Management of Vascular Sarcoma

Subramaniam, Aparna; Giani, Claudia; Napolitano, Andrea; Ravi, Vinod; Frezza, Anna Maria; Jones, Robin L.

doi:10.1016/j.soc.2022.03.014

Cited by 2 publications

(4 citation statements)

References 150 publications

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“…Given the very limited activity of conventional chemotherapy in this disease and the absence of data suggesting a potential advantage in survival, neoadjuvant or adjuvant medical treatments are not recommended [16] . To our knowledge, this was the first case reported in the literature of an EHE of a liver transplant recipient effectively treated with liver re-transplantation.…”

Section: Discussionmentioning

confidence: 99%

Epithelioid Hemangioendothelioma in a Liver Transplant Recipient: A Case Report of an Extremely Rare Tumor

Gonçalves,

Pessegueiro,

Gandara

et al. 2023

GE Port J Gastroenterol

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Epithelioid hemangioendothelioma is a very rare vascular neoplasm, which is often multifocal or metastatic at diagnosis. Most frequently arises in the liver, followed by the lung and bones. The authors present a case of a liver transplant recipient who developed a pattern of hepatic cholestasis associated with the appearance of a proliferative hepatic lesion with infiltrative growth. Histological examination and immunohistochemical study were compatible with the diagnosis of epithelioid hemangioendothelioma. Pulmonary micronodules were detected and lung metastases were hypothesized. Therefore, bronchoscopy was performed, which turned out to be normal, and cytology was negative for neoplastic cells. After a multidisciplinary discussion, liver re-transplantation was decided. After 8 years of follow-up, the patient is clinically stable, with no graft dysfunction, no neoplastic recurrence, and dimensional stability of the pulmonary micronodules. Patients with organ transplant have higher risk of developing carcinoma compared to the general population. The development of cancer is a multifactorial process and little is known about the etiology of epithelioid hemangioendothelioma. No standard treatment strategy has been defined yet, and the natural course of the disease is heterogenous and the individual prognosis unpredictable. Complete surgical resection is offered to patients with unifocal disease, and those with unresectable disease should be evaluated for orthotopic liver transplantation.

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Section: Discussionmentioning

confidence: 99%

Epithelioid Hemangioendothelioma in a Liver Transplant Recipient: A Case Report of an Extremely Rare Tumor

Gonçalves,

Pessegueiro,

Gandara

et al. 2023

GE Port J Gastroenterol

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“…For extensive or unresectable tumors, combined treatment strategies are recommended, particularly if accompanied by KMP ( 20 ). Based on the available literature, combination treatment with vincristine and corticosteroids, antifibrinolytic agents, systemic interferons, and mammalian target of rapamycin (mTOR) inhibitors; transarterial embolization; and radiation therapy have shown clinical benefits in KHE ( 2 , 11 , 16 , 22 ). Previously, vincristine and corticosteroids have been recommended as the first-line treatment for KHE ( 23 ).…”

Section: Introductionmentioning

confidence: 99%

“…Histologically, KHE is characterized by irregular tumor margins, infiltrating rounded nodules composed of endothelial cells of spindled morphology, and slit-like vascular channels (13,14). Immunohistochemistry (IHC) shows positive staining for both lymphatic markers and vascular endothelial markers (4,7,15,16). A major prognostic factor is the presence of the Kasabach-Merritt phenomenon (KMP), which is characterized by life-threatening consumptive coagulopathy and severe thrombocytopenia (16)(17)(18).…”

Section: Introductionmentioning

confidence: 99%

“…Immunohistochemistry (IHC) shows positive staining for both lymphatic markers and vascular endothelial markers (4,7,15,16). A major prognostic factor is the presence of the Kasabach-Merritt phenomenon (KMP), which is characterized by life-threatening consumptive coagulopathy and severe thrombocytopenia (16)(17)(18). Despite KMP being less common in adults (11%) compared to children (77%), it is present in up to 70% of KHE cases at the time of presentation (8,19).…”

Section: Introductionmentioning

confidence: 99%

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Adult-onset multifocal kaposiform hemangioendothelioma in the bone marrow, lung, liver, and brain: a case report

Bello,

Alikhan,

Subramaniam

et al. 2024

Front. Oncol.

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Kaposiform hemangioendothelioma (KHE), a rare form of vascular neoplasm, is typically seen in children. In this paper, we report a unique case of KHE replacing bone marrow tissue mimicking myeloproliferative neoplasm with additional involvement in the lung, liver, and brain in a 60-year-old Caucasian woman. The patient was initially seen in the hematology department for the chief complaint of epigastric pain and anemia. Abdominal magnetic resonance imaging (MRI) revealed mild splenomegaly with iron deposition secondary to extramedullary hematopoiesis. Additional workup was inconclusive. Subsequent bone marrow and lung biopsies eventually revealed bone marrow with extensive grade 3 fibrosis and multiple foci of low-grade vasoformative neoplasm in the lung suggestive of KHE. Although rare, KHE can present as an aggressive disease with indolent behavior in adults and can be distinguished from other vascular malignancies based on histopathology and imaging findings.

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Management of Vascular Sarcoma

Cited by 2 publications

References 150 publications

Epithelioid Hemangioendothelioma in a Liver Transplant Recipient: A Case Report of an Extremely Rare Tumor

Epithelioid Hemangioendothelioma in a Liver Transplant Recipient: A Case Report of an Extremely Rare Tumor

Adult-onset multifocal kaposiform hemangioendothelioma in the bone marrow, lung, liver, and brain: a case report

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