Adult-Onset Kaposiform Hemangioendothelioma in a Posttraumatic Site

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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“…Recent reports of adult-onset KHE also point to trauma as a possible trigger of the tumor proliferation. [27]…”

Section: Discussionmentioning

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

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331

381

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“…In rare scenarios, signs and symptoms of KHE/TA can worsen with trauma or infections. There is also evidence that aggravation or manipulation of the tumor either from surgery or trauma can incite KMP, whether the patient had a history of KMP or not [15][16][17]. In addition, rapid enlargement of the lesion with severe KMP development shortly after vaccination has been reported in several patients [18][19][20][21].…”

Section: Etiologymentioning

confidence: 99%

Kaposiform hemangioendothelioma: current knowledge and future perspectives

Chen

Yang

et al. 2020

Orphanet J Rare Dis

115

180

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Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm with high morbidity and mortality. The initiating mechanism during the pathogenesis of KHE has yet to be discovered. The main pathological features of KHE are abnormal angiogenesis and lymphangiogenesis. KHEs are clinically heterogeneous and may develop into a lifethreatening thrombocytopenia and consumptive coagulopathy, known as the Kasabach-Merritt phenomenon (KMP). The heterogeneity and the highly frequent occurrence of disease-related comorbidities make the management of KHE challenging. Currently, there are no medications approved by the FDA for the treatment of KHE. Multiple treatment regimens have been used with varying success, and new clinical trials are in progress. In severe patients, multiple agents with variable adjuvant therapies are given in sequence or in combination. Recent studies have demonstrated a satisfactory efficacy of sirolimus, an inhibitor of mammalian target of rapamycin, in the treatment of KHE. Novel targeted treatments based on a better understanding of the pathogenesis of KHE are needed to maximize patient outcomes and quality of life. This review summarizes the epidemiology, etiology, pathophysiology, clinical features, diagnosis and treatments of KHE. Recent new concepts and future perspectives for KHE will also be discussed.

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“…Two-thirds of the adult patients have a tumor with a diameter less than 2 cm. Adult-onset KHE does not show thrombocytopenia, whereas over 50% of children with KHE show KMP [1,5,6,11]. KHE occurring in adults also seem to be sensitive to chemotherapy [8,11,12].…”

Section: Discussionmentioning

confidence: 99%

Kaposiform Hemangioendothelioma in Adolescent-Onset Scoliosis: A Case Report and Review of Literature

Kim

et al. 2020

Case Reports in Orthopedics

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Background and Objectives. Kaposiform hemangioendothelioma (KHE) is a vascular tumor of very low incidence, which occurs mostly in children and infants. The tumor is recognized for its locally aggressive, yet rarely metastatic behavior. It may cause consumptive coagulopathy known as Kasabach-Merritt phenomenon. We report a distinctive case, where an 11-year-old boy is presented with progressive thoracolumbar scoliosis without any symptom or neurological sign. Case Report. The patient underwent spinal deformity correction via posterior pedicle screw instrumentation and fusion, along with tumor biopsy. The pathology report confirmed KHE. The patient did not show a prominent progression of scoliosis after the surgery without any further treatments. Conclusions. Many of scoliotic patients do not have any apparent cause, thereby regarded as idiopathic scoliosis. The presented case is where kaposiform hemangioendothelioma is likely to be linked to the patient’s scoliosis. We demonstrate the possibility of secondary scoliosis should always be kept in mind of orthopaedic doctors. We also conclude that secondary scoliosis does not show exacerbation after growth completion.

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Adult-Onset Kaposiform Hemangioendothelioma in a Posttraumatic Site

Cited by 21 publications

References 22 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

Kaposiform hemangioendothelioma: current knowledge and future perspectives

Kaposiform Hemangioendothelioma in Adolescent-Onset Scoliosis: A Case Report and Review of Literature

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