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

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.

[1]  V. Artiko,et al.  Littoral-cell angioma as a rare cause of splenomegaly. , 2006, Clinical and laboratory haematology.

[2]  A. Levy,et al.  Littoral cell angioma of the spleen: CT features with clinicopathologic comparison. , 2004, Radiology.

[3]  B. Lucey,et al.  Percutaneous nonvascular splenic intervention: a 10-year review. , 2002, AJR. American journal of roentgenology.

[4]  N. Loberant,et al.  Littoral cell angioma of the spleen: Appearance on sonography and CT , 2002, Journal of clinical ultrasound : JCU.

[5]  E. Vlodavsky,et al.  Splenic littoral cell haemangioendothelioma: a new low‐grade variant of malignant littoral cell tumour , 2001, Histopathology.

[6]  K. Mortelé Imaging of tumoral conditions of the spleen. , 2000, JBR-BTR : organe de la Societe royale belge de radiologie (SRBR) = orgaan van de Koninklijke Belgische Vereniging voor Radiologie.

[7]  Tadashi Shimizu,et al.  Color Doppler sonographic findings in splenic hamartoma , 2000, Journal of clinical ultrasound : JCU.

[8]  A. Supe,et al.  Isolated massive splenic peliosis. , 2000, Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology.

[9]  S. Hai,et al.  Primary splenic angiosarcoma: case report and literature review. , 2000, Journal of the National Medical Association.

[10]  J. Yee,et al.  Littoral cell angioma of the spleen: imaging features. , 2000, AJR. American journal of roentgenology.

[11]  Diébold,et al.  Isolated diffuse hemangiomatosis of the spleen with Kasabach–Merritt‐like syndrome , 1999, Histopathology.

[12]  H. Naganuma,et al.  Splenic lymphangioma: US and CT diagnosis and clinical manifestations , 1999, Abdominal Imaging.

[13]  W. Peh,et al.  Splenic vascular lesions: unusual features and a review of the literature. , 1999, The Australian and New Zealand journal of surgery.

[14]  T. Kinoshita,et al.  Splenic hemangiosarcoma with massive calcification , 1999, Abdominal Imaging.

[15]  D. Bova,et al.  Hemangioendothelioma of the spleen: imaging findings at color Doppler, US, and CT. , 1999, Clinical imaging.

[16]  M. Martín-González,et al.  Littoral cell angioma of the spleen: US and MR imaging findings , 1998, Abdominal Imaging.

[17]  M. Bisceglia,et al.  Littoral Cell Angioma of the Spleen: An Additional Report of Four Cases with Emphasis on the Association with Visceral Organ Cancers , 1998, Tumori.

[18]  H. Fischer,et al.  [Littoral cell angiosarcoma of the spleen. Morphologic, immunohistochemical findings and consideration of histogenesis of a rare splenic tumor]. , 1997, Der Pathologe.

[19]  P. Hollett,et al.  Radiological features of a symptomatic splenic hamartoma , 1996, Pediatric Radiology.

[20]  G. Garlaschi,et al.  CT findings in primary vascular tumors of the spleen. , 1996, AJR. American journal of roentgenology.

[21]  R. Warnke Tumors of the Lymph Nodes and Spleen , 1996 .

[22]  W. Schwerk,et al.  Color Doppler imaging of focal splenic masses. , 1994, European journal of radiology.

[23]  R. Edelman,et al.  Hepatic hemangiomas: a multi-institutional study of appearance on T2-weighted and serial gadolinium-enhanced gradient-echo MR images. , 1994, Radiology.

[24]  M. Ikezu,et al.  Splenic hemangiosarcoma with sinus endothelial differentiation , 1993, Acta pathologica japonica.

[25]  J. Krishnan,et al.  Primary Angiosarcoma of the Spleen A Clinicopathologic Study of 40 Cases , 1993, The American journal of surgical pathology.

[26]  M. Kudo,et al.  Multiple hemangiopericytomas of the spleen. , 1992, The American journal of gastroenterology.

[27]  R. Semelka,et al.  Spleen: dynamic enhancement patterns on gradient-echo MR images enhanced with gadopentetate dimeglumine. , 1992, Radiology.

[28]  A. Esparza,et al.  Hemangioendothelioma of the spleen. , 1992, Archives of pathology & laboratory medicine.

[29]  L. Morgenstern,et al.  The clinical spectrum of lymphangiomas and lymphangiomatosis of the spleen. , 1992, The American surgeon.

[30]  S. Suster Epithelioid and spindle-cell hemangioendothelioma of the spleen. Report of a distinctive splenic vascular neoplasm of childhood. , 1992, The American journal of surgical pathology.

[31]  G. Dodd,et al.  Epithelioid hemangioendothelioma of the liver: imaging findings with pathologic correlation. , 1992, AJR. American journal of roentgenology.

