Primary Splenic Angiosarcoma Research Articles

Primary Splenic Angiosarcoma Revealed by Bone Marrow Metastasis

Primary splenic angiosarcomas are the most common malignant non-hematopoietic tumors of the spleen. Metastatic diseases were found in 69% of patients in a reported series but the incidence of bone marrow involvement is unclear. We report a rare case of a 25-years-old Moroccan woman with unsuspected primary splenic angiosarcoma revealed by bone marrow metastasis. She presented with serious anemia and splenomegaly. Bone marrow biopsy revealed proliferating spindle cells. Computed tomography scanning showed an enlarged spleen with heterogeneous lesions. Splenectomy was performed and retrospective histological study of the spleen confirmed the diagnosis. She died 1 year after splenectomy.

Nonneoplastic, benign, and malignant splenic diseases: cross-sectional imaging findings and rare disease entities.

Splenic lesions are commonly encountered and are often incidental in nature. Benign splenic vascular neoplasms include hemangioma, hamartoma, lymphangioma, extra-medullary hematopoiesis (EMH), and sclerosing angiomatoid nodular transformation (SANT). Uncommonly encountered entities of the spleen include focal EMH, focal myeloma, angiomyolipoma, and SANT. Primary splenic angiosarcoma is the most common malignant nonhematolymphoid malignancy of the spleen. Lymphoma, myeloma, and metastases are the other malignant entities involving the spleen. The clinical presentation, key imaging findings, and associations of benign, neoplastic, and malignant diseases that can involve the spleen will be discussed. Radiologists can use multimodality imaging to diagnose entities involving the spleen by recognizing key imaging features and considering patient characteristics. However, biopsy may be warranted for definitive diagnosis when imaging findings are nonspecific.

Primary angiosarcoma of the spleen: an oncological enigma.

Introduction. Primary splenic angiosarcoma is an extremely unusual neoplasm originating from sinusoidal vascular endothelium. Surgical extirpation is the mainstay of treatment of this highly malignant disease. Case Presentation. An 82-year-old woman was admitted with left pleural effusion and a palpable left upper quadrant abdominal mass, secondary to splenomegaly by two large splenic tumors. Classic open splenectomy was performed and angiosarcoma of the spleen was the final histopathological diagnosis, which was primary since no other disease site was revealed. Discussion. The incidence of the disease is 0.14–0.23 cases per million, with slight male predominance. Etiology is not established and clinical presentation may confuse even experienced physicians. Imaging modalities cannot differentiate the lesion from other vascular splenic neoplasms and the correct diagnosis is mainly set after histopathological examination of the resected spleen. As with other sarcomas, surgery is the only curative approach, while chemo- and radiotherapy have poor results. Prognosis remains dismal.

Angiosarcoma of the Spleen with Spontaneous Rupture

Primary splenic angiosarcoma is an extremely rare solid tumour. Splenic angiosarcoma is a highly aggressive malignancy associated with early metastases and poor prognosis. Most patients present with abdominal pain or a palpable abdominal mass. Spontaneous splenic rupture with fatal haemorrhage is a fairly common presentation and is reported in the literature in up to 30% of patients. This report is of a patient with spontaneous splenic rupture due to splenic angiosarcoma.

Spontaneous rupture of primary splenic angiosarcoma: a case report and literature review

BackgroundPrimary angiosarcoma of the spleen is a rare mesenchymal malignant tumor of vascular origin often with a poor prognosis, due to its high metastatic potential. This disease often presents with atraumatic rupture and lethal hemorrhage.Case presentationWe report a case of a 65-year-old man who presented with abdominal pain, anemia, thrombocytopenia, and palpable abdominal mass with unstable blood pressure. Laparotomy revealed a huge actively bleeding spleen, thus splenectomy was performed. Some liver metastasis foci were also found during the procedure. Histopathology diagnosis of the removed spleen was primary splenic angiosarcoma. The patient was discharged on the 10th day post operation with no complication.ConclusionsSplenic angiosarcoma should be considered one of the differential diagnoses in patients with spleen parenchymal lesions. Definitive diagnosis requires laparotomy followed by splenectomy. In the majority of the patients with spleen angiosarcoma, metastatic diseases have already occurred at the time of laparotomy, so splenectomy is an approach more for diagnostic purpose rather than curative purpose.

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

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.

Systemic therapy in primary angiosarcoma of the spleen

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.

Primary splenic angiosarcoma in a 2.5-year-old boy with hepatic metastasis

Primary splenic angiosarcoma in children is extremely rare and has a very poor prognosis. We reported a 2.5-year-old boy who had this rare entity and hepatic metastasis. The patient presented with left upper quadrant abdominal mass and anemia. The patient received multidisciplinary treatment and died 32 months after splenectomy.

