Haemangiopericytoma of the greater omentum: a rare tumour requiring long-term follow-up

Haemangiopericytoma of the greater omentum: a rare tumour requiring long-term follow-up Haemangiopericytomas (HPC) are highly vascularized tumours located in any part of the body where capillaries can be found. Since 2002, they have been re-classified under the umbrella ‘extrapleural Solitary Fibrous Tumour (SFT)’ and the term HPC is nowadays used to describe a growth pattern rather than a clinical entity. Their biological behaviour varies and they require a long-term follow-up since they may recur or metastasise several years after successful treatment. We present the case of a gentleman with HPC of the greater omentum initially appeared in 1998. HPC rarely develops in the greater omen- tum and only 20 cases have been described in the literature till today. Despite complete excision the mass re-appeared in 2011 and 2017, 13 and 19 years after initial treatment. Surgical management included en bloc excision of three lesions along with greater omentum. No further treatment was required. appeared gradually over several months causing a dull pain. INTRODUCTION No other symptoms were described. Clinical examination Haemangiopericytomas (HPC) are highly vascularized tumours revealed a 11 × 10 cm soft and well defined, palpable mass located in any part of the body where capillaries can be found on the right side of the abdominal wall (Fig. 1). Computer [1]. They were first described in 1942 and since 2002 they have tomography (CT) of the abdomen presented several space- been grouped into the umbrella ‘extrapleural solitary fibrous occupying lesions in the peritoneal cavity, the right side of tumour (SFT)’ [2]. Their biological behaviour varies and long- the lower pelvis and the rectovesical pouch, measuring 11 × term follow-up is required, even after successful management 10.4 × 10.7 cm, 8 × 6.5 cm and 7.5 × 5.7 cm, respectively (Figs [3]. We present the case of a gentleman with HPC of the greater 2 and 3). Due to compression, the veins of the anterior omentum initially appeared in 1998 and, despite successful abdominal wall presented dilated. Thorax CT scan revealed management, it recurred in 2011 and 2017. no tumour. On exploratory laparotomy four large lesions were revealed CASE REPORT arising from the greater omentum. No peritoneal or organ A 72-year-old gentleman presented to our unit with a recur- metastasis were seen. The lesions were excised en bloc with rent mass of the anterior abdominal wall. This mass the greater omentum (Fig. 4). The masses were surrounded by Received: February 26, 2018. Accepted: April 18, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy087/4995818 by Ed 'DeepDyve' Gillespie user on 21 June 2018 2 D. Vasdeki et al. Figure 1: The patient presented with a 11 × 10 cm soft and well defined, palp- able mass on the right side of the abdominal wall. Figure 3: Abdominal CT scan showing two hypervascular lesions in the abdomen. Figure 2: Abdominal CT scan showing a inhomogeneous hypervascular mass. Figure 4: Intra-operatively three lesions derived from the greater omentum were excised along with the greater omentum. blood vessels of large calibre, approximately 0.5 cm. Due to their vascular nature, there was a significant amount of blood loss intra-operatively and therefore the patient remained in DISCUSSION ICU for 24 hours post-operatively. Subsequently he had an uneventful recovery and was discharged 7 days after his Haemangiopericytoma has been considered a controversial operation. highly vascularized tumour. It was first described by Stout and The patient had undergone excision of similar lesions from Murray in 1942 as a neoplasm derived from the pericytes of the anterior abdominal wall in 1998 and 2011 (19 and 6 years Zimmerman, modified smooth muscle cells that regulate the before the present case, respectively). Both operations were calibre of the capillary lumen [4, 5]. Since 2002 haemangioperi- performed in other institutions and only the biopsy reports cytomas have been reclassified as solitary fibrous tumour (SFT), were available to us. The first biopsy confirmed haemangioperi- since the term HPC gathers numerous non-related entities that cytoma, more likely malignant, due to display of pleomorph- share certain morphologic characteristics. The term ‘extra-pleural ism, hypercellularity and infiltration of the capsule of the mass. SFT’ describes extra-meningeal SFTs, haemangiopericytomas, In 2011, two lesions were excised, along with part of the greater lipomatous haemangiopericytomas and giant cell angiofibromas, omentum. Histological evaluation showed haemangiopericyto- as defined by the World Health Organization (WHO) [2, 6–8]. ma. Immunohistochemical analysis showed positivity for The term haemangiopericytoma is nowadays used to vimentin, CD34 and CD99 and a mitotic count of 1 mitosis per describe a growth pattern rather than a clinical entity [5]. 