Case Of Intravascular Papillary Endothelial Hyperplasia Research Articles

Cutaneous Intravascular papillary endothelial hyperplasia – A case series

Intravascular papillary endothelial hyperplasia (IPEH) is rare intravascular endothelial proliferation that may mimic an angiosarcoma. They are most commonly located in veins on head and neck, fingers and trunk and usually presents as a small superficial mass. It is now considered as an exuberant form of organising thrombus. We present here a series of cases of IPEH with varied clinical presentation and clinical diagnosis which on microscopy confirmed as Intravascular papillary endothelial hyperplasia

A Rare Case of Intravascular Papillary Endothelial Hyperplasia (Masson's Tumor) of Vocal Cord.

Intravascular papillary endothelial hyperplasia (IPEH) also known as Masson's tumour named after Pierre Masson is a benign lesion of vascular origin which is characterized by reactive proliferation of endothelial cells with papillary formation. It is an extremely rare entity which can present anywhere in the body but is particularly found in the extremities and head and neck regions most commonly the lips, oral mucosa, tongue, gingiva, or buccal mucosa and due to its close resemblance to Angiosarcoma, it becomes important to distinguish between these two entities to avoid unnecessary aggressive treatment. Here, we describe a case of 23-year-old male who presented with change in voice since 8 months. Preoperatively, laryngoscopic examination showed right vocal cord polyp in the anterior one-third of the cord with hemorrhagic changes. He underwent micro-laryngeal surgery using suspension laryngoscope and the lesion was excised completely. Histopathological report was consistent with IPEH. Herein, we describe a rare benign case of IPEH arising from the vocal cord and we provide a brief review of relevant literature and a detailed report of this rare entity.

Intravascular papillary endothelial hyperplasia as a rare cause of cervicothoracic spinal cord compression: A case report

BACKGROUNDIntravascular papillary endothelial hyperplasia (IPEH) is a rare benign reactive vascular lesion that grows into an expansile compressing mass. It most commonly involves the skin and subcutaneous tissue. Spinal involvement is rare, with only 11 reported cases in the literature. We report, to our knowledge, the first case of IPEH in the cervicothoracic spinal canal and present a literature review.CASE SUMMARYA 27-year-old man presented with acute-onset neck pain, numbness, and weakness in his extremities. Magnetic resonance imaging showed an epidural mass in the cervicothoracic (C6-T1) spinal canal and vertebral hemangioma (VH) involving the C7 vertebral body. C6-T1 Laminectomy and radical excision of the mass were performed. Histopathological examinations revealed papillary proliferation of vascular endothelial cells with thrombus formation, and an IPEH diagnosis was made. By his 6-mo follow-up appointment, his symptoms were relieved without recurrence. The possible pathogenesis, clinical and imaging features, differential diagnosis, and management of IPEH were reviewed.CONCLUSIONWe report, to our knowledge, the first case of IPEH in the cervicothoracic spinal canal, treated via complete resection, and showing a favorable outcome. We found a causal relationship between spinal IPEH and VH; this partly explains the mechanism of IPEH.

Periocular intravascular papillary endothelial hyperplasia: A retrospective study.

Intravascular papillary endothelial hyperplasia (IPEH) is a rare proliferation of endothelial cells with uncertain etiology related to thrombus formation. Diagnosis is usually confirmed histopathologically. This condition has been previously described in the periocular region but not in the conjunctiva. It is a retrospective case series in which we evaluated seven patients with histopathologically confirmed IPEH cases. Data regarding the demographics, clinical presentation, radiological description, histopathological features including any IHC staining, suspected underlying vascular etiology, management options, and follow up outcome were collected. A total of seven cases of histologically confirmed IPEH were included. Five out of seven patients were male (71.4%). The age range was between 6 and 69 years with a median age of 36 years. Three cases involved the eyelid (42.8%) and another three were found in the conjunctiva (42.8%). Pre-existing underlying vascular lesions were observed in all patients, five malformations (mostly lymphatic-venous) and two conjunctival hemorrhagic lymphangiectasis. All cases were treated with excisional biopsy with no signs of recurrence within an average of 7 months follow up. Periocular IPEH is a rare tumor that is likely to coexist with underlying vascular lesions and thrombus formation. We are reporting its existence in the conjunctiva for the first time. Therefore, pathologists should be aware of the histopathological spectrum of this lesion.

