for malignancy or benignancy. However, prominent mitoses (greater than four per high power field), foci of necrosis and increased cellularity are all suggestive of malignancy. The one definite sign is reoccurrence and development of metastatses[3].

Hemangiopericytomas can also be associated with paraneoplastic hypoglycemia, hypertension or gynecomastia due to production of various growth factors by the tumor like insulin growth factor I and II and renin[7],[8].

Plain radiograph features include a well demarcated mass with occasional calcification or there can be osseous changes. Osseous changes can be primary or secondary. Primary tumor of the bone is rare and can arise from ilium, vertebrae, ribs, ischium, clavicle, femur, fibula, skull, mandible and periosteum. Secondary changes can be due to localized pressure erosion or bony destruction adjacent to the soft tissue tumor or there can be enlargement of the intervertebral foramina[2]. Barium studies may also have a significant role in the preoperative management if bowel invasion is suspected[3].

Sonographic findings include well defined predominantly cystic masses, mixed solid and cystic mass and solid mass with or without calcification. Sonography may define the limits of tumor, give information about the internal structure and offer a way of following response to therapy. However, the sonographic features are varied and the often cystic nature of these tumors may be misleading[9]. Color Doppler sonography can demonstrate intratumoral arteriovenous shunting and may be used to help avoid profuse bleeding when performing a preoperative biopsy[10].

On Computed Tomography, the tumor tends to be bulky displacing the part of the gastriointestinal tract, large, well encapsulated, occurring in the abdominal and pelvic retroperitoneal space. On contrast enhanced scan, the tumor demonstrates hypervascularity which is the most definitive radiological feature and differentiate it from other more common retroperitoneal neoplasms like liposarcoma, leiomyosarcoma and malignant fibrous histiocytoma. Computed Tomography and angiography plays an important role by demonstrating its vascular nature, by revealing the exact source of its blood supply and by demonstrating its size and relationship to adjacent viscera and in planning for preoperative management like embolizaton. However, radiological findings cannot differentiate between malignant and benign tumor[2],[3].

Surgical removal is the treatment for this tumor. In general survival rates are poor, with less than 50% of patients being alive and well at five years. Both local reoccurrence and distal metastases occur, commonly by hematogenous and to a lesser extent by lymphatic route. The metastasis affect predominantly lung, bone and liver[2],[3],[9].

This neoplasm must be considered in the differential diagnosis of pelvic and retroperitoneal soft tissue masses, despite its relative rarity compared with other more commonly occurring retroperitoneal tumors of mesenchymal origin.

 
   References Top

1.Ayella RJ. Hemangiopericytoma: A case report with arteriographic findings. Radiology 1970; 97: 611-612.  Back to cited text no. 1  [PUBMED]  
2.Issa Yaghmai. Angiographic manifestations of soft tissue and osseous hemangiopericytomas. Radiology 1978; 126: 653-659.  Back to cited text no. 2    
3.Stanford M, Alan JD, Joseph N. Retroperitoneal and Pelvic hemangiopericytomas: clinical, radiological and pathologic correlation. Radiology 1988; 168: 13-17.  Back to cited text no. 3    
4.Stout AP. Tumors featuring pericytes: glomus tumor and hemangiopericytoma. Lab Invest 1956; 5: 217-223.  Back to cited text no. 4  [PUBMED]  
5.Barba I, Moreno A, Martinez PI, Tata AR, Cabanas ME, Baquero M et al. Magnetic resonance spectroscopy of brain hemangio-pericytomas: high myoinositol concentrations and discrimination from meningiomas. J. Neurosurg 2001; 94(1): 55-60.  Back to cited text no. 5    
6.Watanabe K, Saito A, Suzuki M, Yamanobe S, Suzuki T. True hemangiopericytoma of the nasal cavity. Arch Pathol Lab Med 2001; 125(5): 686-90.  Back to cited text no. 6    
7.Kruskal JB, Kane RA. Paraneoplastic hypoglycemia associated with hepatic hemangiopericytoma. J. Ultrasound Med 2002; 21(8): 927-932.  Back to cited text no. 7    
8.Derakhshani P, Neubauer S, Bram M, Heidureich A, Engelmann U. Giant retroperitoneal hemangiopericytoma with paraneoplastic gynecomastia. Urologia internationalis 1999; 62: 223-225.  Back to cited text no. 8    
9.Grant EG, Gronvall S, Sarosi TE, Borts FT, Holm HH, Schellinger D. Sonographic findings in four cases of hemangiopericytoma. Correlation with computed tomographic, angiographic and pathologic findings. Radiology 1982; 142: 447-451.  Back to cited text no. 9    
10.Juan C, Huang G, Chin S, Hsuch C, Wu C, Hsiao H et al. Color and duplex doppler sonography of hemangiopericytoma. J Clin ultrasound 2001; 29(1): 51-55.  Back to cited text no. 10    

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Correspondence Address:
V Kalia
464-sec 8/Ii, Urban Estate, Karnal, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.29020

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



 

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