Find best premium and Free Joomla templates at GetJoomlaTemplatesFree.com

Infantile hemangiopericytoma: two case reports and literature review

Gamal Al-Saied¹, Rafik Shalaby², Mohamed Al-Saied³, Akram Awadalla*, Ehab Zayed**, Dalal Nemenqani***, Mo’omen Mohamed****
¹²³Departments of Pediatric Surgery,
 *Neurosurgery,
 *Plastic Surgery
***, ****Pathology 
King Abdul Aziz Specialist Hospital, Taif, Kingdom of Saudi Arabia

 

 

Correspondence 

Gamal Al-Saied, MD, FEBPS,
Consultant and Head of Pediatric Surgery Department,
King Abdul Aziz Specialist Hospital, Taif, Saudi Arabia, POB: 10127
Tel: 0096627310998 / Fax: 0096627310801 / Mobile: 00966532919449
Email: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

 

Abstract

Hemangiopericytoma is a rare vascular tumour of infants. Although generally considered to be benign, local recurrence and metastases can occur. Herein, we report on two full term girls, delivered with lumbosacral swelling and left thigh swelling respectively. Complete surgical excision with safety margins was performed for each lesion. Histologic examination of both lesions showed picture of infantile hemangiopericytoma. There is no evidence of local recurrence or distant metastasis during last 20 and 17 months for 1st case and 2nd case respectively. In conclusion most infantile hemangiopericytoma follow a benign course. Rarely these tumours behave aggressively with local infiltration, recurrences and even distant metastases. Careful follow up is therefore essential.

keywords: infantile hemangiopericytoma, vascular tumour

 

 

Introduction

Hemangiopericytoma is an uncommon tumour of infants which originates from the vascular pericytes, which are contractile cells that spiral around capillary walls. Therefore, hemangiopericytoma have a wide distribution in both soft tissue and skeletal system [1]. Although generally considered to be benign, distant metastases can occur [2]. Histologic examination is the only way to establish the diagnosis of hemangiopericytoma. Hemangiopericytoma is unresponsive to steroid therapy, unlike other vascular malformations, resection is the treatment of choice [2]. The prognosis of the tumour behaviour is still not feasible and it has the potential to demonstrate a highly malignant course [1]. Long-term postoperative follow-up is therefore es- sential for the early detection of local recurrence and distant metastases [2]. Herein, we report on the outcome of the initial treatment of two full term girls, delivered on January and March 2009 at King Abdul Aziz Specialist Hospitl, Taif, Saudi Arabia; with lumbosacral swelling and left thigh swelling respectively finally diagnosed as infantile hemangiopericytoma by histologic examination.

Case I

Three day-old, full term girl, weight 3.5kg, delivered with lumbosacral swelling. The baby was generally doing fine, pinkish on room air, active and crying. Moving her legs actively. The swelling was measuring 7x6 cm, heterogeneous consistency, firm in some areas and cys- tic in others, non tender, non reducible, non compress- ible, non fluctuant. Immovable, fixed to the overlying skin and under lying structures. On auscultation there is no bruit. The skin over it was normal. No lymphadenopathy and no viceromegaly. No other associated congenital anomalies ( fig. 1A). CBC and chemistry were within normal limit. Alpha fetoprotein was 47000 ng/ml and b-hCG 3.05 UI/l. Chest X-rays was normal. CT scan with IV contrast of lumbosacral region showed a well defined oval mass with normal density and sign of calcification of fat or cystic components but there was no sign of bone erosion or defect ( fig.1B).