Myriad pillars formed by intussusceptive angiogenesis as the basis of intravascular papillary endothelial hyperplasia (IPEH). IPEH is intussusceptive angiogenesis made a lesion.

Intussusceptive angiogenesis (IA) is the process by which pre-existing blood vessels split, expand and remodel through intravascular pillar formation. In previous works, we studied the morphologic characteristics of intravascular papillary endothelial hyperplasia (IPEH) and suggested the participation of IA in the histogenesis of the lesion. Our current goal is to demonstrate that myriad papillae in IPEH are in fact myriad pillars, the hallmarks of IA. For this purpose, specimens of 14 cases of IPEH were used for conventional histologic techniques, immunohistochemistry and immunofluorescence in confocal microscopy. The studies showed the following pillar characteristics: a) structural composition by an endothelial cell (EC) cover and a connective core, b) characteristic pillar image and its appearance and disappearance in whole-mounted and series of individual views in confocal microscopy (requirements for pillar identification), c) arrangement in masses, alignments and meshes, and d) formation from vein intimal ECs, which extend and originate loops that encircle vein wall components (interstitial tissue structures: ITSs) and fibrin. The encircling ECs form the pillar cover and the encircled ITSs or fibrin form the initial core. Intraluminal endothelial bridges also originate from the vessel wall and from the pillars (nascent and thin pillars). In conclusion, the formation of myriad pillars, predominantly in veins, is the basis of IPEH. This lesion may therefore be considered an excessive expression of IA: IA becomes a lesion.

Intravascular papillary endothelial hyperplasia in the oral mucosa and jawbones: A collaborative study of 20 cases and a systematic review.

Intravascular papillary endothelial hyperplasia is an unusual vascular lesion characterized by the proliferation of endothelial cells. The aim of this study was to determine the frequency and general features of this lesion. Biopsy records of three oral pathology services were reviewed for intravascular papillary endothelial hyperplasia cases from 1959 to 2020. In addition, a systematic review of case reports and case series was carried out in eight electronic databases. Of the 65205 retrieved cases, 20 (0.03%) were diagnosed as intravascular papillary endothelial hyperplasia. Mean patient age was 46.55years, and females (12 cases/60%) were more affected. The lower lip (9 cases/47.36%) was the most commonly affected site, and the lesions were generally asymptomatic (7 cases/63.63%). Clinically, 90% of the lesions presented (18 cases) as a nodule, with a mean size of 1.13cm. The clinical diagnostic hypotheses most frequently raised were mucocele (6 cases/37.50%) and hemangioma (5 cases/31.25%). An excisional biopsy was chosen in all cases for treatment. Forty-nine studies of the systematic review were included, yielding 105 cases. The literature showed similarity in all variables. Despite the uncommon frequency, clinicians and oral pathologists should familiarize themselves with the similarities between intravascular papillary endothelial hyperplasia and some other lesions in terms of clinical and histological features.

A Case of Masson’s Tumor in Lateral Neck

Masson’s tumor, also known as intravascular papillary endothelial hyperplasia (IPEH), is a rare, benign vascular tumor characterized by the proliferation of endothelial cells with papillary formations. Differential diagnosis between IPEH and angiosarcoma is important because both have microscopic similarity. Herein, we report a rare case of IPEH on the right lateral neck of a 50-year-old female presenting with a neck mass, which was completely removed without complication.

