Abstract Introduction A hemangiopericytoma is a rare, soft-tissue tumor of vascular origin derived from a pericyte of Zimmerman, which is a modified smooth muscle cell that surrounds the small blood vessels. Hemangiopericytomas can occur wherever there are vascular capillaries. However, there are no previous reports of a hemangiopericytoma in the sacrococcygeal space. Case presentation We describe the first reported case of a hemangiopericytoma found in the sacrococcygeal space. A 47-year-old Japanese woman presented with a palpable tumor on the left side of her anus. Preoperative imaging indicated that the tumor was in the sacrococcygeal space without invasion of other organs. A complete resection was performed via a parasacral incision. The histological and immunohistochemical staining patterns supported the diagnosis of a hemangiopericytoma. Conclusion A complete resection without piecemeal excision is the best way to treat a hemangiopericytoma. Recognizing the presence of a hemangiopericytoma in the sacrococcygeal space requires appropriate surgery.</p

C Gengler

E Cristi

FM Enzinger

FR Spitz

G Vennarecci

Hiroki Yamaue

J Aoki

Junji Ieda

Katsunari Takifuji

Kenji Matsuda

M Fukunaga

ML del Rosario

N Jha

S Hart

Shozo Yokoyama

SM Goldman

Takashi Watanabe

Toshiji Tominaga

Tsukasa Hotta

Yoshimasa Oku

Yuji Kitahata

English

PubMed

Abstract Introduction A hemangiopericytoma is a rare, soft-tissue tumor of vascular origin derived from a pericyte of Zimmerman, which is a modified smooth muscle cell that surrounds the small blood vessels. Hemangiopericytomas can occur wherever there are vascular capillaries. However, there are no previous reports of a hemangiopericytoma in the sacrococcygeal space. Case presentation We describe the first reported case of a hemangiopericytoma found in the sacrococcygeal space. A 47-year-old Japanese woman presented with a palpable tumor on the left side of her anus. Preoperative imaging indicated that the tumor was in the sacrococcygeal space without invasion of other organs. A complete resection was performed via a parasacral incision. The histological and immunohistochemical staining patterns supported the diagnosis of a hemangiopericytoma. Conclusion A complete resection without piecemeal excision is the best way to treat a hemangiopericytoma. Recognizing the presence of a hemangiopericytoma in the sacrococcygeal space requires appropriate surgery.</p

Watanabe Takashi

Oku Yoshimasa

Tominaga Toshiji

Matsuda Kenji

Hotta Tsukasa

Takifuji Katsunari

Yokoyama Shozo

Kitahata Yuji

Ieda Junji

Yamaue Hiroki

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Journal of Medical Case Reports

