Splenic Angiosarcoma: A Diagnostic Splenectomy Finding

Azevedo, Otavio Schmidt de; Santos, Bruna do Nascimento; Liboni, Nelson de Souza; Costa, Juliano Fernandes da; Campos, Olímpio Daniel de

doi:10.1159/000452619

Cited by 7 publications

(2 citation statements)

References 18 publications

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“…More than 300 PSA cases have been described 4. Azevedo et al5 reported the case of a 57-year-old woman previously treated for breast ductal carcinoma, and as far as we know, to date, our case was the first case report of PSA with spontaneous rupture in a patient in Brazil.…”

Section: Introductionmentioning

confidence: 61%

Primary splenic angiosarcoma: a rare entity often associated with rupture and hemoperitoneum

et al. 2019

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Primary splenic angiosarcoma (PSA) is a rare neoplasm of vascular origin associated with aggressive behavior and poor prognosis. The clinical presentation is usually non-specific and is mostly characterized by a wasting disease with anemia and splenomegaly, mimicking a wide range of entities. The authors present the case of an 80-year-old woman with cardiovascular comorbidities with a 6-month history of weight loss, fatigue, weakness, pallor, and abdominal pain. The physical examination showed massive splenomegaly and pallor. After a thorough evaluation that ruled out lymphoproliferative diseases, the working diagnosis was a myelodysplastic disorder. A few days after discharge, she returned to the emergency room with severe abdominal pain, worsening fatigue, and a remarkable pallor. Point-of-care ultrasound showed free intraperitoneal fluid. Spleen rupture was confirmed by abdominal computed tomography (CT) scan, and an emergency laparotomy with splenectomy was performed. The postoperative period was uneventful, and the patient recovered in a few days. The histopathology confirmed the diagnosis of PSA and the patient was referred to an oncological center. Two months later staging CT demonstrated liver and peritoneal metastases, and despite the chemotherapy she died 6 months after the diagnosis.

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Section: Introductionmentioning

confidence: 61%

Primary splenic angiosarcoma: a rare entity often associated with rupture and hemoperitoneum

et al. 2019

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“…Paclitaxel was considered as an adjuvant and neoadjuvant therapy by Vakkalanka and Milhem, [28] suggesting that chemotherapy may decrease local recurrence and improve overall survival. de Azevedo et al [29] also reported a patient remained disease free for 1 year and 2 months after paclitaxel adjuvant chemotherapy. Hara et al [30] presented a patient who received autologous peripheral blood stem cell transplantation combined with high-dose chemotherapy after splenectomy.…”

Section: Discussionmentioning

confidence: 99%

A primary splenic angiosarcoma hepatic metastasis after splenectomy and its genomic alteration profile

et al. 2019

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Rationale: Primary splenic angiosarcoma (PSA) is a rare mesenchymal malignancy of the splenic vascular origin often with a dismal prognosis. Genomic profile may provide evidence for the solution of therapy. Patient concerns: We reported a case of a 51-year-old woman with splenectomy 4 years ago and the postoperative histopathology diagnosis revealed “splenic hemangioma” with spontaneous rupture. Two years after the operation, the patient's rechecked abdominal computed tomography (CT) showed multiple hepatic occupations. Diagnoses: Pathological test suggested PSA hepatic metastasis. Interventions: The patient was treated with trans-catheter arterial chemoembolization (TACE) and a pathological diagnosis of PSA was highly suspected in the hepatic biopsy. Four somatic alterations, phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha (PIK3CA), Fos proto-oncogene, AP-1 transcription factor subunit (FOS), MCL1 apoptosis regulator (MCL1), and phosphoinositide-3-kinase regulatory subunit 1 (PIK3R1) were detected in the tumor tissue using a Next generation sequencing (NGS) technology. The results prompted that the patient may get clinical benefit from using some agents for targeted therapy, Everolimus, Temsirolimus, or Copanlisib. Outcomes: The patient refused targeted therapy. As a result, the patient passed away within 51 months after splenectomy. Lessons: PSA is an aggressive disease that often presented with a high propensity for metastasis and rupture hemorrhage. Some of these mutations were first discovered in PSA and these findings added new contents to the genomic mutation profile of PSA.

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Sclerosing Angiomatoid Nodular Transformation of the spleen in a four-year-old with anemia

Delgado

Fleming

El-Gohary

et al. 2018

Journal of Pediatric Surgery Case Reports

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Splenic Angiosarcoma: A Diagnostic Splenectomy Finding

Cited by 7 publications

References 18 publications

Primary splenic angiosarcoma: a rare entity often associated with rupture and hemoperitoneum

Primary splenic angiosarcoma: a rare entity often associated with rupture and hemoperitoneum

A primary splenic angiosarcoma hepatic metastasis after splenectomy and its genomic alteration profile

Sclerosing Angiomatoid Nodular Transformation of the spleen in a four-year-old with anemia

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