Splenic Hamartoma, Vascular Type, with Endothelial Proliferation

ABSTRACT. An 11-year-old girl with low-grade fever, night sweats, thrombocytopenia, and an 8-year history of progressive splenomegaly underwent an elective splenectomy. Pathologic diagnosis was multiple splenic hamartoma. The patient's symptoms resolved after the splenectomy. Since first described by Rokitansky in 1861, ϳ140 cases of splenic hamartoma have been described in the literature. Most of the splenic hamartomas were discovered incidentally. A minority of these lesions were associated with hematologic symptoms such as pancytopenia, anemia, and thrombocytopenia. Only 20 of the reported cases of splenic hamartoma occurred in pediatric patients. However, compared with the adult patients, nearly half of these cases in pediatric patients was associated with symptoms. Splenectomy and partial splenectomy have relieved these symptoms. With advances in imaging, splenic hamartomas are being discovered with increasing frequency. A multimodal radiologic work-up has enabled some cases of splenic hamartoma to be diagnosed preoperatively. Inclusion of this benign entity in the differential diagnoses of symptomatic splenomegaly in a pediatric patient is important in the preoperative management and counseling of the patient and family. In patients who have discrete lesions, consideration of this entity preoperatively may avoid total splenectomy. Pediatrics 1998;101(5). URL: http://www.pediatrics.org/ cgi/content/full/101/5/e10; splenic hamartoma, pancytopenia, hypersplenism, splenomegaly, hemangiomas.

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“…12 The current case showed similar ultrastructural features, with occasional Weibel-Palade bodies identified.…”

Section: Discussionsupporting

confidence: 68%

Symptomatic Splenic Hamartoma: Case Report and Literature Review

Hayes¹,

Britton

Mewborne

et al. 1998

Pediatrics

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“…This may be due to the fact that they seldom cause clinical symptoms (Iozzo et al 1980). If present, symptoms are usually related to hypersplenism with subsequent thrombocytopenia and/or anaemia (Videbaek 1953, Hardmeier 1962, Ross & Schiller 1972, Shalev & Ariel 1978, Pardo-Mindan et al 1983), a finding *at is also reflected in our series. Larger lesions may cause upper abdominal discomfort related to splenomegaly.…”

Section: Discussionsupporting

confidence: 59%

Hamartomas of the spleen: a study of 20 biopsy cases

Falk

Stutte

1989

Histopathology

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Twenty hamartomas of the spleen were studied by histological, immuno- and enzyme-histochemical methods. In all cases nodules of varying size resembling proliferation of splenic red pulp structures were present that exhibited considerable morphological variation with regard to plasmacytosis, extramedullary haematopoiesis and phagocytic activity of macrophages. Splenic red pulp structures could be identified by immuno- and enzyme-histochemical markers reacting with sinus endothelium, including Factor VIII related antigen, proteinase inhibitors and non-specific esterases. In conjunction with the absence of organized lymphoid tissue and of dendritic reticulum cells these studies allow the differentiation of splenic hamartomas from haemangiomas that may possess a deceptively similar morphological appearance.

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“…Many cases were documented as case reports or in small series. To date, in excess of 130 splenic hamartomas have been documented in the English literature 1 –63 . Overall, hamartomas were detected over a wide age range (11 months to 86 years) and with a mean age of 47 years at presentation.…”

Section: Discussionmentioning

confidence: 99%

Splenic Vascular Lesions: Unusual Features and a Review of the Literature

Lam¹,

Yip²,

Peh

1999

Aust. N.Z. J. Surg.

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Hamartoma and peliosis are uncommon splenic lesions. Approximately 120 splenic hamartomas and 40 splenic peliosis have been reported in the English literature. In the present study, a unique case of multiple splenic hamartomas with peliosis was reported. The splenic lesions were incidental findings in a 36-year-old man with ruptured sarcomatoid renal cell carcinoma. They were diagnosed clinically as metastatic renal cell carcinoma. On pathological examination, peliosis was noted in the splenic hamartomas as well as in the splenic parenchyma. In addition, the clinicopathological features of five other splenic hamartomas (including one giant hamartoma) noted in our department were presented. A re-evaluation of the features of the splenic hamartomas documented in the English literature was also done.

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Splenic Hamartoma, Vascular Type, with Endothelial Proliferation

Cited by 26 publications

References 22 publications

Symptomatic Splenic Hamartoma: Case Report and Literature Review

Symptomatic Splenic Hamartoma: Case Report and Literature Review

Hamartomas of the spleen: a study of 20 biopsy cases

Splenic Vascular Lesions: Unusual Features and a Review of the Literature

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