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1.
Diagn Pathol ; 18(1): 25, 2023 Feb 20.
Artigo em Inglês | MEDLINE | ID: mdl-36803395

RESUMO

OBJECTIVES: Pseudomyogenic hemangioendothelioma (PHE) is a rare intermediate hemangioendothelioma. This article aims to study the clinicopathological features of PHE. METHODS: We collected the clinicopathological features of 10 new PHE, and examined their molecular pathological features by fluorescence in situ hybridization. In addition, we summarized and analyzed the pathological data of 189 reported cases. RESULTS: The case group consisted of six men and four women aged 12-83 years (median: 41 years). Five instances occurred in the limbs, three in the head and neck, and two in the trunk. Tumor tissues were composed of spindle cells and round or polygonal epithelioid cells, which were either arranged in sheets or interwoven, along with areas of transitional morphology. Scattered or patchy stromal neutrophil infiltration was observed. Tumor cells had abundant cytoplasm, and some contained vacuoles. The nuclei had mild to moderate atypia, with visible nucleoli, and mitosis was rare. PHE tissues diffusely expressed CD31 and ERG, but not CD34, Desmin, SOX-10, HHV8 or S100, while some samples expressed CKpan, FLI-1 and EMA. INI-1 stain is retained. The proliferation index of Ki-67 is 10-35%. Seven samples were detected by fluorescence in situ hybridization, six of which had breakages in FosB proto-oncogene (AP-1 transcription factor subunit). Two patients experienced recurrence; however, no metastasis or death occurred. CONCLUSIONS: PHE is a rare soft tissue vascular tumor, which has biologically borderline malignant potential, local recurrence, little metastasis, and good overall survival and prognosis. Immunomarkers and molecular detection are valuable for diagnosis.


Assuntos
Hemangioendotelioma Epitelioide , Hemangioendotelioma , Hemangioma , Lesões Pré-Cancerosas , Neoplasias de Tecidos Moles , Feminino , Humanos , Masculino , Biomarcadores Tumorais/genética , Hemangioendotelioma/diagnóstico , Hemangioendotelioma/patologia , Hemangioendotelioma Epitelioide/diagnóstico , Hemangioendotelioma Epitelioide/genética , Hemangioendotelioma Epitelioide/patologia , Hibridização in Situ Fluorescente , Neoplasias de Tecidos Moles/patologia , Criança , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais
3.
Artigo em Inglês | MEDLINE | ID: mdl-36688881

RESUMO

Kaposiform hemangioendothelioma is a locally invasive tumor and we were unable to find any previous reports of multifocal progression. Sirolimus, a mammalian target of rapamycin inhibitor, has been widely used to treat kaposiform hemangioendothelioma. Herein, we report a case of multifocal progressive kaposiform hemangioendothelioma, wherein sirolimus treatment caused severe thrombocytopenia. A 12-year-old East Asian girl presented with indurated dark-purple masses on her back. The patient had received three surgical interventions following the first appearance of the masses in 2012 and subsequent reappearances in 2014 and 2016. Kaposiform hemangioendothelioma was diagnosed based on radiological and pathological findings. Two more masses appeared in the following year. The patient was treated with oral sirolimus (2.5 mg/ m2/day) and developed grade 3 thrombocytopenia 8 days later. The patient was uneventfully relieved 5 days later after the withdrawal of sirolimus and the administration of appropriate medications. This rare case indicated that kaposiform hemangioendothelioma could be progressive with local metastatic characteristics in children. Besides, the severe sirolimus-induced complication highlights the importance of serum drug level monitoring during treatment. Physicians should be extremely cautious while treating kaposiform hemangioendothelioma patients with sirolimus.


Assuntos
Anemia , Hemangioendotelioma , Síndrome de Kasabach-Merritt , Sarcoma de Kaposi , Criança , Feminino , Humanos , Anemia/induzido quimicamente , Hemangioendotelioma/diagnóstico , Hemangioendotelioma/tratamento farmacológico , Síndrome de Kasabach-Merritt/diagnóstico , Síndrome de Kasabach-Merritt/tratamento farmacológico , Sarcoma de Kaposi/induzido quimicamente , Sarcoma de Kaposi/diagnóstico , Sarcoma de Kaposi/tratamento farmacológico , Sirolimo/efeitos adversos
4.
Rev. esp. enferm. dig ; 115(1): 46-47, 2023. ilus
Artigo em Inglês | IBECS | ID: ibc-214678