[32]  M. Lishner,et al.  Case report: unusual presentation of splenic hamartoma; computed tomography and ultrasonic findings. , 1992, Clinical radiology.

[33]  K. Ohtomo,et al.  CT and MR appearances of splenic hamartoma. , 1992, Journal of computer assisted tomography.

[34]  H. Honda,et al.  MRI of primary spleen angiosarcoma with iron accumulation. , 1992, Journal of computer assisted tomography.

[35]  N. Harris,et al.  Splenic hamartoma and capillary hemangioma are distinct entities: immunohistochemical analysis of CD8 expression by endothelial cells. , 1991, Human pathology.

[36]  G. Frizzera,et al.  Littoral Cell Angioma: A Novel Splenic Vascular Lesion Demonstrating Histiocytic Differentiation , 1991, The American journal of surgical pathology.

[37]  J. J. Steinberg,et al.  The spleen in the spleen syndrome: The association of splenoma with hematopoietic and neoplastic disease—compendium of cases since 1864 , 1991, Journal of surgical oncology.

[38]  Á. L. Jiménez,et al.  Hemangiopericytoma of the spleen. , 1989 .

[39]  H. Morehouse,et al.  Isodense splenic mass: hamartoma, a case report. , 1989, Computerized medical imaging and graphics : the official journal of the Computerized Medical Imaging Society.

[40]  R. Sonnino,et al.  Splenic cysts: aspiration, sclerosis, or resection. , 1989, Journal of pediatric surgery.

[41]  H. Stutte,et al.  Hamartomas of the spleen: a study of 20 biopsy cases , 1989, Histopathology.

[42]  S. Allegra,et al.  An immunohistologic study of a splenic cyst. , 1987, Military medicine.

[43]  D. Catovsky,et al.  Splenic angiosarcoma following chemotherapy for follicular lymphoma. , 1986, Human pathology.

[44]  Y. Tsutsumi,et al.  SPLENIC HAMARTOMA , 1986, Acta pathologica japonica.

[45]  M. Joly,et al.  Splenic hamartoma, vascular type, with endothelial proliferation. , 1983, Pathology, research and practice.

[46]  H. Kishimoto,et al.  Peliosis of the spleen. , 1983, American journal of clinical pathology.

[47]  Z. Feigenberg,et al.  Malignant lymphangioma of the spleen. , 1983, Israel journal of medical sciences.

[48]  S. Weiss,et al.  Epithelioid hemangioendothelioma a vascular tumor often mistaken for a carcinoma , 1982, Cancer.

[49]  B. Scheithauer,et al.  Primary nonlymphoreticular malignant neoplasms of the spleen , 1982, The American journal of surgical pathology.

[50]  J. AuBuchon,et al.  Cystic lymphangiomatosis of the spleen: a radiologic-pathologic correlation. , 1981, Radiology.

[51]  L. Axel,et al.  Dynamic CT of the normal spleen. , 1981, AJR. American journal of roentgenology.

[52]  E. Williams,et al.  CT Diagnosis of Splenic Cystic Lymphangiomatosis , 1981 .

[53]  R. Reddick,et al.  Hemangiopericytoma: A light microscopic and ultrastructural study , 1981, Cancer.

[54]  Avery A. Brinkley,et al.  Cystic hamartoma of the spleen: Ct and sonographic findings , 1981, Journal of clinical ultrasound : JCU.

[55]  R. Iozzo,et al.  Symptomatic Splenic Hamartoma: A Report of Two Cases and Review of the Literature , 1980, Pediatrics.

[56]  K. Chan,et al.  Distinctive, multiple lymphangiomas of spleen , 1980, The Journal of pathology.

[57]  H. Pitluk,et al.  Hemangiopericytoma. Literature review and clinical presentations. , 1979, American journal of surgery.

[58]  Chen Kt,et al.  Angiosarcoma of the spleen: a report of two cases and review of the literature. , 1979, Archives of pathology & laboratory medicine.

[59]  D. Benjamin,et al.  A fatal case of peliosis of the liver and spleen. , 1978, American journal of diseases of children.

[60]  K. Matsuura,et al.  Angiographic diagnosis of benign and malignant splenic tumors. , 1978, AJR. American journal of roentgenology.

[61]  W. Dietz,et al.  Splenic consumptive coagulopathy in a patient with disseminated lymphangiomatosis. , 1977, The Journal of pediatrics.

[62]  M. Frand,et al.  Lymphangiomatosis with splenic involvement. , 1976, JAMA.

[63]  S. Komaki,et al.  Angiographic demonstration of a calcified splenic hamartoma. , 1976, Radiology.

[64]  G. Bevilacqua,et al.  A Case of Cavernous Haemangioma of the Spleen , 1976, Tumori.