Transcatheter arterial embolizationが腹腔内出血の止血に有効であった脾原発血管肉腫の1例

Transcatheter arterial embolizationが腹腔内出血の止血に有効であった脾原発血管肉腫の1例

Primary Splenic Angiosarcoma

Primary splenic angiosarcoma is a rare, aggressive malignant neoplasm arising from splenic vascular endothelium. A 70-year-old woman presented with shortness of breath and chest discomfort secondary to a left-sided pleural effusion. A thoracentesis revealed a reactive effusion suspicious for malignancy. Splenic enlargement with heterogeneous enhancement was identified on CT of the abdomen. Laboratory findings at initial presentation revealed mild anemia (10.5 g/dL) with normal platelets (300 × 10⁹/L). Laparoscopic splenectomy was performed, and a primary splenic angiosarcoma was discovered. After 2 rounds of chemotherapy, a CT scan showed progressive disease with metastasis to the liver and lung. The patient's antineoplastic regimen was switched to Ifosfamide and Doxorubicin. She is currently alive with evidence of disease at 9 months but without further progression. Primary splenic angiosarcoma is almost universally fatal despite treatment. The best chance for survival is early diagnosis and prompt splenectomy prior to splenic rupture.

Odontogenic myxofibroma synchronous with primary angiosarcoma of the spleen

Odontogenic myxofibroma of the temporomandibular joint (TMJ) is a rare tumour; moreover, primary splenic angiosarcoma (PAS) in paediatric patients is extremely rare. We report on a 15-year-old boy who presented with right TMJ swelling and subsequently developed epigastric and right upper quadrant pain. The TMJ swelling proved to be odontogenic myxofibroma and the abdominal pain was a result of primary splenic angiosarcoma with hepatic metastasis. We report for the first time the synchronous presentation of PAS and odontogenic myxofibroma in a paediatric patient, and we describe the radiological features along with the histological diagnosis and clinical outcome. Uptake in (18)F-2-fluoro-2-deoxy-D-glucose positron emission tomography is also described for the first time for both these tumours.

Thrombocytopenia as first manifestation of splenic angiosarcoma

Dear Editor, Angiosarcoma is a rare mesenchymal neoplasia with an incidence of only 0.14–0.25 cases per million [1]. Most common sites of occurrence are skin and superficial soft tissues, followed by breast, liver, spleen, and bone. Early stage metastases to lung, liver, and lymph nodes are common and overall prognosis is poor [2, 3]. Only approx. 5% of angiosarcomas originate from the spleen, and the existing casuistic reports suggest a dismal clinical prognosis. Here, we describe a rare case of splenic angiosarcoma leading to thrombocytopenia as first manifestation of the disease. A 64-year-old woman presented as hematologic outpatient inMarch 2008 with isolated thrombocytopenia (58/nl) and chronic, slowly progressive fatigue. Thrombocytopenia was first noted in 2006. Pre-existing medical conditions and physical examination were unremarkable. The remaining full blood count was without pathological findings (hemoglobin 12.2 g/dl, normal erythrocyte indices, leukocytes 6.9/nl with normal differential count). Differential diagnosis for moderate thrombocytopenia included various causes for immune destruction of platelets, impaired thrombocyte production, and hypersplenism. However, laboratory screening tests for lymphoma, autoimmune diseases, and virus infections resulted negative. A bone marrow aspirate gave an unspecific picture with unremarkable cellular properties, normal count of megakaryocytes but diffuse infiltration of mature plasma cells up to 15%. A bone marrow biopsy showed hyperplastic left shifted granulopoiesis, left shifted megakaryopoiesis, and erythropoiesis in a hypercellular tissue, partially accompanied with B-cell-rich non-monoclonal lymphocytic aggregates. An abdominal ultrasound showed massive splenomegaly with multiple highly echogenic circular lesions. Multislice computed tomography (MSCT) confirmed splenomegaly (measuring 21 cm craniocaudal) and revealed multiple hypodense lesions with central hyperdense structures (Fig. 1a). In addition, multiple lytic bone lesions in the vertebral column were present. MSCT-guided biopsy of these lytic bone lesions showed infiltrates of a mesenchymal neoplasia containing atypic mitotic bodies, a proliferation fraction of 40% (MIB-1) and negativity for pancytokeratin and S 100. Immunohistochemistry stained positive for vimentin, CD31, CD34, and factor VIII, but negative for desmin, actin, and HHV8, leading to the diagnosis of angiosarcoma (Fig. 2). Meanwhile, the patient suffered from symptomatic splenomegaly (abdominal pain, symptoms of GI tract displacement), and since splenic angiosarcoma may be complicated by splenic rupture [4, 5], an elective splenectomy was performed. Histological examination confirmed the diagnosis of primary splenic angiosarcoma. In addition, the patient was offered palliative chemotherapy and infusions of bisphosphonates; the latter were started immediately. Chemotherapy with paclitaxel (90 mg/m weekly) began with a delay of several weeks (patient’s choice) at a S. Raffel :B. Hildebrandt : I. Sturm (*) Department of Hematology and Oncology, Charite Campus Virchow Klinikum, Augustenburger Platz 1, 13353 Berlin, Germany e-mail: isrid.sturm@charite.de