RESUMO

A 18-year-old man presented with multiple asymptomatic masses in the spleen that had been detected on ultrasonography performed during a physical screening. He had no history of tuberculosis, and was otherwise well. Abdominal MR demonstrated multiple masses with internal stellate scars, which appeared as marked hypointensity on T2WI and contrast-enhanced MR. Most lesions showed inhomogeneous enhancement. The capsular enhancement was also revealed at delay phase. The patient underwent laparoscopic splenectomy. Pathological examination indicated papillary intralymphatic angioendothelioma (PILA), with the following immunohistochemistry results: CK (-), CR (-), ERG (+), CD34 (+), CD31 (+), D2-40 (+), Ki67 (3%+). The patient was feeling well at 6 months of follow-up (AU)


Assuntos
Humanos , Masculino , Adolescente , Neoplasias Esplênicas/diagnóstico , Hemangioendotelioma/diagnóstico , Sistema Linfático/patologia , Imageamento por Ressonância Magnética , Imuno-Histoquímica
5.
Skeletal Radiol ; 52(1): 119-127, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-35780259

RESUMO

Pseudomyogenic hemangioendothelioma (PMH) is a rare vascular tumor that occurs in young mostly male patients. Seventy percent of PMH cases are multifocal and 25% involve bones. PMH is an indolent tumor with mild local aggressiveness and an unclear pathology. Only two cases of spontaneous regressive bone PMH have been reported. Here, we report the case of a 17-year-old boy with a multifocal bone PMH diagnosed from a chronic pain in his left knee. The PMH affected the right scapula, both humeri, the right olecranon, the second metacarpal bone, the second and fourth right ribs, the thoracic and lumbar spine, the pelvic ring, the left and right femoral neck, and the left patella. Every lesion presented with a lobulated, lytic pattern, sometimes with a peripheral sclerotic rim. MRI showed a tissue lesion with a low intensity on T1-weighted sequences and high intensity on T2-weighted sequences. Enhancement of T1 gadolinium fat-saturated sequences was bright. After discussion, a national specialized board decided to actively monitor the patient and start general chemotherapy in the case of progression. The disease was stable at 3 and 6 months and showed signs of regression at 1 year, which was further confirmed at 2 years. CT scan and MRI highlighted a progressive filling of the tumor with cancellous bone and a regression of the tissue contingent. This case report highlights to a new therapeutic approach for indolent PMH that does not prevent further treatment in the case of progression.


Assuntos
Hemangioendotelioma , Hemangioma , Neoplasias Vasculares , Humanos , Masculino , Adolescente , Feminino , Hemangioendotelioma/diagnóstico por imagem , Hemangioendotelioma/patologia , Patela/patologia , Imageamento por Ressonância Magnética
6.
BMC Geriatr ; 22(1): 952, 2022 12 09.
Artigo em Inglês | MEDLINE | ID: mdl-36494779

RESUMO

BACKGROUND: Composite hemangioendothelioma (CHE) is an intermediate group of tumors with features between hemangioma and angiosarcoma both histologically and biologically. CHE is predominant in young and middle-aged adults, but very infrequently affects the spine. We describe the case of primary CHE in the cervical spine exhibiting kaposiform hemangioendothelioma (KHE)-like components that was associated with cervical myelopathy with vertebral body destruction in an elderly woman. We retrospectively reviewed the case of a primary cervical spinal tumor, diagnosed as CHE with KHE-like components in pathological findings, associated with cervical myelopathy and cervical vertebral body destruction. CASE PRESENTATION: An 80-year-old woman presented with progressive cervical myelopathy caused by a cervical spine tumor. Preoperative cervical MRI revealed a neoplastic lesion invading the cervical spine that strongly compressed the spinal cord, causing right upper-limb paralysis. We performed partial tumor resection along with posterior decompression and fixation. Postoperatively, pathological findings showed that the tumor was CHE with KHE-like features. Following radiotherapy, no recurrences have been observed in 21 months. CONCLUSIONS: This is the first report of CHE with features of KHE in the spine of an elderly patient. Posterior decompression and fusion of the cervical spine and subsequent radiotherapy resulted in a good outcome.