10 high-power fields (HPF). Biopsy of the present case also con- Histologically, HPC are sinusoidal vascular tumours with firmed a haemangiopericytoma; necrosis was present, while staghorn-shaped blood vessels surrounded by spindle shaped mitoses were <4/10HPF. cells [4]. Several tumour types share similar vascularity, Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy087/4995818 by Ed 'DeepDyve' Gillespie user on 21 June 2018 Haemangiopericytoma of the greater omentum 3 resulting in difficulties in their diagnosis, management and limited and more studies still need to be conducted [6]. The risk prognosis. Immunochemistry has not met great success in the of perioperative bleeding has always to be considered. Their diagnosis of HPC, since cell markers present in normal peri- extreme vascularity can make some lesions unresectable due cytes, such as desmin and muscle actins, are not frequently to high risk of significant blood loss. Pre-operative vascular found in HPC cells [4]. Moreover, they display several features embolization of the lesion has been an effective measure to of pericytic, fibroblastic and myofibroblastic differentiation, reduce blood loss intra-operatively [9]. while they express CD34, CD99 and Bcl-2 antigens [5]. In conclusion, HPC should be included in the differential Soft tissue SFTs represent <2% of all soft tissue tumours; diagnosis of vascular tumours. After radical excision of the malignant HPCs represent <1% of all vascular tumours and tumour long-term follow-up is essential, since recurrence and around 5% of all sarcomatous tumours [2, 5, 6]. HPCs can more metastasis can happen several years after treatment. frequently be located at the lower extremities (34,9%), retroperi- toneum and pelvic cavity (24,5%) [1, 9]. The greater omentum has been a rare site for its occurrence; only 20 cases of HPC CONFLICT OF INTEREST STATEMENT of the greater omentum have been described [8]. The median None declared. age of diagnosis is 45 years. The distribution is equal in both sexes, while there is no evidence of increased familial incidence [1, 6, 9]. They can present varied biological behaviour from REFERENCES being a slow growing tumour to a mass with aggressive growth. They can metastasise to lungs, while recurrence and metasta- 1. Küçüm HF, Gülmez S, Kaptanoglu ˘ L, Akyol H, Kurt N, sis can develop several years after treatment. Therefore, a long- Yavuzer D. Acute abdomen due to rupture of hemangioperi- term follow-up is required even after radical excision of the cytoma of the greater omentum: case report. Ulus Travma mass [3]. Acil Cerrahi Derg 2009;15:611–3. The majority has a relatively indolent behaviour with present- 2. Pasquali S, Gronchi A, Strauss D, Bonvalot S, Jeys L, ing symptoms being vague for several months and not specific. Stacchiotti S, et al. Resectable extra-pleural and extra- Pain or abdominal fullness can be late symptoms associated with meningeal solitary fibrous tumours: a multi-centre prognos- an enlarging mass or perineural invasion [2, 4, 7, 9]. tic study. Eur J Surg Oncol 2016;42:1064–70. The criteria for malignancy remain a topic of debate. 