6 JPSS 8 1 2010-6-1 6 JPSS 8 1 2010-6-2
Figure 1A: Three day-old full term baby with lumbosacral swelling, 7x 6 x 7 cm Figure 1B: CT with IV contrast: solid mass in the lumbosacral region without extension to spinal canal (white arrow)

MRI of lumbosacral region revealed a well defined oval mass appears normointense on T w and slight hyperintense on T w images in the right side of lumbo-sacral canal continued with fibrotic band at sacral region without bone erosions or tethering of the cord. Conus medullaris was normal. Initial diagnosis was teratoma versus myelomeningocele. 2 After induction of general endotracheal anaesthesia, the girl is placed in prone position with the pelvis elevated. The skin of lumbosacral region down to mid-thigh is prepared by cleaning with prewarmed povidone iodine. A single dose of broad spectrum antibiotics is given on induction of anaesthesia. A horizontal skin incision is made over the lumbosacral mass. Then, the upper and lower skin flaps are dissected off the mass using bipolar cautery and optical magnification. The mass was dark red in colour with heterogeneous consistency, firm in some areas and cystic in other areas. Then, the mass is dissected off the fascia lumbodorsalis and the fibrotic band tethering the mass to sacral region. There was no bony defect or connection with dura of lumbo-sacral canal or tethering of the cord. The subcutaneous tissue is re-approximated with Vicryl 3/0 and the skin closed with subcuticular sutures of Vicryl 4/0 ( fig.1C,1D). The excised mass is sent to histopathological examination. The postoperative course was uneventful. The girl is discharged on the 3 in outpatient clinic. rd postoperative day and followed up The histological and immunhistochemistry features were consisting with infantile hemangiopericytoma ( fig.1E, 1F, 1G). There was no evidence of local recurrence or distant metastasis during last 20 months.

6 JPSS 8 1 2010-6-3

Figure 1C: Complete surgical excision was done for the lumbosacral mass with safety margins

6 JPSS 8 1 2010-6-4

Figure 1D: 1 month after surgery, the wound is nicely healed (arrow) 

 

6 JPSS 8 1 2010-6-5

Figure 1E: Hematoxylin and Eosin stained section showed plump spindle shaped cells with prominent vascular pattern consisting of thin walled branching vessel (antler configuration), (H&E x200) 

6 JPSS 8 1 2010-6-6

Figure 1F: Reticulin stained section showed dense reticulin network surround blood vessels and individual tumour cells (Strept.A.B.tech. x400) 

6 JPSS 8 1 2010-6-7

Figure 1G: Immunhistochemistry: Branching vascular spaces some of them showed stag horn configuration (arrow). 

Case II

Seven day-old full term girl, weight 4.140 kg. The baby was pinkish on room air, active and crying. Moving her legs actively. Systemic examination was unremarkable. Local examination revealed a firm, violet-coloured swelling on the anterior surface of left thigh, and measuring 5x6 cm. There was marked surface telangiectasia and focal crusting. The surface was easily indented. Non tender to palpation. Non compressible, non fluctuant. Attached to the overlying skin and partially movable over the under lying muscle and fascia. The lesion was non-pulsatile. On auscultation there was no bruit. No lymphadenopathy and no hepatomegaly. No other associated congenital anomalies ( fig. 2A).

CBC and chemistry were within normal limit. CT angiogram of the pelvis and thigh revealed a subcutaneous well defined multilocular cystic lesion in the anterior aspect of the left upper thigh. It shows faint enhancement of its wall and septae with no dominant feeding arteries. There are two main draining veins for this lesion, one on its upper medial aspect draining into the left femoral vein, and the other one extends from the lower medial aspect and drains into the left great saphenous vein ( fig. 2B). The initial diagnosis was hemangiomatous malformation.