Assuntos
Hemangioendotelioma , Síndrome de Kasabach-Merritt , Idoso , Feminino , Humanos , Pessoa de Meia-Idade , Idoso de 80 Anos ou mais , Estudos Retrospectivos , Hemangioendotelioma/diagnóstico , Hemangioendotelioma/diagnóstico por imagem , Síndrome de Kasabach-Merritt/complicações , Vértebras Cervicais/diagnóstico por imagem , Vértebras Cervicais/cirurgia , Vértebras Cervicais/patologia
7.
Biomolecules ; 12(12)2022 12 08.
Artigo em Inglês | MEDLINE | ID: mdl-36551267

RESUMO

BACKGROUND: Vascular anomalies comprise a diverse group of rare diseases with altered blood flow and are often associated with coagulation disorders. The most common example is a localized intravascular coagulopathy in venous malformations leading to elevated D-dimers. In severe cases, this may progress to a disseminated intravascular coagulopathy with subsequent consumption of fibrinogen and thrombocytes predisposing to serious bleeding. A separate coagulopathy is the Kasabach-Merritt phenomenon in kaposiform hemangioendothelioma characterized by platelet trapping leading to thrombocytopenia and eventually consumptive coagulopathy. Our previous work showed impaired von Willebrand factor and platelet aggregometry due to abnormal blood flow, i.e., in ventricular assist devices or extracorporeal membrane oxygenation. With altered blood flow also present in vascular anomalies, we hypothesized that, in particular, the von Willebrand factor parameters and the platelet function may be similarly impacted. METHODS: We prospectively recruited 73 patients with different vascular anomaly entities and analyzed their coagulation parameters. RESULTS: Acquired von Willebrand syndrome was observed in both of our patients with Kasabach-Merritt phenomenon. In six out of nine patients with complex lymphatic anomalies, both the vWF antigen and activity were upregulated. Platelet aggregometry was impaired in both patients with Kasabach-Merritt phenomenon and in seven out of eight patients with an arteriovenous malformation. CONCLUSIONS: The analysis of coagulation parameters in our patients with vascular anomalies advanced our understanding of the underlying pathophysiologies of the observed coagulopathies. This may lead to new treatment options for the, in part, life-threatening bleeding risks in these patients in the future.


Assuntos
Transtornos da Coagulação Sanguínea , Coagulação Sanguínea , Malformações Vasculares , Humanos , Plaquetas , Coagulação Intravascular Disseminada/etiologia , Coagulação Intravascular Disseminada/fisiopatologia , Hemangioendotelioma/etiologia , Hemangioendotelioma/fisiopatologia , Síndrome de Kasabach-Merritt/etiologia , Síndrome de Kasabach-Merritt/fisiopatologia , Fator de von Willebrand/metabolismo , Malformações Vasculares/complicações , Malformações Vasculares/fisiopatologia , Transtornos da Coagulação Sanguínea/etiologia , Transtornos da Coagulação Sanguínea/fisiopatologia
9.
Pediatrics ; 150(5)2022 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-36193691

RESUMO

Kaposiform hemangioendothelioma is classified as a locally aggressive vascular tumor of childhood resulting from abnormal angiogenesis and lymphangiogenesis. Most commonly, KHE presents as a single tissue mass, ranging from an erythematous papule to a violaceous indurated tumor. Definitive diagnosis requires tissue sampling with the demonstration of ill-defined nodules and fascicles of spindle-shaped D2-40 positive endothelial cells, forming slit-like vascular channels. This newborn presented with multifocal cutaneous Kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon confirmed on histopathology with immunostaining.


Assuntos
Hemangioendotelioma , Síndrome de Kasabach-Merritt , Sarcoma de Kaposi , Recém-Nascido , Humanos , Síndrome de Kasabach-Merritt/diagnóstico , Síndrome de Kasabach-Merritt/complicações , Células Endoteliais , Hemangioendotelioma/diagnóstico , Hemangioendotelioma/complicações , Sarcoma de Kaposi/diagnóstico , Sarcoma de Kaposi/complicações
10.
J Pediatr Endocrinol Metab ; 35(12): 1560-1564, 2022 Dec 16.
Artigo em Inglês | MEDLINE | ID: mdl-36190305

