Diagnosis Of Kaposiform Hemangioendothelioma Research Articles

Prenatal detection of Kaposiform Hemangioendothelioma with Kasabach–Merritt phenomenon: a case report

BackgroundKaposiform Hemangioendothelioma is a rare vascular neoplasm that typically presents in neonates and infancy. Most of the cases of Kaposiform Hemangioendothelioma are reported in neonates (60%) and infancy (93%), and very few cases have been reported prenatally. We describe here a case which was diagnosed prenatally in the 3rd trimester with rapid growth of upper limb and appearance of non-immune hydrops.Case presentationA 25-year-old primigravida, an antenatally booked case, presented for routine third trimester level III scan at 34-week gestation. Level I scan for nasal bone and nuchal translucency at 12 weeks and level II anomaly scan at 20 weeks were normal. Level III ultrasound scan revealed increased echogenicity and hypertrophy of soft tissues of right upper limb and trunk involving both the subcutaneous and muscular compartments. Fetal middle cerebral artery Doppler, echocardiography and magnetic resonance imaging revealed fetal anemia, cardiac failure, and non-immune hydrops. These findings with hemodynamic changes and rapid evolution were suggestive of Kaposiform Hemangioendothelioma with Kasabach–Merritt phenomenon.ConclusionThe diagnosis of Kaposiform Hemangioendothelioma should be suspected in prenatal period if there is unilateral hypertrophy of limb which has evolved rapidly over a short interval of time and shows findings suggestive of Kasabach–Merritt Phenomenon. Prenatal presentation is life threatening if it is associated with poor fetal hemodynamics. Prenatal counseling in such cases should include advice on termination of pregnancy with emphasis on neonatal management.

Comprehensive Therapy for Infant Vascular Tumor Associated With Kasabach-Merritt Phenomenon-Single-Center Primary Experience.

ObjectiveTo introduce our single-center experience of infant vascular tumor associated with Kasabach–Merritt phenomenon (KMP) which received combined medicine treatment with intralesional laser photocoagulation (ILP) and sclerotherapy.MethodsA retrospective study was conducted using medical records of all children with a diagnosis of kaposiform hemangioendothelioma (KHE) or tufted angioma (TA) associated with KMP treated with medicine, intralesional laser photocoagulation (ILP), and sclerotherapy between February 2017 and November 2020. Clinical features, response to comprehensive therapy, and outcomes were recorded.ResultsA total of 23 patients including nine females (39%) and 14 males (61%) were identified. The mean age was 6.9 months (age range, 11 days−2 years) at the time of treatment. Nine children (39%) demonstrated sensitivity to single corticosteroid therapy; 14 children (61%) received combined therapy with intravenous Vincristine (VCR) and corticosteroid therapy. All children had at least two ILP and sclerotherapy performed, with a mean of 3.5 procedures (range: 2–6). Of these 14 children, only one experienced a relapse of thrombocytopenia and the remaining 13 children had no clinical symptoms recurred.ConclusionThe combined therapy modalities could induce a more rapid tumor response and resolution of KMP and decrease the rebound rates. This research presents a novel and safe multi-modality treatment for infant vascular tumors associated with KMP.

Congenital kaposiform hemangioendothelioma: don’t let the appearances fool you

Abstract Objectives To describe challenges in diagnosis and treatment of congenital Kaposiform hemangioendothelioma (KHE). Case presentation The neonate was born with an upper extremity vascular tumor and stable clinical state. His biopsy was inconclusive before discharge. At three weeks follow-up he presented with Kasabach-Merritt phenomenon (KMP), leading to the diagnosis of KHE, a rare locally aggressive vascular tumor that was managed with combination of steroids and mammalian target of rapamycin inhibitor (mTOR). Conclusions KMP in combination with the lesion biopsy can enhance KHE diagnosis, leading to successful treatment with mTOR inhibitor.

A not so harmless mass: Kaposiform hemangioendothelioma complicated by a Kasabach–Merritt phenomenon

A vascular mass localized in the face and the neck was displayed by ultrasonography in a 38-week-old male fetus. At birth, the mass was bulky and purplish. The newborn breathed spontaneously but with severe desaturation. During laryngoscopy, we observed an obstruction of the larynx with a left-shift caused by the hemorrhagic mass. Blood analysis revealed anemia, severe thrombocytopenia, and coagulation disorders. The diagnosis of kaposiform hemangioendothelioma (KHE) complicated by a Kasabach–Merritt phenomenon (KMP) was put forward and treatment with propranolol, corticoids, and vincristine was initiated. Platelets were transfused daily for 8 days but did not resolve the thrombocytopenia. At day 8, we added sirolimus to the treatment and noted a rapid response with the normalization of the platelet count within 1 week and a significant regression of the mass. In this paper, we review the clinical and biological features of hemangioendothelioma associated with KMP and discuss its current and future treatment. Sirolimus seems to be very promising.

