Hamartomas of the spleen: a study of 20 biopsy cases

Falk, Stephan; Stutte, H. J.

doi:10.1111/j.1365-2559.1989.tb02201.x

Cited by 71 publications

(38 citation statements)

References 11 publications

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“…Splenic hamartomas and littoral cell angiomas are both considered primary splenic lesions, most likely derived from the splenic red pulp. Similar to the angiosarcomas in our study, both of these tumors show "biphasic" immunoreactivity for vascular and histiocytic markers (31)(32)(33)(37)(38)(39). Further support for the theory that splenic angiosarcomas arise from the splenic red pulp is lent by the finding of only red pulp involvement in three cases.…”

Section: Discussionsupporting

confidence: 83%

Splenic Angiosarcoma: A Clinicopathologic and Immunophenotypic Study of 28 Cases

et al. 2000

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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 fibroushistiocytoma 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.

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Section: Discussionsupporting

confidence: 83%

Splenic Angiosarcoma: A Clinicopathologic and Immunophenotypic Study of 28 Cases

et al. 2000

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“…Monoclonal antibodies against the following antigens were used: CD8 (C8/144B; Dako North America, Carpinteria, CA, USA), collagen type IV (CIV 22; Dako), smooth muscle actin (1A4; Dako), low-affinity nerve growth factor receptor/LNGFR (ME20. 4 …”

Section: Immunohistochemistrymentioning

confidence: 99%

“…1,2 Splenic hamartoma has no gender predilection, and has been diagnosed at all ages (11 months to 86 years). [2][3][4] The majority of patients with splenic hamartoma are asymptomatic. The 15% or so symptomatic patients present with abdominal discomfort, malaise, fever, splenomegaly, cytopenias, and rarely portal hypertension or rupture.…”

mentioning

confidence: 99%

Clonal X-chromosome inactivation suggests that splenic cord capillary hemangioma is a true neoplasm and not a subtype of splenic hamartoma

et al. 2011

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Splenic hamartoma is a rare tumor-like lesion composed of structurally disorganized red pulp elements. It has been hypothesized that two other splenic lesions, cord capillary hemangioma and myoid angioendothelioma, may fall within the spectrum of splenic hamartoma, simply representing morphological variants. In this study, we compared the vascular and stromal composition of cord capillary hemangioma and myoid angioendothelioma with those of classical hamartoma. In addition, we assessed the clonal vs polyclonal nature of the lesions in nine female cases by performing clonality analysis for X-chromosome inactivation at the human androgen receptor locus (HUMARA) on laser-assisted microdissected samples. In 15 of 17 cases, increased reticulin and/or collagen content was observed. The classical hamartoma cases showed a vasculature predominantly composed of CD8 þ CD31 þ CD34À splenic sinuses, whereas cases of cord capillary hemangioma and myoid angioendothelioma contained many CD8À CD31 þ CD34 þ cord capillaries, but very little CD8 þ vasculature. All cases lacked expression of D2-40 and Epstein Barr virus-encoded RNA. All cases showed a proliferation index of r5% by Ki-67. Cases of classical hamartoma lacked significant perisinusoidal expression of collagen IV and low-affinity nerve growth factor receptor. Both markers were variably expressed in the other lesions. Increased CD163-positive histiocytes were found in four cases (three cord capillary hemangiomas and one myoid angioendothelioma). HUMARA analysis was informative in all nine tested cases, of which three cases showed a non-random X-chromosome inactivation pattern, indicating clonality. All three clonal cases were cord capillary hemangiomas. Our study has shown that in spite of considerable morphologic heterogeneity and overlapping features, classical hamartoma and cord capillary hemangioma and myoid angioendothelioma are different in terms of their vascular and stromal composition. Clonality analysis supports a true neoplastic origin for the cord capillary hemangioma. A larger study using additional immunohistochemical and molecular studies is necessary to further evaluate the biological significance of the current findings.

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“…3,8 Clinically, it usually presents as an incidental finding on imaging studies but can present with mass effect or rupture. 9 Regardless of the method of presentation, the clinical course is benign.…”

Section: Discussionmentioning

confidence: 99%