RESUMO

OBJECTIVES: Consumptive hypothyroidism may occur in hepatic hemangioendothelioma. The altered expression of deiodinases inactivates peripheral thyroid hormones. As a result, serum levels of free triiodothyronine and free thyroxine are reduced to varying degrees. There are no established recommendations for the dosage of sirolimus for this particular indication. We describe for the first time the course of treatment with low-dose sirolimus. CASE PRESENTATION: We present a 5-week-old infant with hepatic hemangioendothelioma and severe consumptive hypothyroidism. Due to hepatic infiltration he showed signs of right heart strain. Therapy of hemangioendothelioma was initiated with propranolol and, in the absence of response, methylprednisolone was added. Treatment was continued with low-dose sirolimus (due to side effects) and propranolol. Hypothyroidism was managed with levothyroxine and liothyronine. CONCLUSIONS: Consumptive hypothyroidism due to cutaneous hemangioma and hepatic hemangioendothelioma can be managed with propranolol and low-dose sirolimus. Treatment of severe hypothyroidism may require a combinational therapy by substitution of both T3 and T4.


Assuntos
Hemangioendotelioma , Hipotireoidismo , Neoplasias Hepáticas , Lactente , Masculino , Humanos , Propranolol/uso terapêutico , Neoplasias Hepáticas/complicações , Hipotireoidismo/complicações , Hipotireoidismo/tratamento farmacológico , Tiroxina , Hemangioendotelioma/complicações , Hemangioendotelioma/tratamento farmacológico , Tri-Iodotironina , Hormônios Tireóideos/uso terapêutico , Sirolimo/uso terapêutico
11.
Dermatol Clin ; 40(4): 401-423, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-36243428

RESUMO

Vascular tumors are classified into three categories by the International Society for the Study of Vascular Anomalies (ISSVA): benign, locally aggressive/borderline, and malignant. Many of these tumors are rare, cutaneous in nature, and present in childhood. The characterization and delineation of these distinct vascular tumors is an evolving area of clinical research. The diagnosis of these lesions relies on history and clinical presentation, location, histologic appearance, immunohistochemistry, and more recently, associated genetic mutations. This article provides a brief, yet comprehensive overview of all cutaneous vascular tumors currently recognized by the ISSVA, including presentation, diagnosis, and treatment.


Assuntos
Granuloma Piogênico , Hemangioendotelioma , Hemangioma , Malformações Vasculares , Neoplasias Vasculares , Granuloma Piogênico/diagnóstico , Hemangioendotelioma/patologia , Hemangioma/diagnóstico , Hemangioma/terapia , Humanos , Malformações Vasculares/diagnóstico , Malformações Vasculares/terapia , Neoplasias Vasculares/diagnóstico , Neoplasias Vasculares/patologia , Neoplasias Vasculares/terapia
12.
Rev Esp Patol ; 55(4): 254-258, 2022.
Artigo em Espanhol | MEDLINE | ID: mdl-36154733

RESUMO

Epithelioid haemangioendothelioma is a rare vascular tumor, first described in 1975 by Dail and Liebow as a bronchioloalveolar carcinoma. Although it usually behaves like a low-grade neoplasm, cases have been reported in which the tumor shows a high grade of malignancy, spreading rapidly throughout the body. We present the case of a 41-year-old man with dermatosis in the left thigh with rapid extension to the abdomen; the initial differential diagnoses were metastatic carcinoma versus lymphoma. When the histopathology was re-examined, a diagnosis of skin epithelioid hemangioendothelioma was confirmed and treatment with radiotherapy was initiated. This tumour rarely affects the skin; there are only a few previously reported cases.


Assuntos
Hemangioendotelioma Epitelioide , Hemangioendotelioma , Neoplasias Cutâneas , Adulto , Criança , Diagnóstico Diferencial , Hemangioendotelioma/diagnóstico , Hemangioendotelioma Epitelioide/diagnóstico , Humanos , Masculino
13.
J Card Surg ; 37(11): 3867-3869, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36073116

RESUMO

Composite hemangioendothelioma (CHE) is a rare vascular tumor which shows varying combination of benign, low-grade, and malignant vascular components on pathology. CHE is usually located on the surface of the dermis and subcutaneous tissue of the extremities. We report an unusual case of CHE in the heart.