[65]  J. W. Darden,et al.  Hamartoma of the spleen: a manfestation of tuberous sclerosis. , 1975, The American surgeon.

[66]  S. Weitzner,et al.  Hemangiosarcoma of spleen with spontaneous rupture , 1975, Cancer.

[67]  A. Louvel,et al.  [Hamartomas of the spleen]. , 1974, Journal de chirurgie.

[68]  Cohen Ah,et al.  Hepatic and splenic lymphangiomatosis with skeletal involvement: report of a case and review of the literature. , 1974 .

[69]  R. Deterling,et al.  Intra-abdominal hemangiopericytoma. Report of four cases and review of the literature. , 1973, Archives of surgery.

[70]  Foote Fw,et al.  Primary splenic angiosarcoma. A case report. , 1968 .

[71]  E. Husni The clinical course of splenic hemangioma with emphasis on spontaneous rupture. , 1961, Archives of surgery.

[72]  A. Stout,et al.  HEMANGIOPERICYTOMA: A VASCULAR TUMOR FEATURING ZIMMERMANN'S PERICYTES. , 1942, Annals of surgery.

[73]  C. Cañas,et al.  Hepatosplenic AIDS-Related kaposi's sarcoma , 2005, Gastrointestinal Radiology.

[74]  K. Ito,et al.  Cystic lymphangioma of the spleen: MR findings with pathologic correlation , 2004, Abdominal Imaging.

[75]  M. Uder,et al.  Littoral cell angioma of the spleen: CT and MR imaging appearance , 2000, European Radiology.

[76]  A. Capelastegui,et al.  Atypical MRI presentation of a small splenic hamartoma , 1999, European Radiology.

[77]  K. Sugimura,et al.  CT and MR findings of splenic angiosarcoma. , 1999, Radiation medicine.

[78]  R. Semelka,et al.  Splenic hemangiomas and hamartomas: MR imaging characteristics of 28 lesions. , 1997, Radiology.

[79]  R. Lorenzo,et al.  Splenic lymphangiomatosis in children. , 1997, Radiology.

[80]  P. Mergo,et al.  Cystic masses of the spleen: radiologic-pathologic correlation. , 1996, Radiographics : a review publication of the Radiological Society of North America, Inc.

[81]  G. Kanel,et al.  Peliosis hepatis in a patient with human immunodeficiency virus infection. , 1991, AJR. American journal of roentgenology.

[82]  M. De Coster,et al.  Angiosarcoma of the spleen. Case report and review of literature. , 1989, Acta chirurgica Belgica.

[83]  F. Pistoia,et al.  Splenic lymphangiomatosis: CT diagnosis. , 1988, AJR. American journal of roentgenology.

[84]  A. Dachman,et al.  Hemangioma of the spleen: radiologic-pathologic correlation in ten cases. , 1987, Radiology.

[85]  L. Weiss,et al.  Histology : cell and tissue biology , 1983 .

[86]  E. Williams,et al.  Case report. CT diagnosis of splenic cystic lymphangiomatosis. , 1981, Journal of computer assisted tomography.

[87]  Garvin Df,et al.  Cysts and nonlymphomatous tumors of the spleen. , 1981 .

[88]  D. Garvin,et al.  Cysts and nonlymphomatous tumors of the spleen. , 1981, Pathology annual.

[89]  E. Gilbert,et al.  Angiosarcoma of the spleen: a report of two cases and review of the literature. , 1979, Archives of pathology & laboratory medicine.

[90]  R. J. Tuttle,et al.  Splenic cystic lymphangiomatosis: an unusual cause of massive splenomegaly. , 1978, Radiology.

[91]  B. H. Smith,et al.  Hemangiopericytoma. An analysis of 106 cases. , 1976, Human pathology.

[92]  M. Asch,et al.  Hepatic and splenic lymphangiomatosis with skeletal involvement: report of a case and review of the literature. , 1974, Surgery.

[93]  Pillai Pg,et al.  Cystic lymphangioma of the spleen - a case report. , 1974 .

[94]  N. Devi,et al.  Cystic lymphangioma of the spleen - a case report. , 1974, Indian journal of pathology & bacteriology.

[95]  A. P. Krasovskii,et al.  [Hemangioma of the spleen]. , 1971, Klinicheskaia khirurgiia.

[96]  F. W. Foote,et al.  Primary splenic angiosarcoma. A case report. , 1968, Archives of pathology.

[97]  D. Sumner,et al.  A UNIFIED CONCEPT OF LYMPHANGIOMA AND CYSTIC HYGROMA. , 1965, Surgery, gynecology & obstetrics.

[98]  F. Barner [Peliosis in spleen]. , 1952, Zentralblatt fur allgemeine Pathologie u. pathologische Anatomie.

[99]  P. Hollett,et al.  Price Radiological features of a symptomatic splenic hamartoma , 2022 .