Hemangioendothelioma arising from the spleen: A case report and literature review

Wang, Zhendan; Zhang, Liang; Zhang, Bo; Mu, Dianbin; Cui, Kai; Li, Sheng

doi:10.3892/ol.2014.2693

Cited by 16 publications

(12 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Deep tissue KHE are less common than cutaneous lesions, and reports of isolated splenic KHE are exceedingly rare, with only two pediatric cases and two adult cases reported. [11][12][13][14] Multifocal KHE has been reported as a simultaneously cutaneous and deep tissue structure, further illustrating the wide range of possible presentations. 6,15,16 Out of these four reports of isolated splenic KHE, only Shabtaie et al's description of a prenatally diagnosed splenic mass was associated with KMP.…”

Section: Discussionmentioning

confidence: 95%

Splenic kaposiform hemangioendothelioma presenting as insidious consumptive coagulopathy

et al. 2021

View full text Add to dashboard Cite

A healthy 2-year-old male with a history of mild persistent asthma visited the outpatient hematology clinic to evaluate intermittent epistaxis. There was no history of recent illness. His exam was notable for left upper extremity bruising secondary to recent venipuncture, but no lymphadenopathy or hepatosplenomegaly. Complete blood count (CBC) revealed hemoglobin 9.3 g/dL, leukocyte count 20.7 10 9 /L, and platelet count 33 k 10 9 /L. Peripheral blood smear exhibited polychromasia, numerous large platelets, and a few reactive lymphocytes.The differential diagnosis for both thrombocytopenia and anemia include processes leading to impaired hematopoiesis, increased cell destruction, or abnormal cell pooling. Impaired hematopoiesis could stem from infectious suppression, infiltrative processes (leukemia, metabolic disorders, etc.), or inherited and acquired bone marrow failure syndromes. Possible destructive or consumptive processes included microangiopathy, disseminated intravascular coagulation (DIC), or immune destruction. Sequestration within the liver or spleen was considered, but he was without hepatosplenomegaly to suggest a site of abnormal pooling. We approached his work-up with a stepwise approach. Screening tests were normal for common infectious diseases implicated in marrow suppression (Table 1). Since the patient appeared well and did not exhibit any findings that would prompt an immediate evaluation for malignancy or sepsis, we decided to repeat a short interval CBC to see if his cytopenias self-resolved. When they did not, further evaluation of the bone marrow was warranted. Bone marrow biopsy and aspirate did not demonstrate marrow infiltration or failure (Table 1). Lab work was sent to investigate consumptive processes. Lactate dehydrogenase levels and reticulocyte counts were elevated (Table 1), suggesting a hemolytic process. A hemoglobinopathy screen and glucose-6-phosphate dehydrogenase testing were normal, though active hemolysis can skew results. In an ill-appearing child, the presence of hemolysis and thrombocytopenia should prompt an investigation for DIC or microangiopathy, but in this well appearing-child, an immune process was thought likelier. We, therefore, pursued an immunologic work-up, including screening for autoimmune lymphoproliferative syndrome (ALPS) (Table 1). The results were unrevealing except for a low IgG level at 228 (505-1280 mg/dL) with normal lymphocyte subsets.Low IgG is often associated with systemic autoimmune disorders, vasculitides, atopy, and asthma. We attributed the low IgG and eosinophilia to the patient's known history of asthma. A consumptive autoimmune process was high on the differential, and the involvement of two cell lines would be classified as Evans syndrome. Evans syndrome, though never a primary diagnosis, is treated upfront with

show abstract

Section: Discussionmentioning

confidence: 95%

Splenic kaposiform hemangioendothelioma presenting as insidious consumptive coagulopathy

et al. 2021

View full text Add to dashboard Cite

show abstract

“…The patient showed no recurrence or complications during the 15-month follow up. 13 In our case, the only presented symptom was left-side abdominal pain. Hematological parameters were completely normal.…”

Section: Discussionmentioning

confidence: 98%

Primary Splenic Hemangioendothelioma: A Case Report and Literature Review

Ekmen¹,

Helvacı²,

Günaldı³

et al. 2016

Turkiye Klinikleri J Intern Med

View full text Add to dashboard Cite

plenic tumors are more rarely seen comparing to other visceral organ tumors. Primary tumors of spleen are divided into non-lymphoid and lymphoid groups. Vascular neoplasms of non-lymphoid tumors consist the vast majority part. In the literature review, among splenic hemangioendothelioma cases, rarely of them were reported as primary splenic epithelioid hemangioendothelioma. Its frequency among splenic tumors is 0.3-14%. These tumors do not become in large sizes and have better prognosis. If they become large, they may cause symptoms such as pancytopenia and hemolytic anemia due to splenomegaly and hypersplenism. Early diagnosis and treatment may prevent conditions such as widespread metastasis, splenic rupture and operative complications. In this article, we present clinical and imaging findings of epitheloid splenic hemangioendothelioma with review of other reported cases in the light of literature.

show abstract

“…Since it is a benign tumor of the spleen, it is possible to perform partial splenectomy, especially in younger people [19]. In general, the small number of cases described so far, as well as the lack of reliable information on the application of partial splenectomy in the treatment of MAE, are a limiting factor [20].…”

Section: Discussionmentioning

confidence: 99%

Report on four cases of the rarest benign splenic tumor - myoid angioendothelioma, with literature review

Milosavljević¹,

Grubor

Matić

et al. 2021

Srp Arh Celok Lek

View full text Add to dashboard Cite

Introduction. Myoid angioendothelioma of the spleen is an extremely rare form of a benign splenic tumor. There is no characteristic symptomatology for this disease. Case outline. We present four patients operated on for myoid angioendothelioma of the spleen. Three patients were without problems while one had nonspecific problems. Two patients underwent laparoscopic surgery and two underwent open splenectomy. In all patients, the definitive diagnosis was confirmed by histopathological examination. Discussion. By reviewing the aforementioned medical databases of published papers in English, we found a total of eight cases of myoid angioendothelioma of the spleen. In addition to the mentioned number, we have added our series of cases. Conclusion. Twenty-one years after the discovery of myoid angioendothelioma of the spleen, the small number of cases presented in the literature is still a limiting factor in making conclusions and in learning lessons about this disease

show abstract

Hemangioendothelioma arising from the spleen: A case report and literature review

Cited by 16 publications

References 10 publications

Splenic kaposiform hemangioendothelioma presenting as insidious consumptive coagulopathy

Splenic kaposiform hemangioendothelioma presenting as insidious consumptive coagulopathy

Primary Splenic Hemangioendothelioma: A Case Report and Literature Review

Report on four cases of the rarest benign splenic tumor - myoid angioendothelioma, with literature review

Contact Info

Product

Resources

About