Hemangiopericytoma in the sacrococcygeal space: a case report

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CASE REPORT Open Access
Hemangiopericytoma in the sacrococcygeal
space: a case report
Yuji Kitahata, Shozo Yokoyama*, Katsunari Takifuji, Tsukasa Hotta, Kenji Matsuda, Toshiji Tominaga, Yoshimasa Oku,
Takashi Watanabe, Junji Ieda, Hiroki Yamaue
Abstract
Introduction: A hemangiopericytoma is a rare, soft-tissue tumor of vascular origin derived from a pericyte of
Zimmerman, which is a modified smooth muscle cell that surrounds the small blood vessels.
Hemangiopericytomas can occur wherever there are vascular capillaries. However, there are no previous reports of
a hemangiopericytoma in the sacrococcygeal space.
Case presentation: We describe the first reported case of a hemangiopericytoma found in the sacrococcygeal
space. A 47-year-old Japanese woman presented with a palpable tumor on the left side of her anus. Preoperative
imaging indicated that the tumor was in the sacrococcygeal space without invasion of other organs. A complete
resection was performed via a parasacral incision. The histological and immunohistochemical staining patterns
supported the diagnosis of a hemangiopericytoma.
Conclusion: A complete resection without piecemeal excision is the best way to treat a hemangiopericytoma.
Recognizing the presence of a hemangiopericytoma in the sacrococcygeal space requires appropriate surgery.
Introduction
A hemangiopericytoma is a rare, soft-tissue tumor of
vascular origin derived from a pericyte of Zimmerman,
which is a modified smooth muscle cell that surrounds
the small blood vessels. This type of tumor was first
described by Stout and Murray in 1942 [1]. It represents
approximately 5% of all sarcomatous tumors, and can
occur anywhere, but more usually in the musculature of
the extremities, retroperitoneum, pelvis (uterus, ovary,
and urinary bladder), head, neck and lungs [2]. There
are no reports of a hemangiopericytoma in the sacrococ-
cygeal space. Since the recommended treatment for a
hemangiopericytoma is wide excision, due to high local
recurrence [3,4], it is important to recognize the pre-
sence of this malignant tumor in the area surrounding
the anus, where various tumors occur. We describe a
rare case of a hemangiopericytoma in the sacrococcygeal
space.
Case presentation
A 47-year-old Japanese woman presented with a palp-
able tumor on the left side of her anus. The tumor was
elastic and hard and had a smooth surface. She had no
pain or tenderness associated with the lesion, and no
other clinical symptoms. Computed tomography (CT)
scan showed a mass in the sacrococcygeal space with a
smooth surface and no invasion to the rectum (Figure
1A). Magnetic resonance imaging (MRI) showed that
the outer layer was heterogeneous with high intensity,
and that the central layer had an extremely high inten-
sity in T2-weighted images, indicating that the inside of
the mass had a rich blood flow (Figure 1B). Rectoscopy
revealed that the mucosal surface was intact, and an
endoscopic ultrasonography (EUS) demonstrated that
the tumor was not derived from the rectum. Fluoro-
deoxyglucose positron emission tomography (FDG-PET)
showed that the maximum standardized uptake value
(SUV) at the area was 2.64. The expected SUV is 1.80
to 1.42 in benign soft tissue masses, and 4.20 to 3.16 in
malignant soft tissue masses [5].
The pre-operative images indicated that the tumor
was not derived from the rectum. Because of its vascu-
larity, the pre-operative diagnosis was a soft-tissue
* Correspondence: yokoyama@wakayama-med.ac.jp
Second Department of Surgery, Wakayama Medical University, School of
Medicine, Kimiidera, Wakayama, 641-8510, Japan
Kitahata et al. Journal of Medical Case Reports 2010, 4:8
http://www.jmedicalcasereports.com/content/4/1/8 JOURNAL OF MEDICAL
CASE REPORTS
© 2010 Kitahata et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons
Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in
any medium, provided the original work is properly cited.
tumor such as a solitary fibrous tumor, fibrous histiocy-
toma, synovial sarcoma, mesenchymal chondrosarcoma,
or hemangiosarcoma. A biopsy was avoided due to the
risk of needle track seeding. The patient underwent a
tumorectomy via parasacral incision, without a rectect-
omy, because pre-operative examinations including a
CT and EUS revealed that the rectum was intact. The
tumor was completely removed (Figure 2A). The excised
tumor was 80 × 75 × 65 mm in diameter with a capsule.
Its cut surface was mostly grayish-white and partially