6 JPSS 8 1 2010-6-8 6 JPSS 8 1 2010-6-9
Figure 2A: Seven day-old full term girl with firm, violet-coloured swelling on the anterior surface of left thigh, measuring 5 x 6cm Figure 2B: CT angiogram of pelvis and thigh showing a well defined multilocular cystic lesion 

After induction of general endotracheal anaesthe- sia, the girl is placed in supine position. The skin of left thigh is prepared by cleaning with prewarmed povidone iodine. A single dose of broad spectrum antibiotics is given on induction of anaesthesia. An elliptical transverse skin incision is made around the mass. Then, mass is dissected off the underlying quadriceps femoris muscle using bipolar cautery and optical magnification. On cut section the mass was white-grey in colour and soft in consistency. The upper and lower skin edges are undermined with good hemostasis. The subcutaneous tissue is re-approximated with Vicryl 3/0 and the skin closed with subcuticular sutures of Vicryl 4/0 ( fig. 2C, 2D). The excised mass is sent to histopathological examination. The girl is discharged on the 3 postoperative day and followed up in outpatient clinic. The histological and immunhistochemistry features were consisting with infantile hemangiopericytoma ( fig. 2E, 2F, 2G). There was no evidence of local re- rd currence or distant metastasis during last 17 months.

6 JPSS 8 1 2010-6-10

6 JPSS 8 1 2010-6-11
Figure 2C: Complete elliptical surgical excision was performed with safety margins  Figure 2D: After complete surgical excision with safety margins, the biopsy was sent for histopathological examination

6 JPSS 8 1 2010-6-12

Figure 2E: Hematoxylin and Eosin stained section showed plump spindle shaped cells with prominent vascular pattern consisting of thin walled branching vessel (antler configuration). Increased mitotic activity was noted. (H&E x400)

 

6 JPSS 8 1 2010-6-13

Figure 2F: Reticulin stained section showed dense reticulin network surround blood vessels and individual tumour cells (Strept.A.B.tech. x400)

6 JPSS 8 1 2010-6-14 

Figure 2G: Immunhistochemistry result 

Microscopic description of both lesions

Hematoxylin and eosin stained sections revealed plump spindle shaped cells with prominent vascular pattern consisting of thin walled vessels lined by single layer of flattened endothelial cells with gapping vascular spaces, some of them showed stag horn configuration. Increased mitotic activity was noted ( fig. 1E, 2E). Reticulin stained section showed dense reticulin network surrounding blood vessels and individual tumour cells. Focal myxoid area was noted. Isolated tumour nodules within the fibro pseudocapsule of the main mass were seen ( fig. 1F, 2F).

Immunhistochemistry of both lesions (fig. 1G, 2G)

 - positive → Vimentin

 - positive → CD34 (endothelial lining of inter- spersed vascular spaces)

 - positive → S100 8 Focal positive Actin (myopericytes) 

 - negative → Desmin 

- negative → Cytokeratin 

The morphological criteria suggested by Enzinger and Smith were used to distinguish a benign from a malignant tumour. The criteria were: large size (>5cms), increased mitotic rate (4 or more per 10 HPF), high degree of cellularity, immature and pleomophic tumour cells, foci of hemorrhage and necrosis [3]. These histological and immunhistochemistry features of both lesions were consisting with infantile hemangiopericytoma. In accordance with Enzinger and Smith’s criteria both lesions were histologically benign. No evidence of malignancy at the time of examination. 

Discussion

Infantile hemangiopericytoma is a rare vascular tumour in children below one year of age [4]. In 1942, Stout and Murray [5] first described hemangiopericytoma in the lungs as arising from the vascular pericytes of Zimmermann which are contractile cells that spiral around capillary walls and differing from a glomus tumour or a hemangioma but they did not differentiate between infantile and adult forms. The infantile type occurs in 10% of cases [6] and in boys more than in girls [7, 8]. Approximately 30% to 50% are found in the limbs with the remainder either in the head and neck or the trunk. There is seldom discolouration of the lesions in the limb in contrast to those in the head and neck [6]. Infantile hemangiopericytoma tends to be located more superficially than that in adults [9]. In our two cases, both masses were lying subcutaneously, in lumbosacral region without skin discoloration in 1 st cases, the 2 mass was violet-coloured, on the anterior surface of left thigh.

