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At least 19 recordsLinked to original sources
PubMed1993

[Splenic neoplasms].

Carcinomas of the spleen are rare, whether primary or secondary. Two patients are described, one with a primary angiosarcoma. In this patient the first symptom was abdominal pain, caused by spontaneous rupture of the spleen. The other case was a woman with carcinoma of the breast which metastasized to the spleen. Both underwent splenectomy and adjuvant oncological treatment. Angiosarcomas constitute less than 1% of all sarcomas. Only about 60 cases of angiosarcomas in the spleen have been reported in the world literature. Spontaneous rupture of the spleen occurred in 30% of these cases. The prognosis for both untreated and treated splenic angiosarcomas is poor. Virtually all malignant tumours have been shown to metastasize to the spleen, most frequently from primary tumours localised to the breast, lung or ovary. Neoplasm should be considered for patients with splenomegaly having no apparent cause.

Adult↗
PubMed1988

A clinicopathologically distinctive primary splenic histiocytic neoplasm. Demonstration of its histiocyte derivation by immunophenotypic and molecular genetic analysis.

We describe a primary splenic neoplasm composed of cytomorphologically malignant-appearing erythrophagocytic histiocytoid cells reminiscent of those seen in malignant histiocytosis. However, this neoplasm displayed certain distinctive clinicopathologic features--including localization to the spleen, where it grew as separate discrete nodules--that distinguish it from all previously reported cases of malignant histiocytosis. The cells expressed a monocyte/histiocyte immunophenotype and lacked clonal immunoglobulin and T-cell receptor beta-chain gene rearrangements. Our results suggest that this neoplasm represents a clinicopathologically distinctive and possibly unique tumor derived from the tissue macrophage lineage.

Adult↗
PubMed2003

Marginal zone-related neoplasms of splenic and nodal origin.

BACKGROUND: The marginal zone is an anatomically distinct B-cell compartment of lymphoid tissue with an abundant antigenic influx. Among marginal zone-derived lymphomas the WHO classification listed, in addition to extranodal marginal zone B-cell lymphoma of MALT type, two other marginal zone B-cell neoplasms: splenic marginal zone B-cell lymphoma (+/- villous lymphocytes) and nodal marginal zone B-cell lymphoma (+/- monocytoid B cells). These two entities are well characterized histologically, but specific biological markers are lacking. Treatment options are heterogeneous, including a watch-and-wait policy, surgery with or without chemotherapy, purine analogs, and interferon. No prospective studies have been conducted so far. INFORMATION SOURCES: Clinical and pathologic data were reviewed by searches of the published medical literature, including searches in PubMed , important printed publications, and abstracts presented at recent hematology and pathology meetings. STATE OF THE ART: Splenic and nodal marginal zone lymphomas are typical low-grade lymphomas with an indolent course. A subset of patients, however, presents with more aggressive disease and have a shorter survival. Clinical and biological prognostic factors identified in reported series are heterogeneous. The role played by hepatitis C virus (HCV) in marginal zone lymphomas is not fully elucidated, but there is demonstration that eradication of HCV infection in splenic lymphoma with villous lymphocytes causes regression of the lymphoma. The optimal treatment has not yet been identified. Retrospective series, however, show that splenectomy is a good option if symptoms from the presence of spleen enlargement or cytopenias need to be treated. The utility of purine analogs and of anti-CD20 immunotherapy needs to be clarified in prospective trials. PERSPECTIVES: Clinicians and pathologists should co-operate to define stringent diagnostic criteria for these indolent disorders. The optimal therapeutic approach and the role of new treatments need to be assessed in prospective clinical trials.

Humans↗
PubMed1992

Epithelioid and spindle-cell hemangioendothelioma of the spleen. Report of a distinctive splenic vascular neoplasm of childhood.

A case of a distinctive vascular neoplasm of the spleen in a 3-year-old boy is described. The tumor was characterized histologically by a biphasic growth pattern, with discrete nodular areas composed of atypical round, epithelioid cells with large nuclei and prominent nucleoli, and areas showing an intricate proliferation of vascular channels lined by elongated spindle cells. Immunohistochemical studies showed cytoplasmic staining of the tumor cells with factor VIII-related antigen, Ulex europaeus lectin, and vimentin antibodies. Stains for keratin, actin, desmin, lysozyme, and S-100 protein were negative in the tumor cells. Electron microscopy revealed a fairly cohesive population of cells that contained mature and immature cell junctions, basal lamina material, and surface pinocytotic activity consistent with vascular endothelial cells. Five-year follow-up has shown the patient to be alive and free of disease. This case appears to represent a previously unreported primary vascular neoplasm of the spleen showing combined features of epithelioid and spindle-cell hemangioendothelioma. The lesion should be distinguished from other benign and malignant vascular proliferations of the spleen such as Kaposi's sarcoma, angiosarcoma, and the recently described littoral-cell angioma.

