Uma lesão vascular reativa e bem circunscrita. É caracterizada pela formação de papilas delgadas que se projetam no interior do lúmen de vasos sanguíneos. As papilas são revestidas por células endoteliais túrgidas. Coágulos sanguíneos também estão presentes.
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A hiperplasia endotelial papilar intravascular é definida como uma lesão vascular reativa e bem circunscrita. Suas características incluem a formação de papilas delgadas que se projetam no interior do lúmen de vasos sanguíneos, sendo essas papilas revestidas por células endoteliais túrgidas, acompanhadas pela presença de coágulos sanguíneos.[1]
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A hiperplasia endotelial papilar intravascular, também chamada de tumor de Masson ou IPEH, é uma lesão vascular benigna e reativa que surge dentro dos vasos sanguíneos. Ela se forma pela proliferação de pequenas projeções de células associadas a coágulos de sangue no interior do vaso. Em geral, o paciente percebe um pequeno nódulo ou caroço localizado em áreas como pele, mãos, bochecha ou extremidades. Não existem remédios específicos ou protocolos clínicos (PCDT) do Ministério da Saúde para esta condição rara no SUS. O cuidado principal relatado é a retirada cirúrgica da lesão para confirmação diagnóstica por biópsia.
Uma lesão vascular reativa e bem circunscrita. É caracterizada pela formação de papilas delgadas que se projetam no interior do lúmen de vasos sanguíneos. As papilas são revestidas por células endoteliais túrgidas. Coágulos sanguíneos também estão presentes.
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Publicações mais relevantes
Case Report: Prenatal presentation of Masson's tumor: first reported case and review of the literature.
Intravascular papillary endothelial hyperplasia (IPEH), or Masson's tumor, is a rare, benign vascular lesion that can closely resemble malignant vascular tumors. While primarily diagnosed in adulthood, pediatric cases are uncommon, and no prenatal diagnoses have been reported to date. Here, we present the first documented prenatal presentation of fetal intracranial IPEH detected in utero at 34 weeks of gestation. This case highlights the importance of considering IPEH in the differential diagnosis of fetal intracranial masses and underscores the role of early prenatal detection in optimizing perinatal and surgical management.
A case report: Masson's tumor (intravascular papillary endothelial hyperplasia) of the abdominal cavity.
Masson's tumor, also known as intravascular papillary endothelial hyperplasia (IPEH), is a rare benign vascular lesion that can arise in a wide range of anatomical sites, most frequently within the dermis and subcutaneous tissues. Because its clinical manifestations and radiological appearances are nonspecific and may closely mimic those of malignant vascular neoplasms or soft-tissue sarcomas, it is frequently misinterpreted as a malignant tumor, particularly in the preoperative setting. To draw attention to this important diagnostic pitfall, we present a case of IPEH that was initially misdiagnosed as a malignant lesion, with the aim of improving recognition of its characteristic features and thereby enhancing diagnostic accuracy and reducing the likelihood of future misdiagnosis. A 52-year-old woman was incidentally found to have a well-circumscribed, hypervascular intra-abdominal mass during a chest computed tomography (CT) scan. Magnetic resonance imaging showed a T1-hypointense/T2-hyperintense lesion with restricted diffusion and progressive enhancement. Given its marked hypervascularity and intra-abdominal location, a neoplastic process was initially suspected. However, following surgical exploration with complete excision of the mass and histopathological evaluation, the lesion was ultimately diagnosed as IPEH. Intra-abdominal Masson's tumor is exceedingly rare, and its imaging characteristics have not been systematically defined. In this report, we describe a surgically confirmed case of intra-abdominal Masson's tumor and place particular emphasis on its radiological features, including a well-circumscribed hyper vascular mass with progressive enhancement but without overt signs of malignancy. Despite these suggestive imaging findings, the definitive diagnosis continues to depend on histopathological assessment combined with immunohistochemical staining. By presenting this case, we hope to raise awareness of this benign yet deceptive vascular lesion and highlight that a pattern of progressive enhancement in the absence of malignant imaging features may prompt consideration of Masson's tumor in the differential diagnosis, thereby facilitating earlier and more accurate diagnosis.
Post-Traumatic Intravascular Papillary Endothelial Hyperplasia (Masson Tumor) of the Eyelid.
Intravascular papillary endothelial hyperplasia (IPEH), also known as Masson tumor, is a rare benign vascular lesion characterized by reactive endothelial proliferation within the vessel lumen. Eyelid involvement is exceedingly uncommon and may clinically mimic malignant vascular tumors. The authors report a case of post-traumatic IPEH of the eyelid in a 50-year-old man presenting with a slowly enlarging purplish nodule in the lateral canthal region. Complete surgical excision was performed. Histopathologic examination revealed tuft-like papillary endothelial proliferation confined within an expanded vein with associated thrombus, without cytologic atypia or mitotic activity, consistent with the pure type of IPEH. Immunohistochemical staining showed diffuse CD34 positivity and negative p53 expression, supporting the diagnosis and excluding angiosarcoma. No recurrence was observed during follow-up. This case underscores the extreme rarity of eyelid involvement and emphasizes that, despite a history of trauma, accurate subtype classification of IPEH relies on histopathologic rather than clinical findings.