INTRAVASCULAR PAPILLARY ENDOTHELIAL HYPERPLASIA: A SYSTEMATIC REVIEW

Objectives The aim of this study was to integrate the available data published on intravascular papillary endothelial hyperplasia (IPEH) into a comprehensive analysis of their clinic, radiographic, and imaginologic features, treatment, and recurrence. Methods An electronic search was undertaken in April 2018 in 3 databases, including cases reports and cases series of IPEH. Eligibility criteria included publications having enough clinical, radiologic, and histopathologic information to confirm a definite diagnosis of the lesion. Results Thirty studies were included in the systematic review with 86 cases of IPEH. The mean age was 46 years—with a predilection for females (55%). Asymptomatic (88%) lesions on the lower lip (39%), with a clinical presentation of firm nodular lesion, bluish, or reddish coloration and mean size of 1.6 cm were the most observed. When intraosseous, the radiographic features were multilocular radiolucent area. Of informed cases, treatment chosen was excisional biopsy (96%) and no recurrence was observed. The diagnostic hypothesis most prevalent was hemangioma, in 27 cases (31%). Conclusions The features presented in this study are more similar to than different from those reported in the literature. The aim of this study was to integrate the available data published on intravascular papillary endothelial hyperplasia (IPEH) into a comprehensive analysis of their clinic, radiographic, and imaginologic features, treatment, and recurrence. An electronic search was undertaken in April 2018 in 3 databases, including cases reports and cases series of IPEH. Eligibility criteria included publications having enough clinical, radiologic, and histopathologic information to confirm a definite diagnosis of the lesion. Thirty studies were included in the systematic review with 86 cases of IPEH. The mean age was 46 years—with a predilection for females (55%). Asymptomatic (88%) lesions on the lower lip (39%), with a clinical presentation of firm nodular lesion, bluish, or reddish coloration and mean size of 1.6 cm were the most observed. When intraosseous, the radiographic features were multilocular radiolucent area. Of informed cases, treatment chosen was excisional biopsy (96%) and no recurrence was observed. The diagnostic hypothesis most prevalent was hemangioma, in 27 cases (31%). The features presented in this study are more similar to than different from those reported in the literature.

The role of differential diagnosis in intravascular papillary endothelial hyperplasia of the sinonasal cavity mimicking angiosarcoma: A case report.

Intravascular papillary endothelial hyperplasia (IPEH), also known as Masson's tumour, is an unusual vascular lesion characterized by the proliferation of endothelial cells. The principal significance of IPEH is its clinical and radiological resemblance to angiosarcoma, resulting in a potential misdiagnosis and overtreatment. The aim of the present study is to report a rare case of IPEH of the right maxillary sinus, focusing on the radiological and histopathological features of the neoplasm and their role in the differential diagnosis with angiosarcoma. Herein we present a case of a 67-year-old woman with history of right-sided nasal obstruction. A computed tomography scan indicated opacity in the right maxillary sinus and significant bony erosion, and an magnetic resonance imaging revealed a highly vascularized polylobed neoplasm, suggesting a malignant neoplasm. The mass was removed surgically through a combined open and endoscopic approach. A perioperative biopsy excluded malignancy and histological examination was consistent with IPEH. IPEH in the sinonasal cavity is an extremely rare condition in which differential diagnosis serves a central role due to its close clinical and radiological resemblance to angiosarcoma. Awareness of IPEH is recommended for a correct diagnosis, and in order to avoid unnecessarily aggressive treatment.

Sinusoidal hemangioma and intravascular papillary endothelial hyperplasia: Interrelated processes that share a histogenetic piecemeal angiogenic mechanism

Sinusoidal hemangioma, characterized by interconnecting thin-walled vascular spaces, may present papillae/pseudo-papillae and zones that resemble intravascular papillary endothelial hyperplasia (IPEH). Our objectives are to explore the existence of zones in IPEH with sinusoidal hemangioma characteristics, the mechanism of papillary and septa formation in sinusoidal hemangioma and the comparison of this mechanism with that in IPEH. For these purposes, specimens of 4 cases of each entity were selected and studied by serial histologic sections and by immunochemistry and immunofluorescence procedures. The results showed a) zones with characteristics of sinusoidal hemangioma in IPEH cases, b) presence in both entities of papillae with a cover formed by a monolayer of CD34+ and CD31+ endothelial cells (ECs) and a core formed by either type I collagen and αSMA+ cells (presenting a pericyte/smooth muscle cell aspect) or thrombotic components, and c) a similar piecemeal angiogenic mechanism in papillary formation, including sprouting of intimal ECs toward the vessel wall itself or intravascular thrombi, formation of vascular loops that encircle and separate vessel wall or thrombus components, and parietal or thrombotic papillae development. The major differences between both entities were the number, arrangement and substrate of papillae: myriad, densely grouped, parietal and thrombotic papillae in IPEH, and a linear arrangement of predominant parietal papillae in sinusoidal hemangioma, originating septa (segmentation). In conclusion, sinusoidal hemangioma and IPEH are interrelated processes, which share morphologic findings and a piecemeal angiogenic mechanism, combining sprouting and intussusceptive angiogenesis, and leading to papillary formation and vessel segmentation.