3. Deshmukh S, Kalele KP, Patil KP, Nayyar AS. Hemangioperi- Enzinger and Smith proposed large lesion size (>5 cm), high cel- cytoma: a rare case. Int J Case Rep Images 2015;6:517–22. lularity, high mitotic count (≥4/10 HPF), display of pleomorph- 4. Espat NJ, Lewis JJ, Leung D, Woodruff JM, Antonescu CR, ism and the presence of tumour haemorrhage and necrosis as Shia J, et al. Conventional hemangiopericytoma: modern criteria indicating malignancy [1, 8, 9]. Pasquali et al. [2] pre- analysis of outcome. Cancer 2002;95:1746–51. sented that high mitotic rate, hypercellularity and pleomorph- 5. Radulescu D, Pripon S, Ciuleanu TE, Radulescu LI. Malignant ism have been associated with a higher risk of recurrence, primary pulmonary tumor with hemangiopericytoma-like approximately 50% at 5 years, while tumour size has been features: conventional hemangiopericytoma versus solitary inversely associated with prognosis. fibrous tumor. Clin Lung Cancer 2007;8:504–8. Treatment options of HPC cases should be discussed in a 6. Renel N, Amela EY, Decanter G, Robin YM, Marec-Berard P. team with experience in sarcoma surgery. En-bloc surgical exci- Solitary fibrous tumor and so-called hemangiopericytoma. sion has been the mainstay of treatment, the aim being to Sarcoma 2012, Article ID 690251, 6 pages, 2012. doi:10.1155/ achieve complete resection with negative margins. Radiation 2012/690251. therapy can improve local control rates post-operatively, but 7. Fard-Aghaie M, Stavrou GA, Honarpisheh H, Niehaus KJ, cannot result in complete remission of HPC. It can be recom- Oldhafer KJ. Large hemangiopericytoma of the pelvis—towards mended for tumours displaying criteria for malignancy and as a multidisciplinary approach. World J Surg Oncol 2015;13:261. an alternative treatment for unresectable or recurrent HPC. 8. Archid R, Schneider CC, Adam P, Othman A, Zieker D, There is little evidence to support the use of adjuvant chemo- Königsrainer A. Hemangiopericytoma/solitary fibrous tumor therapy in their management; partial or short-term remission of the greater omentum: a case report and review of the lit- of metastatic disease has been sporadically documented after erature. Int J Surg Case Rep 2016;23:160–2. chemotherapy [1, 2, 6, 9]. Tyrosine kinase inhibitors could pro- 9. Chan SC, Lee CM, Ng YB, Tsai CH. Giant retroperitoneal vide long-lasting stable disease, although the experience is hemangiopericytoma. Postgrad Med J 1995;71:692–700. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy087/4995818 by Ed 'DeepDyve' Gillespie user on 21 June 2018 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Surgical Case Reports Oxford University Press

Haemangiopericytoma of the greater omentum: a rare tumour requiring long-term follow-up

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Abstract

Haemangiopericytomas (HPC) are highly vascularized tumours located in any part of the body where capillaries can be found. Since 2002, they have been re-classified under the umbrella ‘extrapleural Solitary Fibrous Tumour (SFT)’ and the term HPC is nowadays used to describe a growth pattern rather than a clinical entity. Their biological behaviour varies and they require a long-term follow-up since they may recur or metastasise several years after successful treatment. We present the case of a gentleman with HPC of the greater omentum initially appeared in 1998. HPC rarely develops in the greater omen- tum and only 20 cases have been described in the literature till today. Despite complete excision the mass re-appeared in 2011 and 2017, 13 and 19 years after initial treatment. Surgical management included en bloc excision of three lesions along with greater omentum. No further treatment was required. appeared gradually over several months causing a dull pain. INTRODUCTION No other symptoms were described. Clinical examination Haemangiopericytomas (HPC) are highly vascularized tumours revealed a 11 × 10 cm soft and well defined, palpable mass located in any part of the body where capillaries can be found on the right side of the abdominal wall (Fig. 1). Computer [1]. They were first described in 1942 and since 2002 they have tomography (CT) of the abdomen presented several space- been grouped into the umbrella ‘extrapleural solitary fibrous occupying lesions in the peritoneal cavity, the right side of tumour (SFT)’ [2]. Their biological behaviour varies and long- the lower pelvis and the rectovesical pouch, measuring 11 × term follow-up is required, even after successful management 10.4 × 10.7 cm, 8 × 6.5 cm and 7.5 × 5.7 cm, respectively (Figs [3]. We present the case of a gentleman with HPC of the greater 2 and 3). Due to compression, the veins of the anterior omentum initially appeared in 1998 and, despite successful abdominal wall presented dilated. Thorax CT scan revealed management, it recurred in 2011 