Case Report Iran J Ped Hematol Oncol. 2016, Vol6.No3, 203- 208
Congenital Hemangiopericytoma: A Case Report
Mahdi Shahriari MD 1, Fazl Saleh MD 1, Bita Geramizadeh MD 2, Sezaneh Haghpanah MD 1, Mohammadreza Bordbar MD 1,*
1. Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran 2. Pathology department, Shiraz University of Medical Sciences, Shiraz, Iran Correspondening author: Mohammadreza Bordbar, MD. Hematology Research Center, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran. Email: [email protected] .
Received: 28 July 2015 Accepted: 12 June 201 6
Abstract Hemangiopericytoma is a rare vascular tumor observed mostly in adults. It usually presents with a painless slowly enlarging mass. The infantile type with much rarer occurrence has a different course compared to adults. Very few case reports have been described in the literature with disease onset in the infancy. The first reported case of infantile hemangiopericytoma of limbs from the Middle East was described in the current study. The clinical presentation as well as diagnostic investigations and treatment options are discussed. By considering the existing information in the literature, the clinical course and outcome of this patient with similar reported cases was reported. Key Words : hemangiopericytoma, infantile, treatment.
Introduction Hemangiopericytoma is an uncommon description of some of these cases has tumor that was first described by Stout and been shown in Table 1. To our knowledge, Murray in 1942 (1). It originates from the this is the first case of congenital vascular pericytes which are contractile hemangiopericytoma reported in Iran. cells around the capillary walls. Therefore, it has a wide distribution in both soft tissue Case presentation and skeletal system (2). A three-month-old Iranian infant was Downloaded from ijpho.ssu.ac.ir at 20:45 IRST on Sunday September 26th 2021 This tumor presents as a slowly enlarging investigated who was delivered with good painless mass, and generally considered to APGAR score without any complication. be a benign tumor, though distant General physical examination was metastases can occur. Histologic completely normal, except a firm, violet- examination by immunohistochemical colored swelling on the ulnar aspect of left techniques is the only way to establish the forearm measuring 4 × 2.5 cm (Figure diagnosis (3).Congenital or infantile 1A). The mass was non-tender, non- hemangiopericytoma is encountered even compressible, non-fluctuant, and there was much rarer, and till 2014,only three no bruit on auscultation. No cervical or patients younger than 1 month have been axillary lymphadenopathy, organomegaly reported in Eastern countries (4- or other associated congenital anomalies 6).Detailed report about the number of were detected. Complete cell blood count cases of congenital hemangiopericytoma in (CBC) and chemistry were within normal the world is lacking.Till 1998,only 86 of limits. Color Doppler Ultrasonography these tumors have been reported in the showed a hypoechoic soft tissue mass in literature, which thirty of them were the ulnar aspect of left forearm which located in the extremities(7). A brief consisted of multiple small vessels as well
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as a few large vessels with high blood (Figure 1B). He was discharged on the 3rd flow. With clinical diagnosis of postoperative day and was followed up in hemangiomatous malformation, the soft an outpatient clinic for 3 months before tissue mass was excised under general being referred to pediatric oncologist. anesthesia on the second day of his life. There was no evidence of local recurrence The excised mass was sent for or distant metastasis during the last 3 histopathological examination. months. Immunohistochemistry staining was The patient was treated with 4 courses of positive for CD34 (QBEnd 10), and chemotherapy protocol with Vimentin (Vim 3B4), but negative for Cyclophosphamide 600 mg/m2, BCL2, CD31(JC70A), Cytokeratin Doxorubicin 45 mg/m2 and Dacarbazin (AE1/AE3), Desmin (D33), EMA (E29), 500 mg/m2 repeated every 4 weeks. She is LCA (2B11+PD7/26), MIC2 now in good general health with no (CD99)(12E7), MYOD1 (5.8 A), and S- evidence of tumor recurrence or distant 100, which were consistent with the metastasis. diagnosis of infantile hemangiopericytoma .
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Figure 1. A: The firm, violet- colored swelling on the anterior surface of left forearm with marked surface telangiectasia. B: Sections from soft tissue mass showed a hyper cellular tumor, diffusely and strongly positive for CD34. There are some abnormal looking stag-horn type blood vessels in between.
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Congenital Hemangiopericytoma: A Case Report
Table I: some of the case reports of extremities hemangiopericytoma (18-22)
rative rative
Follow – up 12 during NED follow-up months of Normal leg function and NED for 7 years follow-up. NED in both after cases 2 and 8 follow-up years Respectively. NED after one year follow-up. The patient was not not was The patient to assess available postope remote theas parents outcome the left abruptly hospital. s. s.