Spontaneous Rupture of Primary Angiosarcoma of the Spleen

Primary angiosarcoma of the spleen is an extremely rare malignancy, the pathogenesis of which is not completely understood, with high metastatic potential and an exceedingly poor prognosis, regardless of treatment regimen. The major complication is splenic rupture, which often leads to fatal hemoperitoneum. Overall, since 1879 when Langerhans described the first case of angiosarcoma of the spleen, there have been approximately 200 cases reported in the literature. Moreover, to the best of our knowledge, spontaneous rupture of primary splenic angiosarcoma and spontaneous rupture of remnant or recurred angiosarcoma is extremely rare, and no cases were reported in English literature. We report a case of spontaneous splenic rupture due to angiosarcoma in a 68-year-old man, and also review the existing literature.

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

IntroductionPrimary splenic angiosarcoma is a rare neoplasm of vascular origin carrying a very poor prognosis, partly due to its high metastatic potential. This disease presents frequently with splenic rupture and hemorrhage. We report the case of a 17-year-old woman who presented with rupture of a primary splenic angiosarcoma.Case presentationThe patient presented with diffuse abdominal pain and distention. Clinical examination revealed severe tenderness in the left upper abdominal quadrant, a palpable abdominal mass, and hemodynamic instability with a systolic arterial blood pressure of 75 mmHg and heart rate of 135 beats per minute. Blood tests revealed anemia (hemoglobin 7.0 g/dl) and thrombocytopenia (platelets 70 × 109/liter). After initial fluid resuscitation and stabilization, abdominal ultrasound and computed tomography were performed, revealing a large quantity of intraperitoneal free fluid, an enlarged spleen, and a heterogeneous low-density signal within the splenic parenchyma, which showed varying degrees of contrast enhancement. At laparotomy a huge (weight 1530 g, diameter 19 cm) actively bleeding spleen was identified and splenectomy was performed. Histopathology showed a primary splenic angiosarcoma. After an uneventful recovery, the patient was discharged on the sixth postoperative day.ConclusionPrimary splenic angiosarcoma is rare. Although this malignancy is usually encountered in advanced age, there have been a few reported cases among younger patients. The case reported here presented with splenic rupture, was treated by laparotomy and splenectomy, and the patient is disease free 16 months after surgery.

Primary angiosarcoma of the spleen

Primary splenic angiosarcoma is a very rare and aggressive neoplasm with a high metastatic rate and dismal prognosis. Since only a few cases have been adequately reported in the medical literature, we report here a further six cases. The records of all cases of primary splenic angiosarcoma treated at Chang Gung Memorial Hospital from April 1991 to July 2004 were retrospectively reviewed. Of the six cases identified (three men and three women; range, 7-69 years; median, 44 years), four presented with palpable abdominal masses or left upper quadrant abdominal pain. Other systemic symptoms, such as bleeding gums, fatigue, fever, body weight loss, and gastrointestinal bleeding were noted. An abnormal hematogram was found in five patients, with all of these five showing anemia, and three thrombocytopenia. Splenomegaly was present in all patients. Hemoperitoneum due to splenic rupture was noted in one patient. Three patients had distant metastasis to the liver (n = 2), bone (n = 1), bone marrow (n = 1), and small bowel (n = 1) at diagnosis. Liver (n = 3), bone (n = 1), and bone marrow (n = 1) metastases were found in four patients after initial therapy. Five of the six cases underwent a splenectomy, one underwent partial hepatectomy, and one received chemotherapy. The respective 1-, 3- and 5-year survival rates were 60%, 40%, and 40%, and the median survival time was 36 months. One patient was disease-free 162 months after splenectomy. The clinical presentations of splenic angiosarcoma were similar to those of previous reports apart from the higher rate of splenomegaly observed in this study. In contrast to reported pediatric cases, our patient achieved long-term disease-free survival after splenectomy alone.

Splenic angiosarcoma metastasis to small bowel presented with gastrointestinal bleeding

Primary splenic angiosarcoma is a very rare, aggressive neoplasm with a high metastatic rate and dismal prognosis. This neoplasm usually presents with abdominal pain, splenomegaly, anemia, and thrombocytopenia. Splenic angiosarcoma with bleeding gastrointestinal metastases is extremely rare. The literature contains only two case reports. This study reported a 44-year-old male patient with splenic angiosarcoma with sustained repeated gastrointestinal bleeding due to small bowel metastases. Salvage surgery was performed by splenectomy and resection of the metastatic small bowel tumors. The post-operative course was uneventful; the patient survived with the disease and had no GI bleeding, 7 mo after surgery.