Adrenal Intravascular Papillary Endothelial Hyperplasia

ABSTRACT Aim We describe the rare case of a woman with adrenal intravascular papillary endothelial hyperplasia (IPEH) or Masson's tumor. We present relevant background information on IPEH and our case report, as well as describe a workup and treatment plan for the lesion. Introduction Intravascular papillary endothelial hyperplasia (IPEH) or Masson's tumor is a rare lesion, i.e., predominantly found in the oral cavity, head, and neck.1-4 To our knowledge, only six adrenal IPEH cases have been reported in the literature to date.4 Case report A 49-year-old woman originally presented to us with an incidentaloma found on computed tomography scan. Due to the eventual size increase of the mass, a laparoscopic left adrenalectomy was performed. Pathologic evaluation of the mass revealed a 3 cm IPEH arising in a hemangioma within the adrenal gland. Conclusion Before the diagnosis of IPEH is considered as the etiology for an adrenal incidentaloma, it is crucial to rule out more common tumors. Serological and radiographic studies are critical to the workup. Clinical Significance: The IPEH is a very interesting lesion of the adrenal gland, however, it is exceedingly rare and thus exclusion of more common and serious lesions must be done prior to the diagnosis of IPEH. How to cite this article Holmes C, Akhras A, Schneider A, Yacoub JH, Picken M, Kabaker A. Adrenal Intravascular Papillary Endothelial Hyperplasia. World J Endoc Surg 2017;9(1):16-19.

Intravascular papillary endothelial hyperplasia (IPEH). Evidence supporting a piecemeal mode of angiogenesis from vein endothelium, with vein wall neovascularization and papillary formation.

Intravascular papillary endothelial hyperplasia (IPEH) is a reactive process of questioned pathogenesis (primary proliferation of endothelial cells/ECs versus organizing thrombi). The aim of this study is to assess the organization of morphologic patterns, with precise location of neovascularization and papillary distribution in IPEH to clarify the role of the vein wall (mainly vein intimal ECs) in lesion development and papillary formation. We studied 12 cases of IPEH in skin and subcutaneous veins by serial histological sections and immunohistochemical procedures. In four well-structured cases (the remaining cases showed overlapping events), we found four principal histological patterns organized by zone: 1) invaginated vein wall zone with microvascular networks. The intraparietal microvessels presented CD34+ and CD31+ ECs arising from ECs of the vein intima, and αSMA+ pericyte-like cells originating from modified SMCs of the media layer. 2) Papillary zone, generally with myriad papillae, formed by ECs of intraparietal microvessel networks encircling vein wall components (parietal papillae). 3) Organizing thrombotic zone from microvascular networks of invaginated vein wall zone. 4) Unorganized thrombotic zone partially covered by ECs, also originating from vein intimal endothelium and arranged in a monolayer or encircling thrombotic fibrin (thrombotic papillae). In conclusion, the capacity of vein intimal ECs and those originating from them (in newly-formed microvessels in the vein itself and covering the unorganized thrombi) to encircle vein wall components or fibrin, and to form papillae (ECs form the cover and encircled components the core) supports a piecemeal mode of angiogenesis as a pathogenic basis of IPEH. This mechanism encompasses the two histogenetic hypotheses outlined above.