and 2017. no tumour. On exploratory laparotomy four large lesions were revealed CASE REPORT arising from the greater omentum. No peritoneal or organ A 72-year-old gentleman presented to our unit with a recur- metastasis were seen. The lesions were excised en bloc with rent mass of the anterior abdominal wall. This mass the greater omentum (Fig. 4). The masses were surrounded by Received: February 26, 2018. Accepted: April 18, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy087/4995818 by Ed 'DeepDyve' Gillespie user on 21 June 2018 2 D. Vasdeki et al. Figure 1: The patient presented with a 11 × 10 cm soft and well defined, palp- able mass on the right side of the abdominal wall. Figure 3: Abdominal CT scan showing two hypervascular lesions in the abdomen. Figure 2: Abdominal CT scan showing a inhomogeneous hypervascular mass. Figure 4: Intra-operatively three lesions derived from the greater omentum were excised along with the greater omentum. blood vessels of large calibre, approximately 0.5 cm. Due to their vascular nature, there was a significant amount of blood loss intra-operatively and therefore the patient remained in DISCUSSION ICU for 24 hours post-operatively. Subsequently he had an uneventful recovery and was discharged 7 days after his Haemangiopericytoma has been considered a controversial operation. highly vascularized tumour. It was first described by Stout and The patient had undergone excision of similar lesions from Murray in 1942 as a neoplasm derived from the pericytes of the anterior abdominal wall in 1998 and 2011 (19 and 6 years Zimmerman, modified smooth muscle cells that regulate the before the present case, respectively). Both operations were calibre of the capillary lumen [4, 5]. Since 2002 haemangioperi- performed in other institutions and only the biopsy reports cytomas have been reclassified as solitary fibrous tumour (SFT), were available to us. The first biopsy confirmed haemangioperi- since the term HPC gathers numerous non-related entities that cytoma, more likely malignant, due to display of pleomorph- share certain morphologic characteristics. The term ‘extra-pleural ism, hypercellularity and infiltration of the capsule of the mass. SFT’ describes extra-meningeal SFTs, haemangiopericytomas, In 2011, two lesions were excised, along with part of the greater lipomatous haemangiopericytomas and giant cell angiofibromas, omentum. Histological evaluation showed haemangiopericyto- as defined by the World Health Organization (WHO) [2, 6–8]. ma. Immunohistochemical analysis showed positivity for The term haemangiopericytoma is nowadays used to vimentin, CD34 and CD99 and a mitotic count of 1 mitosis per describe a growth pattern rather than a clinical entity [5]. 10 high-power fields (HPF). Biopsy of the present case also con- Histologically, HPC are sinusoidal vascular tumours with firmed a haemangiopericytoma; necrosis was present, while staghorn-shaped blood vessels surrounded by spindle shaped mitoses were <4/10HPF. cells [4]. Several tumour types share similar vascularity, Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy087/4995818 by Ed 'DeepDyve' Gillespie user on 21 June 2018 Haemangiopericytoma of the greater omentum 3 resulting in difficulties in their diagnosis, management and limited and more studies still need to be conducted [6]. The risk prognosis. Immunochemistry has not met great success in the of perioperative bleeding has always to be considered. Their diagnosis of HPC, since cell markers present in normal peri- extreme vascularity can make some lesions unresectable due cytes, such as desmin and muscle actins, are not frequently to high risk of significant blood loss. Pre-operative vascular found in HPC cells [4]. Moreover, they display several features embolization of the lesion has been an effective measure to of pericytic, fibroblastic and myofibroblastic differentiation, reduce blood loss intra-operatively [9]. while they express CD34, CD99 and Bcl-2 antigens [5]. In conclusion, HPC should be included in the differential Soft tissue SFTs represent <2% of all soft tissue tumours; diagnosis of vascular tumours. After radical excision of the malignant HPCs represent <1% of all vascular tumours and tumour long-term follow-up is essential, since recurrence and around 5% of all sarcomatous