Most KHEs appeared before 12 months of age. KHEs are associated with various major complications, which can occur in combination and develop early in the disease process. Young age, large lesion size and mixed lesion type are important predictors of KMP.

Background Kaposiform lymphangiomatosis (KLA) and kaposiform hemangioendothelioma (KHE) are rare and aggressive vascular disorders. The aim of this study was to examine the clinical features and prognosis of KLA and KHE involving the thorax. Methods The clinical features, imaging and pathological findings, treatments and outcome were retrospectively reviewed for 6 patients with KLA and 7 patients with KHE involving the thorax. Results The mean ages at the time of the presentation of signs/symptoms were 26.7 months and 4.1 months for KLA and KHE, respectively. Respiratory symptoms, pericardial and pleural effusion, thrombocytopenia and coagulopathy were common in both KLA and KHE. Diffuse lesions involving the lung and extrathoracic sites were observed in KLA but not in KHE. Histopathologically, all lesions had spindled tumor cells, which were immunoreactive for CD31 and D2–40. In KLA, the spindle cells were distributed in sparse and poorly marginated clusters, whereas the spindle cells formed more defined and confluent vascularized nodules in KHE. Unlike the refractory behavior of KLA, the majority of patients with KHE responded to medical treatments with regression of the lesion and normalization of the hematologic parameters. Conclusions The presenting and histological characteristics of KLA can overlap with those of KHE. The presence of diffuse vascular lesions in the mediastinum and lung with refractory thrombocytopenia and coagulopathy should suggest the diagnosis of KLA. Given the rarity and high morbidity and mortality of these disorders, the diagnostic process and therapeutic approach should include a multidisciplinary team review and consensus. Electronic supplementary material The online version of this article (10.1186/s13023-019-1147-9) contains supplementary material, which is available to authorized users.

Aim Kaposiform haemangioendothelioma (KHE) is a rare, potentially life‐threatening vascular tumour that is often associated with thrombocytopenia and coagulopathy, known as the Kasabach‐Merritt phenomenon (KMP). Because of the rarity and complexity of KHE, the optimal paradigm for treating KHE has yet to be elucidated. We aim to assess the efficacy and safety of vincristine and sirolimus for the treatment of KHE. Methods A comprehensive review of the literature was conducted from January 1993 to June 2018. A total of 15 studies were selected for the meta‐analysis. Five studies included 75 individuals and reported the response and side effects to vincristine in the treatment of KHE with or without KMP. A total of 10 studies that included 127 individuals reported the response and safety of sirolimus for treating KHE with or without KMP. Results The pooled odds ratio (OR) for the effectiveness of vincristine was 0.72. The pooled OR for the effectiveness of sirolimus was 0.91. The side effects associated with vincristine during the treatment included neuropathy, abdominal pain, loss of appetite and mild elevations of aspartate aminotransferase (AST) and alanine aminotransferase (ALT). The side effects associated with sirolimus therapy included bronchitis; lymphopenia; elevated AST, ALT and platelets; hyperlipidaemia; opportunistic infection; mild reversible leukopenia; mucositis; fever; pain and skin rash/vomiting and diarrhoea. Conclusions This systematic review showed a high efficacy of vincristine and sirolimus in the treatment of KHE. Based on the available data in the literature, it appears that sirolimus is potentially an efficacious and safe treatment option for KHE. Further randomised, controlled trials are recommended.

PurposeMusculoskeletal complications have been associated with kaposiform hemangioendothelioma (KHE) and can lead to disability and reduced quality of life. We aimed to determine the clinical characteristics of musculoskeletal complication in patients with KHE without Kasabach–Merritt phenomenon (KMP) in order to identify features that may aid clinicians in KHE treatment.Patients and methodsWe conducted a cohort study of KHE without KMP associated with musculoskeletal complication between January 2006 and February 2017 at three tertiary medical centers in China. The study included 29 nonthrombocytopenic patients with KHE and musculoskeletal complication.ResultsThe mean age at diagnosis of KHE was 4.5 years (range, 0.3–50.0 years). The mean follow-up was 4.1 years (range, 0.5–9.0 years). In most cases (72.4%), decreased range of motion (ROM) appeared within 2 years of KHE onset. Associated chronic pain was reported in 12 patients. Bone–joint changes were common in patients with decreased ROM (75.9%). All the patients received at least one medical therapy including corticosteroids, vincristine, propranolol, and sirolimus. Sirolimus demonstrated the highest efficacy rate, with 94.7% of patients showing improvements in ROM and chronic pain.ConclusionMusculoskeletal complication can occur early in the disease course of KHE without KMP. Although no uniformly effective treatment modality was found, sirolimus demonstrated the best response in patients with KHE with decreased ROM and chronic pain.

