CASE REPORTS

 

Pelvic hemangiopericytoma. An unusual location of a vascular tumor

Hemangiopericitoma pélvico. Una localización infrecuente de tumor vascular

 

 

Irene Álvarez-Abad¹, José María García-González¹, Leire Agirre-Etxabe¹, Ana María Quintana-Rivera² and Alberto Colina-Alonso

Departments of ¹General and Digestive Surgery, and ²Angiology and Vascular Surgery. Hospital Universitario de Cruces. Barakaldo, Vizcaya. Spain

Correspondence

 

 

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ABSTRACT

Background: The hemangiopericytoma is an uncommon vascular tumour. We report a case of interest because of its rarity, size and location.
Case report: We present the case of a 63-year-old woman who consulted for abdominal pain. TAC, MRI and arteriography showed a pelvic mass occupying Douglas' space, displacing the uterus, bladder and sigma, with vascularisation relative to the inferior mesenteric artery and both hypogastric arteries. The vascularity of the tumour itself was selectively embolised before the mass was resected. There were no intra- or postoperative complications. Pathology confirmed the diagnosis of hemangiopericitoma. The patient is being monitored as an outpatient, with no signs of recurrence to date.
Discussion: The hemangiopericytoma is a tumour of the pericyte cells so it can occur in any location. The pelvic location is exceptional. The tumour may appear as nonspecific abdominal pain, show signs of compression of adjacent organs or occasionally be associated with paraneoplastic syndromes. The diagnosis is suspected via CT and angiography findings, but confirmation is only made by analysing the surgical specimen. The treatment of choice is surgery, in some cases after preoperative embolisation of the vascularisation of the mass. There is no agreement on chemo/radiotherapy as the primary treatment for hemangiopericytoma, although adjuvant radiation therapy has been found to improve local control and reduce recurrences. The prognosis is good if complete resection is achieved, with five- and 10-year survival rates between 70 and 80%, depending on the series.

Key words: Hemangiopericytoma. Vascular. Pelvis.

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RESUMEN

Introducción: el hemangiopericitoma es una neoplasia infrecuente de estirpe vascular. Presentamos un caso clínico de interés dada su escasa frecuencia, tamaño y localización.
Caso clínico: se trata de una mujer de 63 años que consultó por dolor abdominal. Se realizaron TAC, RMN abdominal y arteriografía que mostraban una gran masa pélvica que ocupaba el espacio de Douglas y desplazaba útero, vejiga y sigma y presentaba vascularización dependiente de la arteria mesentérica interior y ambas arterias hipogástricas. Se embolizó selectivamente la vascularización propia de la neoplasia y seguidamente se resecó la masa, sin presentarse complicaciones ni intra ni postoperatorias. La anatomía patológica confirmó el diagnóstico de hemangiopericitoma. La paciente sigue controles en consultas externas sin signos de recidiva hasta la fecha.
Discusión: el hemangiopericitoma procede de las células del pericito por lo que puede presentarse en cualquier localización. La localización pélvica es excepcional. Puede presentarse como dolor abdominal inespecífico, dar síntomas de compresión de órganos vecinos y, ocasionalmente, asociarse a síndromes paraneoplásicos. El diagnóstico de sospecha es mediante TAC y angiografía aunque la confirmación es histológica tras analizar la pieza de resección. El tratamiento de elección es quirúrgico, precediéndose en algunos casos de embolización preoperatoria de la vascularización de la masa. No hay acuerdo sobre la quimio/radioterapia como tratamiento primario del hemangiopericitoma, aunque se ha descrito la radioterapia adyuvante para mejorar del control local y disminuir las recurrencias. El pronóstico es bueno si se consigue una resección completa, con supervivencias a 5 y 10 años entre el 70 y el 80% según las series.

Palabras clave: Hemangiopericitoma. Vascular. Pelvis.

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Introduction

The hemangiopericytoma is a rare and potentially malignant vascular tumor, which includes 1% of vascular tumors (1). It originates from the mesenchymal cells of the pericyte (1,2). We report a case of interest because of its rarity, size and location.

 

Case report

This is a 63-year-old woman who consulted for abdominal pain. The CT and MRI showed a solid cystic mass of 12 x 9 x 9.4 cm with punctate calcifications and a rich vascularity occupying almost the entire Douglas space, displacing the uterus (U), bladder (V) and the right side of sigmoid colon, but without infiltrating them. The study was completed with an arteriography that showed vascular contributions relative to the inferior mesenteric artery (IMA) and both hypogastric arteries (AH) (Fig. 1).

