Kaposiform hemangioendothelioma of the choledochus

Terui, Keita; Nakatani, Yukio; Kambe, Michiyo; Fukunaga, Masao; Hishiki, Tomoro; Saito, Takeshi; Sato, Yoshiharu; Takenouchi, Ayako; Saito, Eizo; Ono, Shun; Yoshida, Hideo

doi:10.1016/j.jpedsurg.2010.05.031

Cited by 9 publications

(2 citation statements)

References 11 publications

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“…[3] Biopsy-proven hepatic KHE has never been reported, although a single case involving the common bile duct was recently described. [4] KHE is described as a “rare” vascular tumor; no epidemiologic studies have reported incidence or prevalence data.…”

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confidence: 99%

Kaposiform Hemangioendothelioma: Atypical Features and Risks of Kasabach-Merritt Phenomenon in 107 Referrals

Croteau

Liang

Kozakewich

et al. 2013

The Journal of Pediatrics

338

381

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Objective To examine the presentation characteristics of patients with kaposiform hemangioendothelioma (KHE) to describe the spectrum of disease and risk factors for Kasabach-Merritt phenomenon (KMP). Study design Retrospective review of 163 patients referred to the Vascular Anomalies Center at Children’s Hospital Boston for KHE between 1991 and 2009 identified 107 patients with sufficient data for inclusion. Results The prevalence of KHE in Massachusetts is approximately 0.91/100,000 children. KHE manifested in infancy in 93% of cases; 60% as neonates. Common presenting features included enlarging cutaneous lesion (75%), thrombocytopenia (56%), and musculoskeletal pain or decreased function (23%). Cutaneous KHE favored the extremities, especially overlying joints. In our cohort 71% developed KMP (11% after initial presentation), and 11% of patients lacked cutaneous findings. Retroperitoneal and intrathoracic lesions, though less common, were complicated by KMP in 85% and 100% of cases, respectively. Compared with superficial lesions, KHE infiltrating into muscle or deeper was 6.3 fold more likely to manifest KMP, and 18-fold higher if retroperitoneal or intrathoracic. KHE limited to bone or presenting after infancy did not manifest KMP. Conclusion An enlarging lesion is the most common presenting feature of KHE in infancy. Older patients with KHE or those lacking cutaneous manifestations present with musculoskeletal complaints or atypical symptoms. The risk of KMP increases dramatically when tumor infiltrates muscle or when KHE arises in the retroperitoneum or mediastinum.

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confidence: 99%

Kaposiform Hemangioendothelioma: Atypical Features and Risks of Kasabach-Merritt Phenomenon in 107 Referrals

Croteau

Liang

Kozakewich

et al. 2013

The Journal of Pediatrics

338

381

View full text Add to dashboard Cite

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“…However, the first liver biopsy revealed no positive pathological results, and the patient’s platelet count remained low after right hepatic artery embolization. Moreover, biopsy-proven hepatic KHE is even rarer than other KHEs, with only one published autopsy in a 9-day-old neonate and one bile duct case ( 3 , 7 , 19 ). In contrast, three cases described a pancreatic mass close to the hepatic portal area on MRI or CT scan.…”

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confidence: 99%

Case Report: Pancreatic and hepatic kaposiform hemangioendothelioma presenting as consumptive coagulopathy and right hepatic atrophy

Qiu

Zhou

et al. 2023

Front. Oncol.

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Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor that causes progressive angiogenesis and lymphangiogenesis, which often occurs in the skin or soft tissue, with an acute onset and rapid progression. A 4-year-old girl was admitted to our hospital with a 2-year history of thrombocytopenia, combined with right hepatic atrophy and pancreatic lesion for 3 months. At the age of two, she developed purpura and thrombocytopenia was detected, after treatment with gamma globulin and corticosteroids, the platelet count normalized, but it dropped immediately at lower doses. One year after the cessation of corticosteroids therapy, the patient presented with abdominal pain and abnormal liver function and the magnetic resonance imaging (MRI) revealed right hepatic atrophy and pancreatic occupancy, but the first liver biopsy did not reveal any positive pathological results. By analyzing the clinical manifestations in conjunction with MRI and abnormal coagulation, we considered that the patient might be diagnosed as KHE with Kasabach-Merritt phenomenon, however, sirolimus treatment was ineffective and pancreatic biopsy only showed a tendency for tumors of vascular origin. Finally, we performed a Whipple operation after the right hepatic artery embolization, histological and immunohistochemical examination suggested KHE. Three months postoperatively, the patient’s liver function, pancreatic enzymes and blood clotting function gradually returned to normal. KHEs may result in significant blood loss with worsening of the coagulopathy and functional impairment, timely surgical intervention for KHE is necessary when non-invasive or minimally invasive treatment is ineffective, or the symptoms of tumor compression are obvious.

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Variants of Hemangioendotheliomas of the Hepatobiliary Tract

Zimmermann

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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Apart from epithelioid hepatic hemangioendothelioma and infantile hepatic hemangioendothelioma/hepatic infantile hemangioma, there are few other endotheliomas that can manifest as primary neoplasms of the liver. Kaposiform hemangioendothelioma is a rare, locally aggressive, vascular spindle-cell tumor that resembles Kaposi's sarcoma. This neoplasm most commonly develops in the skin and the retroperitoneal space of infants and children, but is also observed in the hepatobiliary tract in rare instances. Kaposiform hemangioendothelioma is a wellknown cause of Kasabach-Merritt syndrome. Histologically, the neoplasm is characterized by nodules or lobules of rather bland-looking spindle cells which encircle slit-like, CD31and CD34-positive vascular channels. A further hemangioendotheliomatous neoplasm that develops in the liver as a primary tumor is polymorphous hemangioendothelioma.

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Kaposiform hemangioendothelioma of the choledochus

Cited by 9 publications

References 11 publications

Kaposiform Hemangioendothelioma: Atypical Features and Risks of Kasabach-Merritt Phenomenon in 107 Referrals

Kaposiform Hemangioendothelioma: Atypical Features and Risks of Kasabach-Merritt Phenomenon in 107 Referrals

Case Report: Pancreatic and hepatic kaposiform hemangioendothelioma presenting as consumptive coagulopathy and right hepatic atrophy

Variants of Hemangioendotheliomas of the Hepatobiliary Tract

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