Splenic rupture as the presenting manifestation of primary splenic angiosarcoma in a teenage woman: a case report

Manouras, Andreas; Giannopoulos, Panagiotis; Toufektzian, Levon; Markogiannakis, Haridimos; Lagoudianakis, Emmanuel; Papadima, Artemisia; Papanikolaou, D.; Filis, Konstantinos; Kekis, Panagiotis

doi:10.1186/1752-1947-2-133

Cited by 23 publications

(16 citation statements)

References 10 publications

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“…14 Some studies have suggested this neoplasm shows a slight male predominance, 15 with no predilection for race, geographic location or heritability. Conversely, some studies have suggested a slight female predilection, 5 but no gender predilection was observed in the two largest studies.…”

Section: Discussionmentioning

confidence: 99%

“…2 Conversely, others have proposed that splenic rupture is the worst prognostic factor for survival, due to increased risk of peritoneal dissemination with direct implantation of neoplastic tissue associated with vascular access and hematogenous spread. 15 One rare clinical manifestation is gastrointestinal bleeding secondary to metastatic disease, and to our knowledge there have only been two previous reported cases. 9 In the case of gastrointestinal bleeding, the clinical course is poor, with a survival rate ranging between 10.3 to 13 months.…”

Section: Discussionmentioning

confidence: 99%

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Systemic therapy in primary angiosarcoma of the spleen

et al. 2012

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Primary splenic angiosarcoma is a very rare neoplasm with a high propensity for metastatic disease and poor prognosis. There is a paucity of literature concerning this specific sarcoma subtype and the role of systemic therapy is not well defined. A retrospective review of the prospectively maintained University of Washington/Seattle Cancer Care Alliance Sarcoma Unit database was performed to identify patients with splenic angiosarcoma treated between 2007 and 2012. In total there were 19 patients with angiosarcoma treated at the Seattle Cancer Care Alliance from 2007 to 2012. The number of patients with splenic angiosarcoma was 2 (11%). The first patient was a woman aged 57 years who was referred with metastatic splenic angiosarcoma to the liver, post-splenectomy. She was treated with 4 cycles of weekly paclitaxel prior to metastatic resection and 4 cycles of the same drug in an adjuvant scenario, achieving a pathological complete response to treatment. She is alive and on third-line systemic therapy. The second patient was a male patient aged 30 years who presented with metastatic high-grade splenic angiosarcoma and was treated with 3 lines of systemic therapy, including doxorubicin, paclitaxel and gemcitabine+docetaxel, but developed a gastrointestinal metastasis with subsequent gastrointestinal bleeding. Splenic angiosarcoma is a very rare neoplasm. Surgery remains the mainstay of management for localized disease. Paclitaxel administered weekly proved to be well-tolerated and resulted in a good radiological response in one of our patients, enabling resection of metastatic disease. Durable clinical benefit can be achieved in metastatic splenic angiosarcoma with multi modality management.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Systemic therapy in primary angiosarcoma of the spleen

et al. 2012

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“…Although typically described as rare, primary splenic angiosarcoma nonetheless carries with it an extremely low survival rate upon infection. It has an ever-changing clinical and laboratory profile, with no consideration for genetics, ethnicity, or geographical location [7]. Its diagnostic evasiveness may be further compounded by the fact that although it is typically thought to occur between the 5 th and 6 th decade of life, it may also strike the paediatric population at virtually any age [4, 7].…”

Section: Discussionmentioning

confidence: 99%

Primary splenic angiosarcoma – the same diagnosis yielding two different clinical pictures.

Kamocki¹,

Steward²,

Zaręba³

et al. 2013

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Splenic angiosarcoma has been presented as an extremely rare malignant tumour. Amongst the rarest of all neoplasms, primary splenic angiosarcoma comprises 0.14–0.25 of all annually reported cases per one million persons Splenic angiosarcoma was first described in 1879 by T. Langerhans. The clinical symptoms and diagnostic values associated with splenic angiosarcoma are extremely variable. Nonetheless, majority of the patients (75%) complain of abdominal pain, and a quarter to one-third present with rupture of the involved organ. We hereby report our experience with two splenic angiosarcoma cases, which despite being similar in their diagnosis, nonetheless present with an entirely different clinical picture. Our first case, whereby the patient presented with a liver that was also affected by the angiosarcoma of the spleen. In the second case however, although the patient did show evidence of metastasis to the abdominal cavity as well as the liver, she also suffered from primary adenocarcinoma of the colon. To our knowledge, no previous article has made mention of primary splenic angiosarcoma existing independently of another primary neoplasm. Both may be found in common practice, and ought to be highlighted for their significance as such.

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“…La médiane de survie est d'un an jusqu'à trois ans, après splénectomie pour des tumeurs détectées précocement [13]. Elle est de quatre mois après splénectomie pratiquée en urgence sur rupture spontanée splénique [14].…”

Section: Discussionunclassified

Hématémèse révélant un angiosarcome splénique: à propos d’un cas

Soufi¹,

Benamer²,

Mdaghri³

et al. 2009

J Afr Cancer

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L'angiosarcome primitif de la rate est une tumeur maligne rare avec un pronostic fâcheux. Les hémorragies digestives constituent un mode de révélation inhabituelle. On rapporte le cas d'un patient de 71 ans présentant un angiosarcome primitif de la rate révélé par des hématé-mèses. Le diagnostic était fait en préopératoire grâce à l'imagerie et à la biopsie. La chirurgie et la chimiothérapie constituent les principaux traitements. Avec une revue de la littérature, quelques concepts cliniques radiologiques et thérapeutiques de ces rares tumeurs seront discutés. Mots clés Angiosarcome · Rate · Hématémèse · Chirurgie · ChimiothérapieAbstract Primary angiosarcoma of the spleen is a rare neoplasm with a very poor prognosis. Gastrointestinal bleeding is unusual presentation. We report the case of a 71-year-old man with primary splenic angiosarcoma revealed by hematemesis. Surgery and chemotherapy are the principal treatments. The literature on primary splenic angiosarcoma is reviewed. Clinical, radiological and therapeutic aspects of this rare tumor are discussed.

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Splenic rupture as the presenting manifestation of primary splenic angiosarcoma in a teenage woman: a case report

Cited by 23 publications

References 10 publications

Systemic therapy in primary angiosarcoma of the spleen

Systemic therapy in primary angiosarcoma of the spleen

Primary splenic angiosarcoma – the same diagnosis yielding two different clinical pictures.

Hématémèse révélant un angiosarcome splénique: à propos d’un cas

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