Treatment Chemotherapy GTR Chemotherapy for yearone then over surgical resection. GTR in case both GTR
Intervention SEB SEB SEB SEB SEB SEB
, left antecubital , left antecubital , tumor in left in , tumor st nd Location of tumor right inguinal of upper right side arm lower right leg 1 thigh 2 fossa right hand
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s, 2 of s, 2 of
,3 month, male male ,3 month, ,1 month ,female ,female ,1 month st nd 4-day, male 2 Age/sex day-One female 10 days - male 1 6-month, girl Reported 6 case 6 Reported extremities. them in
2006 Country / year. Taipei, Taiwan, Bangladesh, 2011 Osaka, Japan,2000 California, 1984 York,2010 New
NED= no evidence of disease SEB = surgical excision biopsy. GTR = gross-total resection
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Discussion histiocytoma, mesenchymal Hemangiopericytoma is a vascular tumor chondrosarcoma, and leiomyosarcoma (13) mostly observed in adults. The infantile In this case, the final diagnosis was made type which comprises 5-10% of cases is by excisional biopsy. The histological and histologically identical to the adult type, immunohistochemistry features were but is clinically more indolent and is consistent with infantile approached more conservatively (8). The hemangiopericytoma. As the lesion was incidence is higher in boys, and usually histologically malignant, it was decided to occurs in the limbs, pelvis, or head and start chemotherapy for the patient as neck region.Approximately, 30–50% of described above. cases occur in the extremities The majority of these tumors in adults tend (9,10).Infantile type tends to be located to be highly malignant in contrast to the more superficially than the adults type infantile type which are usually benign. (11). In our case, the mass was lying Good response to chemotherapy, subcutaneously, in the left forearm. spontaneous regression, and lack of In an extensive review of the literature recurrence even in case of residual tumor using Pubmed, Embase, Scopus and have been reported in the infantile type Iranmedex databases with (10).However, in children above 1 year “hemangiopericytoma, infantile, old, the disease have a more malignant congenital” as the keywords, only three course similar to adults. It seems that cases were reported in Eastern countries increased age of onset is a risk factor including Japan, Taiwan and India till associated with a more malignant clinical 2014. This is the first reported case of course (14). infantile hemangiopericytoma from Iran The management of infantile and the Middle East. hemangiopericytoma differs from that in The diagnosis can be suspected by adults, although there is no consensus on radiography which shows a soft tissue the best approach mass, with or without calcification. (12).Hemangiopericytoma is unresponsive
Downloaded from ijpho.ssu.ac.ir at 20:45 IRST on Sunday September 26th 2021 Ultrasound determines the size of the to steroid therapy, unlike other vascular lesion, vascular loops, echogenic content malformations.Wide surgical excision with and whether or not a capsule is present. safe margins is the most accepted method Angiography shows the vascular pattern of treatment. Some centers use adjuvant and the presence of feeder vessels. CT and therapy to reduce the risk of local MRI can provide information about the recurrence (15). Whenever total excision is extent of the tumor and help in impossible or in cases of local recurrence preoperative planning. The diagnosis is after surgery, pre- or post-operation confirmed by excisional biopsy and chemotherapy is usually advised (16). immunohistochemical study (12). The role of radiation therapy in infantile The differential diagnosis of congenital hemangiopericytoma is controversial (14). hemangiopericytoma includes all tumors Jha et al. reported some success with which present as soft tissue mass, radiotherapy when there was gross or including: lipoma, hemangioma, and microscopic evidence of tumor remnants lymphangioma. Some tumors have similar after surgical excision, while others have histological features, such as infantile claimed that radiotherapy is ineffective myofibromatosis, synovial sarcoma, (17). This case was treated with wide fibrosarcoma, malignant fibrous surgical excision followed by four courses
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of chemotherapy. His treatment has ended Formos Med Assoc 2006 Mar; 105(3):247 - successfully, and he is in good general 51. health after about 6 months of follow-up. 5.Hamada Y, Takada K, Akehira K, Longer follow up with larger case series is Sakaida N, Okamura A, Hioki K.. needed to decide about the optimal Congenital hemangiopericytoma: report of treatment approach in this age group. a case. Surg Today 2000; 30 (4): 386 –9. Infantile hemangiopericytoma is a rare 6.Anand V, Rajan R, Hazarika P, Padhee vascular tumor that should be considered A.. Infantile hemangiopericytoma of nasal in the differential diagnosis of vascular cavity. Int J Pediatr Otorhinolaryngol malformations in pediatric age group. The 1990; 18 (3):271 –6. diagnosis is mainly confirmed by 7.Hoey SA1, Letts RM, Jimenez C. immunohistochemistry staining. Rarely, it Infantile Hemangiopericytoma of the behaves aggressively with local Musculoskeletal System:Case Report and infiltration, recurrences and even distant Literature Review. J Pediatr Orthop. 1998 metastases. Close follow up and starting May-Jun;18(3):359 -62. chemotherapy in times of subtotal 8.Billings KR, Fu YS, Calcaterra TC, excision, local recurrence, distant Sercarz JA. Hemangiopericytoma of the metastasis or malignant features is head and neck. Am J Otolaryngol 2000; warranted. 21:238–243. 9.Baker DL, Oda D, Myall RW. Intraoral Conflict of interest Infantile Hemangiopericytoma: Literature The authors declare no conflict of interest Review and Addition of a Case. Oral Surg in this case report or in the production of Oral M ed Oral Pathol. 1992 May; this manuscript. 73(5):596-602. 10.Rodriguez-Galindo C, Ramsey K, Acknowledgments Jenkins JJ, Poquette CA, Kaste SC, The authors thank Dr. Ghorbanpoor M. for Merchant TE, et al. Hemangiopericytoma providing laparatomydata. We also wish to in children and infants. Cancer 2000; thank the patient’s parents for their 88(1):198–204. valuable cooperation. 11.Brar R, Kulkarni S, Sheikh S, Jindal S, Brar P. Hemangiopericytoma associated with multiple keratocystic odontogenic
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