Musculoskeletal complication in kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon: clinical characteristics and management.

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.

Clinical analysis on 13 cases of Kaposiform hemangioendothelioma

Objective To explore the diagnosis and treatment of Kaposiform hemangioendothelioma (KHE), deepen the understanding of KHE and Kasabach-Merritt phenomenon (KMP), and discuss the optimal treatment for KHE. Methods From January 2008 to August 2016, 13 cases of KHE were confirmed by surgery or biopsy pathology and admitted to the First Affiliated Hospital of Sun Yat-Sen University, and the clinical diagnosis and treatment were analyzed retrospectively. Results There were 13 patients(7 males and 6 females) with a median age of 1.0 years(0.2-10.0 years), and 84.6%(11/13 cases) were infants and young children, 76.9% (10/13 cases) involved with deep tissue, 23.1% (3/13 cases) were associated with KMP and they were younger than 1 year old, and 15.4% (2/13 cases) coexisted with hemangioma or lymphangioma.The location, extent and infiltration depth of the lesion were observed by imaging examinations and histopathology showed nodule shaped spindle tumor cells.Radical resection was considered if possible.Dose of Vincristine (0.5 mg/m2 weekly) and Propranolol [1 mg/(kg·d)] were administered.The prognosis was different in thirteen cases undergoing different treatments.After 3 months to 9 years follow-up, 41.7% (5/12 cases) survived after tumor treatment. Conclusions KHE happens mostly in infants and young children, with varying clinical manifestations and a high recurrence rate.The diagnosis of KHE is based on histological examination, computed tomography and magnetic resonance imaging while it still need explicit pathological diagnosis is needed.KHE may be accompanied by hemangioma or lymphangioma.Prognosis is affected by many factors and the comprehensive treatment is required.KMP should be remedied preferentially, individual treatment protocol and long term follow-up are necessary. Key words: Kaposiform hemangioendothelioma; Kasabach-Merritt phenomenon; Diagnosis; Treatment; Analysis

Long-term outcome of vincristine-aspirin-ticlopidine (VAT) therapy for vascular tumors associated with kasabach-merritt phenomenon

. This study aimed to clarify the combinatorial treatment effect of agents as aspirin and ticlopidine associated with vincristine in the management of Kasabach-Merritt phenomenon (KMP), a severe thrombocytopenic coagulopathy that occurs in the presence of an enlarging vascular tumor such as kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). . A retrospective review was conducted of medical records of all children with diagnosis of KHE or TA associated with KMP treated with vincristine-aspirin-ticlopidine (VAT) therapy at two different institutions in the same country from 1994 to 2011. Clinical features, response to VAT therapy and outcomes were recorded. . Eleven patients (mean age 11 months, range 0-36), including seven females (64%) and four males (36%), were identified. Seven patients underwent incisional biopsy and two different histologies were found, KHE in four patients and TA in three patients. Tumors were located in the head and neck (n = 5), chest wall (n = 2), arm (n = 2) and retroperitoneum (n = 2). Mean platelet level was 10,200/mm(3) (range 4,000-21,000). A plaque-like lesion with ecchymosis was the most common cutaneous manifestation (63%). All patients underwent VAT therapy. Mean duration of treatment was 3.9 months for vincristine, 13.9 months for aspirin, and 13.4 months for ticlopidine. All patients are alive with a mean follow-up of 4.5 years (range, 2-17). . Antiaggregant therapy is helpful in combination with vincristine in the treatment of KMP associated with KHE and TA. Prognosis is excellent if severe thrombocytopenia is controlled despite failure in reduction of tumor size.