reddish (Figure 2B). Histopathological features of the
hematoxylin and eosin staining revealed that the tumor
contained spindle-shaped cells surrounding the endothe-
lial-lined vascular spaces, which is consistent with the
histology of hemangiopericytoma (Figure 3A). Argyro-
phil fibers were seen on silver impregnation surrounding
the tumor cells.
We performed an immunohistological analysis to
obtain a diagnosis of the type of mesenchymal tumor.
The mesenchymal tumor cells in our patient stained
positive for CD34 (Figure 3B) and vimentin, and nega-
tive for Bcl-2 (Figure 3C), CD99, c-kit, factor VIII, des-
min, alpha-smooth muscle actin, S-100 protein,
epithelial membrane antigen, and keratin. The mitotic
rate was 1 per 10 high-power fields. No necrotic lesion
was observed in our patient’s tumor. The tumor was
pathologically diagnosed as a hemangiopericytoma.
Discussion
Little has been published about hemangiopericytoma, a
rare, soft-tissue tumor. It can occur anywhere vascular
capillaries are found. The tumors most commonly
occurs in the musculature of the extremities, retroperi-
toneum, pelvis (uterus, ovary, and urinary bladder),
head, neck and lungs [3,4].
The pathological diagnosis of a hemangiopericytoma,
in comparison to other mesenchymal tumors such as
solitary fibrous tumors, can be difficult [6]. Bcl-2 and
CD99 immunohistochemistry were used to distinguish a
hemangiopericytoma from a solitary fibrous tumor
because a solitary fibrous tumor is positive for Bcl-2 [7]
and CD99 [8]. We diagnosed a hemangiopericytoma fol-
lowing an examination of the structural features of the
mass. Spindle-shaped cells surrounding the endothelial-
lined vascular spaces were observed by hematoxylin and
eosin staining, and the mass was positive for CD34 and
vimentin and negative for Bcl-2, CD99, c-kit, factor VIII,
desmin, alpha-smooth muscle actin, S-100 protein,
epithelial membrane antigen, and keratin upon immuno-
histochemical analysis. Making a differential diagnosis
between a solitary fibrous tumor and a hemangiopericy-
toma is particularly difficult and controversial [9], and a
novel molecular marker for distinguishing between the
two close entities is required.
Radiotherapy and chemotherapy are not generally
effective for the treatment of a hemangiopericytoma
[10]. Some have advocated the use of adjuvant
Figure 1 (A) Computed tomography scan showing a large mass in the sacrococcygeal space. (B) Magnetic resonance imaging scan
showing heterogeneous high intensity in the outer layer and extremely high intensity in the central layer in T2-weighted image.
Kitahata et al. Journal of Medical Case Reports 2010, 4:8
http://www.jmedicalcasereports.com/content/4/1/8
Page 2 of 4
radiotherapy in response to the locally aggressive nature
of hemangiopericytomas but, due to tumor radioresis-
tance, no differences in local disease control were
observed between treatment with and without adjuvant
radiotherapy [11]. Spitz et al. reported that hemangio-
pericytomas showed a poor response to chemotherapy.
They treated six patients with pre-operative chemother-
apy, and only one of them responded to anthracycline-
based chemotherapy [3]. Therefore, complete surgical
resection is the only effective therapy for
hemangiopericytoma.
Spitz et al. also reported that 5-year and 10-year survi-
val rates of patients with a hemangiopericytoma were
71% and 54%, respectively. In addition, they noted that
the survival rate differed between a curative and a non-
curative resection. The 5-year survival rate in patients
treated with curative resection and non-curative resec-
tion was 79% and 50%, respectively [3]. These data indi-
cate that a complete resection is necessary to improve
patients’ survival rates. Since many benign and
malignant diseases occur in the area surrounding the
anus, recognizing the presence of this malignant tumor
in the sacrococcygeal space is important in order for an
anorectal surgeon to avoid inappropriate surgery such as
piecemeal excision.
Conclusion
This report presented a rare case of a hemangiopericy-
toma in the sacrococcygeal space. Many diseases are
associated with anal lesions, therefore a thorough differ-
ential diagnosis and complete resection without piece-
meal excision must always be performed in the
management of this type of malignant tumor.
Consent
Written informed consent was obtained from the patient
for publication of this case report and any accompany-
ing images. A copy of the written consent is available
for review by the Editor-in-Chief of this journal.
