Kaposiform haemangioendothelioma of the head and neck

Wong, Billy L. K.; Lee, Vivian N.Y.; Tikka, Theofano; Dae, Kim; Dwivedi, Raghav C.

doi:10.1016/j.critrevonc.2016.06.005

Cited by 21 publications

(16 citation statements)

References 56 publications

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“…e Uniform strong nuclear reactivity for ERG. f HHV8 was expressed in all cases hemangioendothelioma of the head and neck [25]. This especially rare intermediate grade neoplasm occurs predominantly in children, is not associated with immunodeficiency of any type, and lacks HHV8 infection.…”

Section: Discussionmentioning

confidence: 89%

Head and Neck Kaposi Sarcoma: Clinicopathological Analysis of 11 Cases

Agaimy

Müller

Harrer

et al. 2018

Head and Neck Pathol

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Kaposi sarcoma (KS) of the head and neck area is uncommon with limited published case series. Our routine and consultation files were reviewed for histologically and immunohistochemically proven KS affecting any cutaneous or mucosal head and neck site. Ten males and one female aged 42-78 years (median, 51 years; mean, 52 years) were retrieved. Eight patients were HIV-positive and three were HIV-negative. The affected sites were skin (n = 5), oral/oropharyngeal mucosa (n = 5), and lymph nodes (n = 3) in variable combination. The ear (pinna and external auditory canal) was affected in two cases; both were HIV-negative. Multifocal non-head and neck KS was reported in 50% of patients. At last follow-up (12-94 months; median, 46 months), most of patients were either KS-free (n = 8) or had ongoing remission under systemic maintenance therapy (n = 2). One patient was alive with KS (poor compliance). Histopathological evaluation showed classical features of KS. One case was predominantly sarcomatoid with prominent inflammation mimicking undifferentiated sarcoma. Immunohistochemistry showed consistent expression of CD31, CD34, ERG, D2-40 and HHV8 in all cases. This is one of the few series devoted to head and neck KS showing high prevalence of HIV-positivity, but also unusual presentations in HIV-negative patients with primary origin in the skin of the ear and the auditory canal. KS should be included in the differential diagnosis of difficult-to-classify spindle cell lesions at this uncommon location.

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Section: Discussionmentioning

confidence: 89%

Head and Neck Kaposi Sarcoma: Clinicopathological Analysis of 11 Cases

Agaimy

Müller

Harrer

et al. 2018

Head and Neck Pathol

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“…Patients present with a rapidly enlarging mass located superficially and/or deeply. Possible other symptoms are respiratory distress, cranial nerve palsy, epistaxis, hemoptysis, otorrhagia and hyposmia [67]. Kasabach-Merritt-phenomenon or disseminated intravascular coagulation is an adverse clinical condition often associated with a deep anatomic site [68][69][70].…”

Section: Kaposiform Hemangioendothelioma (Khe)/ Tufted Hemangioma (Ta)mentioning

confidence: 99%

Soft Tissue Special Issue: Perivascular and Vascular Tumors of the Head and Neck

Flucke

Karanian

Broek

et al. 2020

Head and Neck Pathol

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Perivascular and vascular neoplasms of the head and neck are a rare group of tumors comprising a spectrum of clinical/ biologic and histological features. They are frequently diagnostically challenging, due to their morphologic and immunohistochemical overlap. In this review, we summarize the pathology of these neoplasms, discussing morphology, immunohistochemistry, associated genetic findings, and the differential diagnoses.

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“…The International Society for the Study of Vascular Anomalies (ISSVA) issued an updated classification system in April 2014 . KHE is divided into borderline or locally extensive vascular tumors that have a tendency of metastasis, as well as to regional lymph nodes, but do not regress spontaneously . Greater than 70% of patients with KHE have associated Kasabach–Merritt phenomenon (KMP), which was characterized by consumptive coagulopathy, thrombocytopenia, and an enlarging vascular tumor with a mortality of 20% .…”

Section: Introductionmentioning

confidence: 99%

“…2 KHE is divided into borderline or locally extensive vascular tumors that have a tendency of metastasis, as well as to regional lymph nodes, but do not regress spontaneously. 3 Greater than 70% of patients with KHE have associated Kasabach-Merritt phenomenon (KMP), which was characterized by consumptive coagulopathy, thrombocytopenia, and an enlarging vascular tumor with a mortality of 20%. 4 Despite the ISSVA classification, the optimal treatment for KHE is not established and variable responses to different treatment strategies, including vincristine, 5 corticosteroids, 6 propranolol, 7 interferon, 8 and radiation therapy, 9 have been reported.…”

Section: Introductionmentioning

confidence: 99%

Sirolimus therapy for kaposiform hemangioendothelioma with long‐term follow‐up

Wang

Yao

Sun³

et al. 2019

The Journal of Dermatology

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Mammalian target of rapamycin inhibitors have shown promising results in the management of kaposiform hemangioendothelioma (KHE). The purpose of this study was to present our experience involving sirolimus therapy for KHE. A retrospective study was conducted to review the medical documents of 26 patients with KHE who were treated with sirolimus at our hospital between March 2012 and December 2016. Fifteen males and 11 females manifested KHE in infancy with an average age of 2.9 ± 1.8 months. Multiple anatomical sites were involved. Four patients had multifocal lesions, while 22 patients had solitary lesions. Twenty‐five patients had Kasabach–Merritt phenomenon (KMP). Twenty patients completed sirolimus therapy in 28.3 ± 12.5 months. Nineteen KHE lesions reduced to small residuals with platelet counts reaching normal levels 3.7 ± 2.8 weeks after treatment; one KHE lesion had no response to therapy. One patient with multifocal lesions died due to a severe infection, although the patient had previously responded to sirolimus. Five patients remained in treatment and had good responses with normal platelet counts. Nineteen patients with anemia had normal hemoglobin levels after 3.5 ± 1.9 weeks of treatment. Mild side effects were observed. The median follow‐up time was 32 months (26–60 months), with no evidence of recurrences. Sirolimus was shown to be efficacious in the management of KHE with an average course of 28 months. The time‐to‐response was variable, with an average of 1 week. After 4 weeks of treatment, the platelet count and hemoglobin level had normalized. Multifocal KHE with KMP is more severe than solitary KHE.

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Kaposiform haemangioendothelioma of the head and neck

Cited by 21 publications

References 56 publications

Head and Neck Kaposi Sarcoma: Clinicopathological Analysis of 11 Cases

Head and Neck Kaposi Sarcoma: Clinicopathological Analysis of 11 Cases

Soft Tissue Special Issue: Perivascular and Vascular Tumors of the Head and Neck

Sirolimus therapy for kaposiform hemangioendothelioma with long‐term follow‐up

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