Kaposiform Hemangioendothelioma with Kasabach-Merritt phenomenon: Successful Treatment with Vincristine and Ticlopidine

Sir, Kaposiform hemangioendothelioma is a rare vascular tumor that often develops Kasabach-Merritt phenomenon. The treatment of this situation is a challenge that is not succesfully solved many times. A 24-d-old-boy was referred from another hospital, for evaluation of a congenital facial tumor. He was born after an uneventful pregnancy via normal spontaneous vaginal delivery. On physical examination, a firm, poorly demarcated, violaceous, warm tumor involved the left cheek area was found (Fig. 1). The lesion progressively enlarged during 4 wk after birth causing upper airway obstruction. Endotracheal intubation followed by tracheostomy was needed. His hemoglobin level at presentation was 9.0 g/dL, and platelets were 38×10/μL. Platelet count decreased down to 16×10/μL, developing a thrombocytopenic consumption coagulopathy, with hypofibrinogenemia (fibrinogen level, 61 mg/dL (reference range, 170–420 mg/dL), that required treatment with endovenous fibrinogen (30mg/kg/daily). Levels of D-dimers >2000 ng/mL (reference range, <215 ng/mL). The diagnosis of Kaposiform hemangioendothelioma (KHE) with Kasabach-Merritt phenomenon (KMP) was made basing on clinical and laboratory findings. Biopsy of the tumor could not be performed because of the potential for hemorrhage. We implemented a therapeutic regimen with endovenous prednisone (5 mg/kg daily) and vincristine (0.05 mg/kg/ wkly), oral ticlopidine plus aspirin (10 mg/kg/daily). Within 3 wk of treatment the platelet count normalized to 120×10/ μL and the tumor considerably diminished in size. After 3 mo follow-up, the patient was still doing well, with normal platelet counts, a minimal residual vascular lesion (Fig. 2), and 100% performance status. Vincristine was maintained during 11 mo until the stability of the lesion was achieved. At the present time the patient is not receiving any treatment and there is no recurrence after 18 mo of follow-up. KHE is an infrequent vascular neoplasm that usually appears in infancy or can present at birth. The skin is the most frequently affected location followed by soft tissue, and bone infiltration due to its locally aggressive behavior [1, 2]. Consumption coagulopathy (KMP) is a frequent expression of this neoplasm. Its presence should arise the suspicion of this vascular tumor [3]. The prognosis depends on tumor location, size, presence of deeper tissues R. Barabash-Neila (*) : J. Bernabeu-Wittel Pediatric Dermatology Unit, Virgen del Rocio Universitary Hospital, Avda. Manuel Siurot, s/n, 41013 Sevilla, Spain e-mail: romanbarabash@hotmail.com

Kaposiform hemangioendothelioma in tonsil of a child associated with cervical lymphangioma: a rare case report

Kaposiform hemangioendothelioma (KHE) is an uncommon vascular tumor of intermediate malignant potential, usually occurs in the extremities and retroperitoneum of infants and is characterized by its association with lymphangiomatosis and Kasabach-Merritt phenomenenon (KMP) in certain cases. It has rarely been observed in the head and neck region and at times, can present without KMP. Herein, we present an extremely uncommon case of KHE occurring in tonsil of a child, associated with a neck swelling, but unassociated with KMP. A 2-year-old male child referred to us with history of sore throat, dyspnoea and right-sided neck swelling off and on, since birth, was clinicoradiologically diagnosed with recurrent tonsillitis, including right sided peritonsillar abscess, for which he underwent right-sided tonsillectomy, elsewhere. Histopathological sections from the excised tonsillar mass were reviewed and showed a tumor composed of irregular, infiltrating lobules of spindle cells arranged in kaposiform architecture with slit-like, crescentic vessels. The cells displayed focal lumen formation containing red blood cells (RBCs), along with platelet thrombi and eosinophilic hyaline bodies. In addition, there were discrete foci of several dilated lymphatic vessels containing lymph and lymphocytes. On immunohistochemistry (IHC), spindle cells were diffusely positive for CD34, focally for CD31 and smooth muscle actin (SMA), the latter marker was mostly expressed around the blood vessels. Immunostaining for HHV8 was negative and Ki-67 (proliferation marker) displayed focal positivity. Diagnosis of KHE was made. Platelet count was towards lower side of range. Postoperative imaging showed discrete, multiple fluid containing lesions in the right neck that were high on T2-weighed sequences, on magnetic resonance imaging (MRI) and ipsilateral intraoral mucosal growth. Fine needle aspiration cytology (FNAC) smears from neck swelling showed blood, fluid and lymphocytes. Possibility of a coexisting lymphangioma was considered. The patient was offered sclerotherapy and is on follow-up. This case forms the second documented case of KHE at this site, along with its unique association with neck lymphangioma. KHE has distinct histopathological features and can be sorted out from its other differentials like juvenile hemangioma and Kaposi's sarcoma. IHC stains are useful in substantiating a definite diagnosis.