The diagnosis can be aided by plain radiography which shows a soft-tissue mass, with or without calcific stippling. Ultrasound determines the size of the lesion, vascular loops, the echogenic content and whether or not a capsule is present. Angiography shows the vascu- lar pattern and the presence of feeder vessels. CT and MRI can provide information about the extent of the tumour and help in preoperative planning [9]. MRI and CT scan of lumbo-sacral canal (pre-and post IV contrast) of 1 case incorrectly interpreted the lesion as teratoma versus myelomeningocele. st CT angiogram of pelvis and thigh of 2 case, initially diagnosed the lesion as a hemangiomatous malformation. The diagnosis is confirmed by excisional biopsy [9]. The gross appearance of this lesion is usually transparent or white-grey [6]. Feeder vessels may be prominent, and the infantile form tends to be more multilobular than in the adult. Microscopically the pericyte may be round or oval in shape. The tumour tends to be densely cellular, with prominent vascular channels. Mitotic figures are usually easily identified. The tumour cells stain positively with vimentin [6, 9].

In our two cases the final diagnosis was achieved by excisional biopsy. The histological and immunhis- tochemistry features were consisting with infantile hemangiopericytoma. In accordance with Enzinger and Smith’s criteria [3], both lesions were histologically benign. There was no evidence of malignancy at the time of examination. The differential diagnosis of congenital hemangiopericytoma includes all tumours which present as a soft tissue mass, including lipoma, hemangioma, and lymphangioma. Some tumours have similar histological features, such as infantile myofibromatosis, synovial sarcoma, fibrosarcoma, malignant fibrous histiocytoma, mesenchymal chondrosarcoma, and leiomyosarcoma [10-13]. It is therefore important to examine multiple areas of the specimen to confirm the diagnosis [9]. The management of infantile hemangiopericytoma differs from that in adults, but there is no consensus as to the best method [9]. Wide surgical excision with safety margin is the most accepted method of treatment [6, 14, 15]. That was our preferred method in management of both cases. Some centres use adjuvant therapy to reduce the risk of local recurrence [16-20] but the value is difficult to assess because of the rarity of these tumours.

Chemotherapeutic regimens have included the use of vincristine, doxorubicin, actinomycin and cyclophosphamide. Jha et al [21] reported success with radiotherapy when there was gross or microscopic evidence of tumour remaining after excision, and adjuvant radiotherapy was used with success by Borg and Benjamin [22]. Other authors have reported that radiotherapy is ineffective [14, 15, 23]. Metastatic disease has only been reported in three cases of congenital hemangiopericytoma [4,15, 24] although the adult form of the tumour has a rate of distant metastasis of up to 56%, mainly to the lung and skeleton [8,25]. Careful follow up is therefore required. When recurrence does occur, adjunctive chemotherapy has been used successfully [15]. Fortunately, there is no evidence of local recurrence or distant metastasis during last 20 and 17 months in 1 st case and 2 nd case respectively.

Conclusion

Most infantile hemangiopericytoma follow a benign course. Rarely these tumours behave aggressively with local infiltration, recurrences and even distant metastases. Careful follow up is therefore essential. 

 

 

 