Child, Preschool↗
PubMed1989

Nonangiogenic and nonlymphomatous sarcomas of the canine spleen: 57 cases (1975-1987).

The case records of and histopathologic findings in 57 dogs with nonangiogenic and nonlymphomatous splenic sarcomas were reviewed. Splenic neoplasms in these dogs included leiomyosarcoma, fibrosarcoma, undifferentiated sarcoma, liposarcoma, osteosarcoma, chondrosarcoma, myxosarcoma, rhabdomyosarcoma, and fibrous histiocytoma. The clinical signs associated with splenic sarcoma included anorexia or decreased appetite, abdominal distention, polydipsia, lethargy, vomiting, weight loss, and weakness. An abdominal mass was detected in 86% of the dogs by use of abdominal palpation (63%), and/or abdominal radiography (74%). The diagnosis was based on histopathologic findings in the spleen. Abdominal exploratory surgery was performed on 43 of the 57 dogs. Twenty-seven dogs were treated by splenectomy, and 16 were euthanatized at the time of surgery because of widespread metastatic lesions. Of the 14 dogs on which surgery was not performed, 11 were euthanatized on the basis of results of preoperative diagnostic tests, and the remaining 3 dogs had splenic neoplasms that were incidental findings at necropsy. Of the 27 surgically treated dogs, 5 died in the immediate postoperative period, 12 died or were euthanatized within 1 year after splenectomy, and only 5 dogs survived greater than or equal to 1 year. Three dogs were lost to follow-up evaluation, and 2 were still alive 6 and 7 months after surgery. The median survival time of the 22 dogs for which survival was known was 2.5 months. The median survival time for 11 dogs with no obvious metastasis at the time of splenectomy was 9 months.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1998

Splenic marginal zone cell lymphoma associated with clonal B-cell populations showing different immunoglobulin heavy chain sequences.

Splenic marginal zone cell lymphomas (SMZCLs) are low-grade B-cell lymphomas that usually present with massive splenomegaly and subtle (subleukemic) peripheral blood involvement. Polymerase chain reaction (PCR) analysis of peripheral blood from a patient with subleukemic SMZCL showed evidence of two clonal immunoglobulin heavy chain (IgH) gene rearrangements. IgH PCR analysis of DNA derived from the patient's splenic neoplasm demonstrated a single clonal IgH rearrangement, which had a different electrophoretic mobility from either of the two PCR products detected in the patient's peripheral blood. Additional characterization of these PCR products by DNA sequencing demonstrated two independent IgH rearrangements in the peripheral blood, one of which used IgH joining region 6c (JH6C) and the other JH4. A different IgH rearrangement was present in the splenic tumor, which used JH4a. No sequences from the splenic neoplasm were detected in the peripheral blood and vice versa. This case illustrates that PCR might reveal monoclonal populations in peripheral blood unrelated to the presence of lymphoma in other anatomic compartments.

B-Lymphocytes↗
PubMed1984

Two-dimensional, gray-scale ultrasonography for assessment of hepatic and splenic neoplasia in the dog and cat.

An analysis was made of 17 cases of microscopically proven hepatic and splenic neoplasms, with the objective of describing the ultrasonographic findings and determining how interpretation of these findings contributed to the diagnosis and prognosis of these cases. The ultrasonographic appearance of these tumors ranged from focal, large (6 to 20 cm diameter) mixed hyperechoic/hypoechoic masses to multifocal (0.5 to 2.0 cm diameter) hypoechoic masses. The ultrasonographic appearance was not specific for the microscopic cell-type of the neoplasm. When the combined clinical, hematologic, serum chemical, and ultrasonographic findings were interpreted, neoplasia was always the primary consideration, but microscopic confirmation was mandatory. The uses of ultrasonography in these cases were: (1) to characterize the internal architecture of a mass identified radiographically, (2) to confirm the organ of origin of the mass, (3) to define the extent of disease in or around an affected organ, (4) to evaluate palpable abdominal mass(es) in the presence of poor abdominal radiographic contrast, and (5) to identify previously undetected or unsuspected metastases. Three interpretive errors were found, but only one would have influenced the prognosis for that specific patient.

Animals↗