Aim: The aim of this study was to assess the efficacy of propranolol treatment in multifocal and diffuse infantile hepatic haemangioma (IHH). Methods: A retrospective study of symptomatic or potentially symptomatic IHH was performed in our hospital between 2011 and 2016. Results: Thirteen patients were identified: 2 patients had diffuse lesions, and 11 patients had multifocal lesions, including 2 patients who had combined lesions that shared features of both multifocal and diffuse lesion patterns. Eleven (84.6%) patients had cutaneous infantile haemangioma. Hepatomegaly was the predominant clinical presentation. Hypothyroidism was identified in three patients, including one patient who had documented congestive heart failure (CHF). The median age at diagnosis and the median duration of treatment were 2.0 months (range 1.2-26.0) and 24.0 months (range 4.0-30.0). The median duration of follow-up was 30.0 months (range 3.0-48.0). For patients with hypothyroidism, the thyroid hormone level was normal after 4 weeks of propranolol and levothyroxine treatment. All but one patient responded well to propranolol treatment. The patient who failed to respond to treatment died of CHF and abdominal compartment syndrome induced by hepatomegaly. No significant side effects of propranolol were observed during follow-up. Conclusions: Most multifocal and diffuse IHH respond well to propranolol. However, progressive cases may be fatal despite aggressive treatments. Our data suggest that propranolol may be considered the first-line treatment for multifocal and diffuse IHH due to its efficacy.

Background Kaposiform haemangioendothelioma (KHE) is a rare, locally aggressive disorder. The presenting and imaging features of KHE can overlap with other vascular anomalies and tumours. We aimed to analyse the imaging findings of KHE disorder and highlight features most suggestive of this diagnosis. Methods The clinical features and imaging findings were retrospectively reviewed in 64 patients with pathological diagnosis of KHE. Results Of the 64 patients diagnosed with KHE, 36 patients were < 6 months and 28 patients were ≥ 6 months. The most common presenting features were Kasabach-Merritt phenomenon (KMP, 42.2 %), visible cutaneous lesions (90.6 %), oedema or swelling (43.8 %) and destructive changes or remodelling of adjacent bone (42.2 %). Compared with patients in the group ≥ 6 months, patients in the group < 6 months have higher odds of KMP (P = 0.000), infiltrative lesion with ill-defined borders (P = 0.044). The group ≥ 6 months have higher odds of destructive changes or remodelling of adjacent bone (P = 0.002). In all patients, the lesions in all of the 64 patients were hypointense or isointense compared with muscle on T1-weighted sequences, and hyperintense on T2-weighted or inversion-recovery sequences, nine patients (14.1 %) showed vascularity. There were 28 patients (43.8 %) with characteristic enhancing and infiltrative soft-tissue thickening. Conclusions Presence of visible cutaneous lesions with ill-defined borders, destructive changes or remodelling of adjacent bone, severe thrombocytopenia and consumptive coagulopathy should favour the diagnosis of KHE.

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Kasabach-Merritt phenomenon (KMP) occurred uniquely in patients with kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). We report the clinical characteristics of two patients with KHE involving the right upper arm. The patients demonstrated rapid enlargement of the lesion with severe KMP shortly after vaccination. Sirolimus was used to treat the KHE with KMP. The patients showed a quick normalization of the platelet level. The follow-up examination revealed that the size of the mass was significantly decreased. This report raises the intriguing possibility that extrinsic factors may contribute to the development of KMP in the context of an already existing KHE.