Primary Splenic Angiosarcoma

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Abstract

Early diagnosis and prompt splenectomy prior to splenic rupture may be the best chance for surviving this rare, aggressive malignant neoplasm arising from splenic vascular endothelium.

Abstract

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 (10.5g/dL) with normal platelets (300 × 10 ⁹/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.

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Most cited references 14

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Angiosarcoma. A report of 67 patients and a review of the literature.

Rufus J. Mark, Joseph Poen, Luu Tran … (1996)

Angiosarcomas (AS) are rare, aggressive tumors. Optimal treatment has not been well defined. The authors undertook a retrospective review of patients seen at their institution with the intent of identifying prognostic factors and optimal treatment strategies. Between 1955 and 1990, 67 patients with AS were seen at the University of California, at Los Angeles Medical Center. Follow-up ranged from 1 to 173 months with a median of 30 months. The overall prognosis was poor. The actuarial 2- and 5-year disease free survivals (DFS) were 44% and 24%, respectively. Of 52 recurrences after primary treatment, 81% (42 of 52) had a component of local failure. Twenty-eight patients had developed distant metastases at last follow-up. Of patients who received surgery (S) and radiation therapy (RT), with or without chemotherapy (CT), 5-year actuarial DFS was 43%, compared with 17% for patients who underwent S +/- CT as initial treatment (P = 0.03). Only 9% of patients (1 of 11) treated with RT +/- CT were rendered free of disease. Patients with AS usually present with high grade histology, and with multifocal disease. There is a propensity for both local recurrence and distant metastases. Our results and a review of the literature, suggest that S plus RT offers the best chance for long term control of this aggressive tumor. The role of CT remains undefined.

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From the archives of the AFIP: primary vascular neoplasms of the spleen: radiologic-pathologic correlation.

Robert Abbott, Angela Levy, Nadine S I Aguilera … (2004)

Primary vascular neoplasms of the spleen constitute the majority of nonhematolymphoid splenic tumors. The benign primary vascular tumors include hemangioma, hamartoma, and lymphangioma, whereas those of variable or uncertain biologic behavior include littoral cell angioma, hemangioendothelioma, and hemangiopericytoma. The primary malignant vascular neoplasm of the spleen is angiosarcoma. Peliosis is a rare lesion of unknown cause that is usually found incidentally in asymptomatic patients but may be associated with hematologic or metastatic disease. Although these vascular neoplasms of the spleen are uncommon, their importance lies in that they must be differentiated from the more common neoplastic disorders of the spleen, such as lymphoma and metastasis. The most common echogenic solid or complex cystic mass in an asymptomatic patient is splenic hemangioma. However, the imaging appearance of splenic hemangiomas may be complex, and differentiation of these lesions from malignant disease may not be possible. The diagnosis of splenic hamartoma may be suggested when findings of increased blood flow on color Doppler images are seen in association with a homogeneous solid echogenic mass. A large subcapsular solitary cystic abnormality discovered incidentally in a child in association with internal septations and tiny mural nodules favors the diagnosis of lymphangioma. Any invasion of the surrounding splenic parenchyma by a splenic lesion should indicate a more aggressive or malignant process. Evaluation of a focal splenic abnormality identified on sonograms should be followed up with computed tomography or magnetic resonance imaging with and without contrast material enhancement. Splenectomy may be required for definitive evaluation of a splenic mass with atypical features.

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Primary angiosarcoma of the spleen. A clinicopathologic study of 40 cases.

Kim Krishnan, J Meis, Christine S. Falk (1993)

Forty primary splenic angiosarcomas occurring in 21 men and 19 women, 19-84 years old (median 59 years) are reported. Patients presented with splenomegaly (35 of 38, 92%), abdominal pain (33 of 40, 83%), and systemic symptoms such as fatigue (2 of 40, 5%), fever (4 of 40, 10%), and/or weight loss (16 of 40, 40%). Five (13%) experienced splenic rupture associated with hemoperitoneum. Abnormal laboratory findings included cytopenia (31 of 34, 91%), leukocytosis (8 of 21, 38%), and thrombocytosis (1/39, 3%). Most spleens weighed 500-1,000 g (mean, 1,180 g). The cut splenic surfaces showed multiple hemorrhagic nodules that were frequently associated with infarction, although some had a diffuse pattern of involvement. Microscopically, there were a variety of histologic patterns displayed by the vasoformative component. A honeycomb or sponge-like pattern was common in some, whereas others simulated a cavernous hemangioma or normal splenic sinuses (pseudosinusoidal pattern). Papillary endothelial tufts and solid proliferations of spindled to round to epithelioid cells were also seen. Factor VIII-related antigen was detected in 19 of 23 cases, BMA-120 in 18 of 23, UEA-1 receptor in 18 of 23, and vimentin in 23 of 23 as well as CD68 antigen in 1 of 23 cases. S-100 protein and cytokeratin were not found in any of the 23 cases studied. Metastases in 22 of 32 patients (69%) were to the liver (13 patients), bone or bone marrow (7 patients), lymph nodes (1 patient), and brain (1 patient). Three patients had concomitant malignancies and one had a prior history of a mixed B-cell lymphoma 5 years previously that had been treated with chemotherapy. Follow-up in 38 patients revealed that 30 (79%) are dead at a median interval of 6 months (range 0-48 months) and 8 are alive 5-21 months after diagnosis. These findings indicate that splenic angiosarcoma is an aggressive neoplasm with a high metastatic rate and an abysmal prognosis. Recognition of the wide range of histologic patterns is of diagnostic value but no apparent prognostic significance.

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Journal

Journal ID (nlm-ta): JSLS

Journal ID (iso-abbrev): JSLS

Journal ID (hwp): jsls

Journal ID (pmc): jsls

Journal ID (publisher-id): JSLS

Title: JSLS : Journal of the Society of Laparoendoscopic Surgeons

Publisher: Society of Laparoendoscopic Surgeons (Miami, FL )

ISSN (Print): 1086-8089

ISSN (Electronic): 1938-3797

Publication date (Print): Jul-Sep 2010

Volume: 14

Issue: 3

Pages: 431-435

Affiliations

Department of Surgery, Scott and White Memorial Hospital; Scott, Sherwood and Brindley Foundation; The Texas A&M University Health Science Center; Temple, Texas, USA.

Author notes

Address correspondence to: Kamran S. Hamid, MD, Department of Surgery, Scott and White Memorial Hospital, 2401 South 31st Street, Temple, Texas 76508, USA. Telephone: (254) 724-2760, Fax: (254) 724-4796, E-mail: kshamid@ 123456medicine.tamhsc.edu

Article

Publisher ID: 09-07-099

DOI: 10.4293/108680810X12924466006521

PMC ID: 3041046

PubMed ID: 21333203

SO-VID: 28273e38-51df-49be-ab97-403fbc9b23fa

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This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivatives License ( http://creativecommons.org/licenses/by-nc-nd/3.0/), which permits for noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited and is not altered in any way.

Primary Splenic Angiosarcoma

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Most cited references 14

Angiosarcoma. A report of 67 patients and a review of the literature.

From the archives of the AFIP: primary vascular neoplasms of the spleen: radiologic-pathologic correlation.

Primary angiosarcoma of the spleen. A clinicopathologic study of 40 cases.

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