Assuntos
Calcinose , Neoplasias Cardíacas , Hemangioendotelioma , Mixoma , Neoplasias Vasculares , Calcinose/diagnóstico por imagem , Calcinose/cirurgia , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/cirurgia , Hemangioendotelioma/diagnóstico , Hemangioendotelioma/patologia , Hemangioendotelioma/cirurgia , Humanos , Mixoma/diagnóstico por imagem , Mixoma/cirurgia , Neoplasias Vasculares/patologia
14.
Pediatr Blood Cancer ; 69 Suppl 3: e29353, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-36070211

RESUMO

Some vascular anomalies can present with challenging hematologic aberrations. Kaposiform hemangioendothelioma (KHE) may be complicated with Kasabach-Merritt phenomenon (KMP) and stagnant blood flow in slow-flow malformations can promote activation and consumption of coagulation factors, which results in bleeding and clotting known as localized intravascular coagulopathy (LIC). These patients can experience significant morbidity secondary to pain due to thrombosis and are at higher risk of hematologic complications during surgical procedures. No standard of care has been established to prevent or manage these complications. This review focuses on the management of coagulopathy in children and adults with vascular anomalies.


Assuntos
Transtornos da Coagulação Sanguínea , Hemangioendotelioma , Síndrome de Kasabach-Merritt , Sarcoma de Kaposi , Malformações Vasculares , Adulto , Transtornos da Coagulação Sanguínea/etiologia , Transtornos da Coagulação Sanguínea/terapia , Criança , Hemangioendotelioma/complicações , Hemangioendotelioma/terapia , Humanos , Malformações Vasculares/complicações , Malformações Vasculares/terapia
15.
Medicine (Baltimore) ; 101(34): e30296, 2022 Aug 26.
Artigo em Inglês | MEDLINE | ID: mdl-36042656

RESUMO

This study aimed to in the management of Kasabach-Merritt phenomenon (KMP), a severe thrombocytopenic coagulopathy that occurs in the presence of an enlarging vascular tumor. Here, we retrospectively evaluated 12 patients with KMP in Guangzhou Women and Children's Medical Center, Guangzhou Medical University, from 2017 to 2021. 12 patients, including 7 females and 5 males, were identified. Tumors were located in the leg (n = 4), neck (n = 1), face (n = 3), chest wall (n = 1), back (n = 2), and retroperitoneum (n = 1). A plaque-like lesion with ecchymosis was the most common cutaneous manifestation. All the patients underwent embolization therapy. Nine patients received steroid treatment and 7 patients were administered with sirolimus. The mean duration of treatment was 1.6 months. All the patients reported in this study were alive when discharged. Embolization combined with steroid and sirolimus appears effective in patients with KMP, as well as in those who experienced disease recurrence. However, a long-term follow-up of the children cured of KMP will be necessary to monitor its recurrence and improve the outcome.


Assuntos
Hemangioendotelioma , Síndrome de Kasabach-Merritt , Sarcoma de Kaposi , Criança , Terapia Combinada , Feminino , Humanos , Lactente , Síndrome de Kasabach-Merritt/tratamento farmacológico , Síndrome de Kasabach-Merritt/patologia , Masculino , Recidiva Local de Neoplasia , Estudos Retrospectivos , Sirolimo
16.
J Cancer Res Ther ; 18(3): 817-819, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35900565

RESUMO

Pseudomyogenic hemangioendothelioma (PMH) is a rare tumor of vascular origin with intermediate malignant potential which commonly presents as a subcutaneous and soft-tissue mass with or without concurrent bone involvement. However, PMH presenting as primary multifocal bone lesions is rare. Histomorphologically, it mimicks other epithelioid tumors and cytokeratin expression in PMH can prompt an erroneous diagnosis of metastatic carcinoma, especially in an elderly patient. Diligent histopathological examination and judicious immunohistochemistry panel can guide to the correct diagnosis. Due to its rarity, the optimal therapeutic strategy has not been established till date. We present a rare case of PMH of primary bone with multifocal bony disease in a 23-year-old male who presented with severe bone pains. The patient has been managed with four weekly denosumab, and the disease is stable with symptomatic relief after 6 months.