 

 

Prior to the surgery, a selective embolization of the tumor vasculature was performed to reduce the risk of intraoperative bleeding, and then, the pelvic mass was successfully removed without intra- or postoperative complications. Histology confirmed the diagnosis of pelvic hemangiopericytoma with free resection edges. The patient is currently being monitored as an outpatient with no clinical or radiological signs of recurrence to date.

 

Discussion

Hemangiopericytoma is a rare tumor of the pericyte that surrounds capillaries and venules, so it can occur at any location (1,3). The most common locations are the lower limbs, followed by the retroperitoneal area. Pelvic location is exceptional.

Clinically, the tumor usually appears as a painless mass, but it may also show signs of compression of neighboring organs or the development of paraneoplastic syndromes (1,2,4,5). CT and angiography confirm the diagnosis. Percutaneous biopsy is not recommended because of the high risk of bleeding. The definitive diagnosis is anatomopathological after analyzing the surgical specimen (6). The distinction between benign or malignant hemangiopericitoma is based on clinical and pathological criteria (7). It is considered to be malignant if it invades nearby structures, develops metastasis or recurs during follow-up (7). Although there is no histological pattern of malignancy, it has been shown that size, high mitotic index, high cellularity and the presence of necrosis or hemorrhage can promote the development of recurrence and/or metastasis (2,7). The treatment of choice is complete resection, sometimes preceded by embolization of the vascularization of the mass to decrease the risk of bleeding (1,7,8). There is disagreement about the effectiveness of chemo/radiotherapy as the primary treatment. The use of adjuvant radiation therapy has been described for improving local control and reducing recurrences, although it is considered to be a relatively radio-resistant tumor (1,2,9,10). The prognosis is conditional on achieving a complete resection, with five- and ten-year survival rates between 70 and 80%, depending on the series (6). The recurrence rate ranges between 20 and 50% after resection. Long-term monitoring is recommended because recurrence may appear at a later time (2).

 

References

1. Villalba V, García R, Gubert J, et al. Hemangiopericitoma de localización intestinal. Cir Esp 2005;77:48-50. DOI: 10.1016/S0009-739X(05)70804-6.         [ Links ]

2. Prats López J, Bellmunt Molins J, Calvo Mateo MA, et al. Hemangiopericitoma pelviano. Arch Esp Urol 1988;41;6;(475-478).         [ Links ]

3. Stout AP, Murray MR. Hemangiopericytoma: A vascular tumor featuring Zimmerman's pericytes. Ann Surg 1942;116:26-33. DOI: 10.1097/00000658-194207000-00004.         [ Links ]

4. Sheldon C, Binder MD, Hubert J, et al. Intraabdominal hemangiopericytoma: Report of four cases and review of literature. Arch Surg 1973;107:536-43. DOI: 10.1001/archsurg.1973.01350220020006.         [ Links ]

5. McMaster MJ, Soule EH, Ivins JC. Hemangiopericytoma: A clinico-pathologic study and long-term follow up of 60 patiens. Cancer 1975;36:2232-44. DOI: 10.1002/cncr.2820360942.         [ Links ]

6. Lema Grille J, Cimadevilla García A, Rodríguez Núñez H, et al. Hemangiopericitoma retroperitoneal gigante. Actas Urológicas Españolas 2001;25;672-675. DOI: 10.1016/S0210-4806(01)72696-4.         [ Links ]

7. Lado Lado FL, Carballo Fernández C, Cutin Prieto C, et al. Hemangiopericitoma de localización pelviana con extirpación amplia y recidiva retroperitoneal que causa la muerte al cabo de cinco años. Neoplasia 1987;4:86.         [ Links ]

8. García Molina FJ, Franco Osorio JD, Medina Díez J. Hemangiopericitoma pélvico. Extirpación combinada abdominoperineal. Rev Esp Enferm Dig 2002;94:558-60.         [ Links ]

9. Mira J, Chu F, Fortner J. The role of radiotherapy in the management of malignant hemangiopericytoma: Report of eleven new cases and review of the literature. Cancer 1977;39:1254-9. DOI: 10.1002/1097-0142(197703)39:3<1254:AID-CNCR2820390335>3.0.CO;2-J.         [ Links ]

10. Wong P, Yagoda A. Chemotherapy of malignant hemangiopericytoma. Cancer 1978;41:1256-60. DOI: 10.1002/1097-0142(197804)41:4<1256:AID-CNCR2820410406>3.0.CO;2-V.         [ Links ]

 

 

Correspondence:
Irene Álvarez-Abad.
General and Digestive Surgery Department.
Hospital Universitario de Cruces.
Plaza Cruces, s/n.
48903 Barakaldo, Vizcaya. Spain
e-mail: irene.alvarezabad@osakidetza.net

Received: 19-12-2015
Accepted: 13-01-2016