Histopathological Aspects of Intravascular Papillary Endothelial Hyperplasia in Primary and Secondary Subtypes

Intravascular papillary endothelial hyperplasia (IPEH) is a reactive lesion caused by abnormal proliferation of endothelial cells during thrombus formation. Here, we report 2 cases of IPEH, emphasizing the histopathological aspects. One patient was a 47-year-old male presenting with a 1-cm submucosal nodule of the lower lip, and the other was an 11-year-old female presenting with a 4-cm enlargement in the anterior portion of the vestibular fold. Both lesions were purplish with smooth surfaces. The working diagnosis was phlebolith or hemangioma. Histopathological examination showed dilated vessels with swollen proliferating endothelial cells surrounding papillary structures of connective tissue projecting toward the lumen. These proliferating cells were associated with an organized thrombus in the first patient and were adjacent to a hemangioma in the second. Inflammatory cells, cytologic atypia, and necrosis were all absent, which excluded other vascular lesions. After being diagnosed with IPEH, the first patient is free of recurrence. The second underwent embolization of the lesion, which is decreasing in size.

Oral Intravascular Papillary Endothelial Hyperplasia (IPEH): Analysis by Confocal Laser Scanning Microscopy in Two Cases

Intravascular papillary endothelial hyperplasia (IPEH) was first described in 1923 as Masson's tumour and is currently believed to be a relatively uncommon benign, non-neoplastic vascular lesion. We report two cases of IPEH occurred on the mouth floor in female patients, presenting as slightly elevated great lesions with oval form, firm consistency, and red-bluish colour. FNAB was performed and the surgical samples were fixed in formaline, stained with Haematoxylin-eosin and Picrosirius red, and analyzed at Confocal Laser Scanning Microscope (CLSM). The histological examination showed an exuberant intravascular endothelial proliferation comprising papillary formations and anastomosing cavernous-like vascular channels. CLSM highlighted a well-defined lesion consisting of cystic spaces with blood content rounded by elongated irregular papillae, and vascular connective stroma. Thus, diagnosis of IPEH was made. Achieving a correct diagnosis is essential to avoid subjecting a patient to unnecessarily aggressive therapy. We performed Diode Laser excision and patients had no evidence of recurrences.

Intravascular Papillary Endothelial Hyperplasia (Masson's Hemangioma) Presenting as Soft Tissue Mass in Left Scapular Region-An Unusual Presentation

Intravascular papillary endothelial hyperplasia (IPEH) or reactive vascular endothelial lesion is a rare benign condition and is also known as Masson’s hemangioma. It may appear as a primary or pure form developing in a distended vessel, or it can be associated with hemangiomas, pyogenic granulomas, or lymphagiomas. It is usually confined to the lumen of preexisting vessels or vascular malformations. Nearly all the lesions are intimately associated with a thrombus in various stages of organization. The main significance of IPEH is its clinical and histological resemblance to soft-tissue sarcoma and possible misinterpretation as such. We hereby report a case of IPEH clinically and radiologically misdiagnosed as soft tissue sarcoma, in a 47-year-old man, presenting as a soft tissue mass in the left scapular region.

Intravascular Papillary Endothelial Hyperplasia of the Chest Wall Misdiagnosed as a Malignancy on Fine Needle Aspiration

Intravascular papillary endothelial hyperplasia (IPEH) is an exuberant proliferation of endothelial cells that may occur within the lumen of a pre-existing vessel or vascular malformation.1 Although IPEH is regarded as a non-neoplastic reactive process, misdiagnoses as a malignancy have been reported, particularly based on fine needle aspiration (FNA) findings.2-4 We report a case of IPEH on the chest wall that was initially misdiagnosed as an adenocarcinoma on FNA.