Assuntos
Denosumab , Hemangioendotelioma Epitelioide , Adulto , Idoso , Denosumab/uso terapêutico , Hemangioendotelioma/patologia , Hemangioendotelioma Epitelioide/diagnóstico , Hemangioendotelioma Epitelioide/tratamento farmacológico , Hemangioendotelioma Epitelioide/patologia , Hemangioma , Humanos , Imuno-Histoquímica , Masculino , Lesões Pré-Cancerosas , Adulto Jovem
17.
Arch Soc Esp Oftalmol (Engl Ed) ; 97(7): 413-416, 2022 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-35779898

RESUMO

Intravascular papillary endothelial hyperplasia (IPEH), also known as "vegetant intravascular haemangioendothelioma", is a rare benign proliferation of vascular endothelial cells secondary to intravascular thrombosis and thrombus organisation. It can develop from vascular lesions such as haemangiomas, pyogenic granulomas or varicose veins. This vascular tumour of the skin and subcutaneous tissue may exhibit rapid and progressive growth, and is usually located in the neck or head. Ocular presentation is unusual and orbital involvement is even rarer. We report a case of a preterm newborn male with a rapidly growing left orbital mass that was histologically diagnosed as intravascular papillary endothelial hyperplasia. He was successfully managed with total excision of the tumour and propranolol therapy and remains recurrence free after an eight-year follow-up.


Assuntos
Hemangioendotelioma , Trombose , Diagnóstico Diferencial , Células Endoteliais/patologia , Hemangioendotelioma/diagnóstico , Hemangioendotelioma/patologia , Humanos , Hiperplasia/patologia , Recém-Nascido , Masculino , Trombose/diagnóstico , Trombose/patologia
18.
Arch. Soc. Esp. Oftalmol ; 97(7): 413-416, jul. 2022. ilus
Artigo em Espanhol | IBECS | ID: ibc-209075

RESUMO

La hiperplasia endotelial papilar intravascular (IPEH), también conocida como «hemangioendotelioma vegetante intravascular», es una proliferación benigna y rara de células endoteliales vasculares, secundaria a trombosis intravascular u organización del trombo. Puede desarrollarse a partir de lesiones vasculares como hemangiomas, granulomas piógenos o várices. Este tumor vascular de la piel y del tejido subcutáneo puede presentarse como crecimiento rápido y progresivo, y es usualmente localizado en cuello o cabeza. La presentación ocular es inusual y la afectación orbitaria es todavía más rara. Presentamos un caso de un varón recién nacido pretérmino, el cual presentó una masa orbitaria izquierda de crecimiento rápidamente progresivo y se obtuvo el diagnóstico por histología de hiperplasia endotelial papilar intravascular. El paciente fue exitosamente tratado mediante una escisión total del tumor y terapia con propranolol. Actualmente, después de un seguimiento de ocho años, ha permanecido sin recurrencias (AU)


Intravascular papillary endothelial hyperplasia (IPEH), also known as “vegetant intravascular haemangioendothelioma”, is a rare benign proliferation of vascular endothelial cells secondary to intravascular thrombosis and thrombus organisation. It can develop from vascular lesions such as haemangiomas, pyogenic granulomas or varicose veins. This vascular tumour of the skin and subcutaneous tissue may exhibit rapid and progressive growth, and is usually located in the neck or head. Ocular presentation is unusual and orbital involvement is even rarer. We report a case of a preterm newborn male with a rapidly growing left orbital mass that was histologically diagnosed as intravascular papillary endothelial hyperplasia. He was successfully managed with total excision of the tumour and propranolol therapy and remains recurrence free after an eight-year follow-up (AU)


Assuntos
Humanos , Masculino , Recém-Nascido , Hemangioendotelioma/diagnóstico , Hemangioendotelioma/patologia , Endotélio Vascular/patologia , Neoplasias Orbitárias/diagnóstico , Neoplasias Orbitárias/patologia , Diagnóstico Diferencial , Progressão da Doença
20.
Pediatr Blood Cancer ; 69 Suppl 3: e29802, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35709330

RESUMO

Vascular anomalies represent a diverse group of complex disorders that can cause significant complications, including coagulopathies, pain, and decreased function. The diagnosis of vascular anomalies is often challenging due to heterogeneity of presenting phenotypes and overlapping clinical features with other pediatric conditions. Pediatric hematologists/oncologists (PHO) are uniquely positioned for an essential role in diagnosing, managing, and coordinating the multidisciplinary care required to maximize the quality of life of these patients. Here, we review the diagnostic approach involved in patients with vascular anomalies and utilize cases to highlight the challenges involved, and how PHOs can play a vital part in the care of these patients.


Assuntos
Hemangioendotelioma , Malformações Vasculares , Humanos , Qualidade de Vida , Malformações Vasculares/diagnóstico , Malformações Vasculares/terapia
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