Intravascular organizing thrombus in the forearm: a unique imaging presentation.
Organizing thrombus is a well described histologic finding involving the reorganizing and recanalization of a healing thrombus. However, it is rarely large enough to be described on imaging. When the thrombus becomes sufficiently large, intravascular papillary endothelial hyperplasia (IPEH) must be considered. IPEH or Masson's tumor is a benign, exuberant form of organizing thrombus that typically occurs in the soft tissues of the head, neck, and distal extremities. It can occur within a vessel, hematoma, or vascular mass and can mimic benign processes or malignant lesions such as angiosarcoma. Histopathological characteristics are needed for final diagnosis. The treatment is typically complete surgical resection with excellent prognosis. We report the case of a patient presenting with swelling in his left forearm in the same site as a previously treated left basilic vein thrombus. Ultrasound demonstrated a noncompressible, heterogeneous intravascular mass with internal vascularity. Subsequent MRI showed an enhancing mass centered in the left basilic vein with a flow void with the differential diagnosis of IPEH versus intravascular neoplasm. Percutaneous biopsy was performed with histology consistent with reactive vascular proliferation and organizing thrombus. The papillary architecture diagnostic of IPEH was absent. Regardless of final specific terminology, this case presents exuberant organizing thrombus as a benign cause of an enhancing intravascular mass. This patient was treated conservatively with no further intervention.
Sinonasal intravascular papillary endothelial hyperplasia (Masson's tumor): report of a new patient with review of literature.
To provide the clinico-radiologic and histopathologic illustration of a unique case of sinonasal intravascular papillary endothelial hyperplasia (IPEH; Masson's tumor). Case report and review of literature. A 29-year-old man presented with right-sided nasal obstruction and occasional blood-tinged mucopurulence for two years. Diagnostic naso-endoscopy revealed a fleshy polypoid mass occupying the right nasal cavity encroaching into the nasopharynx. The mass was tender and firm, and bled on touch. Gadolinium-enhanced magnetic resonance imaging done two months prior showed a large, heterogenous mass in the right nasal cavity, partly involving the maxillary sinus, and extending into the nasopharynx. The findings were corroborated in a recent contrast-enhanced computed tomography scan that revealed mild contrast uptake and bone remodeling due to expansile nature of the mass without any erosion. An infected antrochoanal polyp, vascular tumor, and neoplastic lesions (including inverted papilloma) were considered as differentials. A subsequent punch biopsy revealed histologic pictures suggestive of IPEH. The mass was excised completely through endonasal endoscopic approach, and it was found to originate from the right inferior turbinate. The final histopathology confirmed the biopsy findings. Troublesome bleeding was encountered both during the biopsy and endoscopic excision of the mass. The patient is symptom-free with no recurrence at one-year follow-up. Sinonasal IPEH (Masson's tumor) is an extremely rare entity; only 14 patients have been reported in the last four decades as evident from the PubMed/MEDLINE search. The present report adds to the existing literature, summarizes the clinico-radiologic and histopathologic perspectives, highlights the surgical challenges, and underlines the importance of having a low threshold of suspicion for considering this entity as a differential in a bleeding sinonasal mass.
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Reumatologia clinicaIntravascular papillary endothelial hyperplasia: An unusual histopathological entity.
Indian dermatology online journalAngiosarcoma of the adrenal gland with concurrent contralateral advanced renal cell carcinoma: A diagnostic and management dilemma.
Canadian Urological Association journal = Journal de l'Association des urologues du CanadaExtra-vascular type of oral intravascular papillary endothelial hyperplasia (Masson's tumor) of lower lip: a case report and review of the literature.
Indian journal of dental research : official publication of Indian Society for Dental ResearchIntravascular papillary endothelial hyperplasia as an aneurysm of the brachial artery.
Annals of vascular surgeryTypical MDCT Angiography Findings of an Unusual Cutaneous Neoplasia; Masson Tumor.
Polish journal of radiologyIntravascular papillary endothelial hyperplasia (Masson's tumor) of the scalp with intracranial extension.
Journal of pediatric neurosciencesThirteen-year follow-up of parasellar intravascular papillary endothelial hyperplasia successfully treated by surgical excision: case report.
Journal of neurosurgery. PediatricsAssociações
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Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
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Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
É uma alteração vascular reativa e bem delimitada que ocorre dentro do vaso sanguíneo. A alteração é caracterizada por papilas delgadas revestidas por células endoteliais e associadas a trombos sanguíneos. Também é conhecida na medicina como tumor de Masson ou IPEH.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
3 fontes citadas no texto · 9 publicações do grafo RarasNet (PubMed) · 2 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Citar este verbete
Raras. (2026). Hiperplasia endotelial papilar intravascular. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/hiperplasia-endotelial-papilar-intravascular
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Hiperplasia endotelial papilar intravascular
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