References 

  1. Hatzokos I, Gigis I, Iosifidou E, Drakou A, Pournaras J: primary hemangiopericytoma of the tibia. Treatment by en bloc resection and bone transportation. A case report, Acta Orthop. Belg., 2005, 71, 122-127
  2. Bailey PV, Weber TR, Tracy TF, O’Connor DM, Sotelo-Avila C: Congenital hemangiopericytoma: an unusual vascular neoplasm of infancy, Bottom of Form Surgery 1993; 114:936-41
  3. Hahn MJ, Dawnson R. Esterly JA. Joseph DJ. Hemangiopericytoma. An ultrastructural study. Cancer 1973;31:255-61
  4. Kauffman SL, Stout AP. Hemangiopericytoma in children. Cancer 1960;13:695-710
  5. Stout AP, Murray MR. Hemangiopericytoma: a vascular tumor featuring Zimmermann’s pericytes. Ann Surg 1942;116:26-33 
  6. Hoey SA, Letts RM, Jinemez C. Infantile hemangiopericytoma of the musculoskeletal system: case report and literature review. JPediatr Orthop 1998;18:359-62
  7. Baker DL, Oda D, Myall RW. Intraoral infantile hemangiopericytoma: literature review and addition of a case. Oral Surg Oral Med Oral Pathol 1992;73:596-602
  8. Maillet P, Lamesch A, Dawagne MP. Congenital hemangiopericytoma. Chir Pediatr 1985;26:22-5
  9. O’Donnell T. M., Devitt A. T., Kutty S., Fogarty E. E.: Recurrent congenital hemangiopericytoma in a child, J Bone Joint Surg [Br] 2001;83B:269-72
  10. Reynolds FC, Lansche WE. Hemangiopericytoma of the lower extremity: a case report. J Bone Joint Surg [Am] 1958;40-A:921-4
  11. Coffin CM, Dehner LP. Fibroblastic-myofibroblastic tumours in children and adolescents: a clinico-pathologic study of 108 examples in 103 patients. Pediatr Pathol 1991;11:569-88
  12. Isaacs H. Perinatal (congenital and neonatal) neoplasms: a report of 110 cases. Pediatr Pathol 1985;3:165-216
  13. Mentzel T, Calonje E, Nascimento AG, Fletcher CD. Infantile hemangiopericytoma versus infantile myofibromatosis: study of a series suggesting a continuous spectrum of infantile myofibroblastic lesions. Am J Surg Pathol 1994;18:922-30
  14. Atkinson JB, Mahour GH, Isaacs H Jr, Ortega JA. Hemangiopericytoma in infants and children: a report of six patients. Am J Surg 1984;148:372-4
  15. Bailey PV, Weber TR, Tracy TF, O’Connor DM, Sotelo-Avila C. Congenital hemangiopericytoma: an unusual vascular neoplasm of infancy. Surgery 1993;114:936-41
  16. Craven JP, Quigley TM, Bolen JW, Raker EJ. Current management and clinical outcome of hemangiopericytomas. Am J Surg 1992;163:490-3.
  17. Battifora H. Hemangiopericytoma: ultrastructural study of five cases. Cancer 1973;31:1418-32
  18. Mira JG, Chu FC, Fortner JG. The role of radiotherapy in the management of malignant hemangiopericytoma: a report of eleven new cases and a review of the literature. Cancer 1977;39:1254-9
  19. Variend S, Bax M, van Gorp J. Are infantile myofibromatosis, congenital fibrosarcoma, and congenital hemangiopericytoma histogenetically related? Histopathology 1995;26:57-62
  20. Wong PP, Yagoda A. Chemotherapy of malignant hemangiopericytoma. Cancer 1978;41:1256-60
  21. Jha N, McNeese M, Barkley HT, Kong J. Does radiotherapy have a role in hemangiopericytoma management? Report of 14 new cases and a review of the literature. Int J Radiat Oncol Biol Phys 1987;13:1399402
  22. Borg MF, Benjamin CS. A 20 year review of hemangiopericytoma in Auckland, New Zealand. Clin Oncol (R Coll Radiol)1994;6:371-6
  23. Seibert JJ, Seibert RW, Weisenburger DS, Allsbrook W. Multiple congenital hemangiopericytomas of the head and neck. Laryngoscope 1978;88:1006-12
  24. Morgan A, Evbuomwan I. Congenital hemangiopericytoma of the face with early distant metastasis. J R Coll Surg Edinb 1983;28:123-5
  25. Salloum E, Flamant F, Caillaud JM, et al.: Diagnostic and therapeutic problems of soft tissue tumours other than rhabdomyosarcoma in infants under 1 year of age: a clinicopathological study of 34 cases treated at the Institut Gustave-Roussy. Med Pediatr Oncol 1990;18:37-43