tumours [2, 5, 6]. HPCs can more metastasis can happen several years after treatment. frequently be located at the lower extremities (34,9%), retroperi- toneum and pelvic cavity (24,5%) [1, 9]. The greater omentum has been a rare site for its occurrence; only 20 cases of HPC CONFLICT OF INTEREST STATEMENT of the greater omentum have been described [8]. The median None declared. age of diagnosis is 45 years. The distribution is equal in both sexes, while there is no evidence of increased familial incidence [1, 6, 9]. They can present varied biological behaviour from REFERENCES being a slow growing tumour to a mass with aggressive growth. They can metastasise to lungs, while recurrence and metasta- 1. Küçüm HF, Gülmez S, Kaptanoglu ˘ L, Akyol H, Kurt N, sis can develop several years after treatment. Therefore, a long- Yavuzer D. Acute abdomen due to rupture of hemangioperi- term follow-up is required even after radical excision of the cytoma of the greater omentum: case report. Ulus Travma mass [3]. Acil Cerrahi Derg 2009;15:611–3. The majority has a relatively indolent behaviour with present- 2. Pasquali S, Gronchi A, Strauss D, Bonvalot S, Jeys L, ing symptoms being vague for several months and not specific. Stacchiotti S, et al. Resectable extra-pleural and extra- Pain or abdominal fullness can be late symptoms associated with meningeal solitary fibrous tumours: a multi-centre prognos- an enlarging mass or perineural invasion [2, 4, 7, 9]. tic study. Eur J Surg Oncol 2016;42:1064–70. The criteria for malignancy remain a topic of debate. 3. Deshmukh S, Kalele KP, Patil KP, Nayyar AS. Hemangioperi- Enzinger and Smith proposed large lesion size (>5 cm), high cel- cytoma: a rare case. Int J Case Rep Images 2015;6:517–22. lularity, high mitotic count (≥4/10 HPF), display of pleomorph- 4. Espat NJ, Lewis JJ, Leung D, Woodruff JM, Antonescu CR, ism and the presence of tumour haemorrhage and necrosis as Shia J, et al. Conventional hemangiopericytoma: modern criteria indicating malignancy [1, 8, 9]. Pasquali et al. [2] pre- analysis of outcome. Cancer 2002;95:1746–51. sented that high mitotic rate, hypercellularity and pleomorph- 5. Radulescu D, Pripon S, Ciuleanu TE, Radulescu LI. Malignant ism have been associated with a higher risk of recurrence, primary pulmonary tumor with hemangiopericytoma-like approximately 50% at 5 years, while tumour size has been features: conventional hemangiopericytoma versus solitary inversely associated with prognosis. fibrous tumor. Clin Lung Cancer 2007;8:504–8. Treatment options of HPC cases should be discussed in a 6. Renel N, Amela EY, Decanter G, Robin YM, Marec-Berard P. team with experience in sarcoma surgery. En-bloc surgical exci- Solitary fibrous tumor and so-called hemangiopericytoma. sion has been the mainstay of treatment, the aim being to Sarcoma 2012, Article ID 690251, 6 pages, 2012. doi:10.1155/ achieve complete resection with negative margins. Radiation 2012/690251. therapy can improve local control rates post-operatively, but 7. Fard-Aghaie M, Stavrou GA, Honarpisheh H, Niehaus KJ, cannot result in complete remission of HPC. It can be recom- Oldhafer KJ. Large hemangiopericytoma of the pelvis—towards mended for tumours displaying criteria for malignancy and as a multidisciplinary approach. World J Surg Oncol 2015;13:261. an alternative treatment for unresectable or recurrent HPC. 8. Archid R, Schneider CC, Adam P, Othman A, Zieker D, There is little evidence to support the use of adjuvant chemo- Königsrainer A. Hemangiopericytoma/solitary fibrous tumor therapy in their management; partial or short-term remission of the greater omentum: a case report and review of the lit- of metastatic disease has been sporadically documented after erature. Int J Surg Case Rep 2016;23:160–2. chemotherapy [1, 2, 6, 9]. Tyrosine kinase inhibitors could pro- 9. Chan SC, Lee CM, Ng YB, Tsai CH. Giant retroperitoneal vide long-lasting stable disease, although the experience is hemangiopericytoma. Postgrad Med J 1995;71:692–700. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy087/4995818 by Ed 'DeepDyve' Gillespie user on 21 June 2018

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Journal of Surgical Case ReportsOxford University Press

Published: May 14, 2018

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