Figure 2 (A) This is a macroscopic image of the 80 × 75 × 65 mm-sized tumor in the sacrococcygeal space. (B) This image shows the
excised tumor with a capsule, with its cut surface mostly grayish white and partially reddish.
Figure 3 (A) Hematoxylin and eosin staining revealing spindle-shaped cells surrounding the endothelial-lined vascular spaces. (B)
Immunohistochemistry demonstrating CD34 positive tumor cells. (C) Immunohistochemistry demonstrating Bcl-2 negative tumor cells.
Kitahata et al. Journal of Medical Case Reports 2010, 4:8
http://www.jmedicalcasereports.com/content/4/1/8
Page 3 of 4
Abbreviations
CT: computed tomography; FDG-PET: fluorodeoxyglucose positron emission
tomography; MRI: magnetic resonance imaging; SUV: standardized uptake
value.
Authors’ contributions
YK initiated the concept of the case report, performed the literature search,
and wrote the manuscript. SY performed the pathological investigations,
helped in the literature search, and approved the final write up. KT
performed the surgery. TH and KM helped revise the manuscript. TT assisted
in the surgery and also helped draft the manuscript. YO and TW helped
revise the manuscript. JI helped revise the article. HY contributed to the
clinical management of the patient and helped revise the manuscript. All
authors read and approved the final manuscript.
Competing interests
The authors declare that they have no competing interests.
Received: 19 September 2009
Accepted: 14 January 2010 Published: 14 January 2010
References
1. Hart S, Sisely L: Haemangiopericytoma of the presacral space. Br J Surg
1973, 60:583-584.
2. Vennarecci G, Boschetto A, Esposito A, Giovannelli L, Buscaglia F, Corazza V,
Santoro R, Mancini P, Lorusso R, Marino M, Ettorre GM: Malignant
haemangiopericytoma of the mesorectum. Chir Ital 2004, 56:865-868.
3. Spitz FR, Bouvet M, Pisters PW, Pollock RE, Feig BW: Hemangiopericytoma:
a 20-year single-institution experience. Ann Surg Oncol 1998, 5:350-355.
4. Goldman SM, Davidson AJ, Neal J: Retroperitoneal and pelvic
hemangiopericytomas: clinical, radiologic, and pathologic correlation.
Radiology 1988, 168:13-17.
5. Aoki J, Watanabe H, Shinozaki T, Takagishi K, Tokunaga M, Koyama Y,
Sato N, Endo K: FDG-PET for preoperative differential diagnosis between
benign and malignant soft tissue masses. Skeletal Radiol 2003, 32:133-138.
6. Enzinger FM, Smith BH: Hemangiopericytoma: an analysis of 106 cases.
Hum Pathol 1976, 7:61-82.
7. Fukunaga M, Naganuma H, Ushigome S, Endo Y, Ishikawa E: Malignant
solitary fibrous tumour of the peritoneum. Histopathol 1996, 28:463-466.
8. Cristi E, Perrone G, Battista C, Benedetti-Panici P, Rabitti C: A rare case of
solitary fibrous tumour of the presacral space: morphological and
immunohistochemical features. In Vivo 2005, 19:777-780.
9. Gengler C, Guillou L: Solitary fibrous tumour and haemangiopericytoma:
evolution of a concept. Histopathology 2006, 48:63-74.
10. del Rosario ML, Saleh A: Preoperative chemotherapy for congenital
hemangiopericytoma and a review of the literature. J Pediatr Hematol
Oncol 1997, 19:247-250.
11. Jha N, McNeese M, Barkley HT Jr, 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, 13:1399-402.
doi:10.1186/1752-1947-4-8
Cite this article as: Kitahata et al.: Hemangiopericytoma in the
sacrococcygeal space: a case report. Journal of Medical Case Reports 2010
4:8.
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A: Preoperative chemotherapy for congenital hemangiopericytoma and a review of the literature. J Pediatr Hematol Oncol

BH: Hemangiopericytoma: an analysis of 106 cases. Hum Pathol

C: A rare case of solitary fibrous tumour of the presacral space: morphological and immunohistochemical features. In Vivo

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

E: Malignant solitary fibrous tumour of the peritoneum. Histopathol

Endo K: FDG-PET for preoperative differential diagnosis between benign and malignant soft tissue masses. Skeletal Radiol

Ettorre GM: Malignant haemangiopericytoma of the mesorectum. Chir Ital

Feig BW: Hemangiopericytoma: a 20-year single-institution experience. Ann Surg Oncol

Guillou L: Solitary fibrous tumour and haemangiopericytoma: evolution of a concept. Histopathology

Retroperitoneal and pelvic hemangiopericytomas: clinical, radiologic, and pathologic correlation. Radiology

Sisely L: Haemangiopericytoma of the presacral space.

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Hemangiopericytoma in the sacrococcygeal space: a case report

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