Ｒｅｃｏｍｂｉｎａｎｔ ｉｎｔｅｒｌｅｕｋｉｎ‐２療法が有効であったひ原発血管肉腫の１例

脾原発血管肉腫はまれな疾患で極めて予後不良である. 今回, われわれは脾原発血管肉腫の切除後に転移巣に対しrecombinant interleukin-2 (rIL-2) を投与したところ, 肝転移巣および転移リンパ節において奏功を認めることができ, rIL-2の有用性が伺われた. 症例は52歳の女性で, 2002年9月, 巨大な脾腫瘍に対し脾摘術を行い血管肉腫の診断を得た. 術中多発肝転移を認めたため, これに対しrIL-2の肝動注を行ったところ転移巣は著明に縮小した. 2003年3月, 肝十二指腸間膜リンパ節に転移を認め, rIL-2の持続静注療法を行ったところ転移巣は著明に縮小した. 2003年5月, 脳転移と思われる病巣が出現し脳外科にて摘出手術を行ったが切除標本からは血管肉腫の所見は得られず転移とは断定できなかった. 術後, 肝転移巣と副腎転移巣が増大したが, 副作用のためrIL-2療法が続けられず, 2003年9月死亡した.

Splenic Angiosarcoma and Iron Deficiency Anemia in a 43-Year-Old Man

Thrombocytopenia and microcytic anemia are two laboratory findings that alone or together suggest an underlying disease process. Both are found throughout particular age groups and have broad differential diagnoses. Angiosarcomas are rare neoplasms from the lining of blood vessels. Primary splenic angiosarcoma is an even rarer neoplasm, first reported in the late 1870s. We report a case of primary splenic angiosarcoma in a patient with thrombocytopenia, microcytic anemia, and splenomegaly.

Angiosarcoma: unusual cause of acute abdominal pain and hemoperitoneum

Spontaneous rupture of primary angiosarcoma of the spleen is an uncommon cause of acute abdominal pain and hemoperitoneum. Clinical findings and presentation are nonspecific and variable. CT is frequently used as a first-line imaging modality in the evaluation of acute abdominal pain; however, findings are not specific for angiosarcoma. We report a case of primary splenic angiosarcoma with spontaneous rupture, hemoperitoneum, and hepatic metastases in a patient presenting to the emergency department with acute left upper quadrant pain and fever.

Splenic Angiosarcoma: A Clinicopathologic and Immunophenotypic Study of 28 Cases

Primary angiosarcoma of the spleen is a rare neoplasm that has not been well characterized. We describe the clinical, morphologic, and immunophenotypic findings of 28 cases of primary splenic angiosarcoma, including one case that shares features of lymphangioma/lymphangiosarcoma. The patients included 16 men and 12 women, aged 29 to 85 years, with a mean of 59 years and median of 63 years. The majority of patients (75%) complained of abdominal pain, and 25% presented with splenic rupture. The most common physical finding was splenomegaly (71%). Seventeen of 21 patients were reported to have anemia. Macroscopic examination showed splenomegaly in 85% cases. Sectioning revealed discrete lesions in 88% of cases, ranging from well-circumscribed firm nodules to poorly delineated foci of necrosis and hemorrhage associated with cystic spaces. Microscopically, the tumors were heterogenous; however, all cases demonstrated at least a focal vasoformative component lined by atypical endothelial cells. Solid sarcomatous, papillary, and epithelioid growth patterns were observed. The solid sarcomatous component resembled fibrosarcoma in two cases and malignant fibrous-histiocytoma in one case. Hemorrhage, necrosis, hemosiderin, extramedullary hematopoiesis, and intracytoplasmic hyaline globules were frequently identified. A panel of immunohistochemical studies revealed that the majority of tumors were immunoreactive for at least two markers of vascular differentiation (CD34, FVIIIRAg, VEGFR3, and CD31) and at least one marker of histiocytic differentiation (CD68 and/or lysozyme). Metastases developed in 100% of patients during the course of their disease. Twenty-six patients died of disease despite aggressive therapy, whereas only two patients are alive at last follow-up, one with disease at 8 years and the other without disease at 10 years. In conclusion, primary splenic angiosarcoma is an extremely aggressive neoplasm that is almost universally fatal. The majority of splenic angiosarcomas coexpress histiocytic and endothelial markers by immunohistochemical analysis, which suggest that some tumors may originate from splenic lining cells.