Destructive Radiologic Development of Intravascular Papillary Endothelial Hyperplasia on Skull Bone

Intravascular papillary endothelial hyperplasia (IPEH) is a rare vascular benign lesion that rarely involves the central nervous system with or without skull invasion. We report a rare case of IPEH on the skull bone, which displayed destructive radiologic development associated with hemorrhage. A 14-year-old male presented with an incidentally detected a small enhancing, left frontal osteolytic lesion. Previously, he underwent operation and received adjuvant chemoradiation therapy for cerebellar medulloblastoma. Follow-up magnetic resonance imaging revealed a left frontal bone lesion, which expanded to an approximately 2 cm-sized well-circumscribed osteolytic lesion associated with hemorrhage for 20 months. Frontal craniectomy and cranioplasty were performed. Destructive change was detected on the inner table and diploic space of the skull. The mass had a cystic feature with hemorrhagic content without dural attachment. Pathologic examination showed the capsule consisted of parallel collagen lamellae representing a vascular wall, vascular lumen, which was pathognomonic for IPEH. Immunohistochemical staining revealed that the capsule was positive for CD34 and factor VIII, which favor the final diagnosis of IPEH. This was the first case of intracalvarial IPEH.

Intravascular Papillary Endothelial Hyperplasia (Masson tumor) of the Skull : Case Report and Literature Review

A 10-year-old female patient presented with a rapidly growing nodular mass lesion on her right frontal area. On skull radiography and computed tomography (CT) imaging, this mass had a well-demarcated punch-out lesion with a transdiploic, exophytic soft tissue mass nodule on the frontal scalp. Magnetic resonance (MR) imaging revealed the presence of a 1.5×1.2×1 cm sized calvarial lesion. This lesion was hypointense on T1 and heterogenous hyperintense on T2 weighted MR images, and exhibited heterogeneous enhancement of the soft tissue filling the punch-out lesion after intravenous administration of gadolinium. En block removal of the tumor with resection of the rim of the normal bone was performed. The pathological diagnosis was intravascular papillary endothelial hyperplasia (IPEH). After surgery, no recurrence was found for 8 months. IPEH is a rare and benign reactive lesion usually found in thrombosed subcutaneous blood vessels. Involvement of skull bone is rare. In this article, we present a case of IPEH involving the calvarium, in a 10-year-old woman.

Intravascular papillary endothelial hyperplasia of the oral cavity

To examine the pathogenesis of intravascular papillary endothelial hyperplasia (IPEH), a relatively uncommon benign, non-neoplastic vascular lesion, clinicopathological and immunohistochemical studies were performed. Paraffin-embedded tissue specimens of 78 vascular lesions were examined histologically, and 9 cases of IPEH were investigated immunohistochemically using antibodies against CD34, vimentin, factor VIII antigen, α-smooth muscle actin (α-SMA), podoplanin, CD105, and ki-67 antigen. A thrombus or ulcer was found near the sites of all IPEH specimens. Histologic examination revealed papillary proliferated endothelial cells located toward the lumen of enlarged blood vessels. Immunohistochemistry showed that CD34, α-SMA, and factor VIII antigen were positive in lining endothelial cells. Vimentin was positive in the mesenchymal components. Immunohistochemical staining for podoplanin and CD105 was partially positive. Labeling index was 4.7 to 9.2 in ki-67-positive cases. IPEH is believed to result from reactive proliferation of blood endothelial cells that is caused by an abnormal process of organization in thrombosed blood vessels. The pathogenesis of IPEH might be related to inflammation or mechanical stimulus such as irritation.

Diagnostic implications of oral intravascular papillary endothelial hyperplasia

This study examined the clinical, histological, and immunohistochemical features as well as the differential diagnoses of oral intravascular papillary endothelial hyperplasia (IPEH) to aid clinicians and pathologists in its diagnosis. Clinical features of five oral IPEH cases were obtained from medical records, and all histopathological diagnoses were reviewed. Immunohistochemical reactions, including anti-CD-34, laminin, vimentin, estrogen receptor alpha, and Ki-67, were assessed. Microscopically, a reactive proliferation of vascular cells composed of small papillary structures with hypocellular and hyalinized cores arising in an organized thrombus was seen. CD-34, vimentin, and laminin staining were strongly positive, while estrogen receptor alpha was negative in all cases. A low percentage of cells were positive for Ki-67 in four of five lesions, but one case was strongly positive. A diagnosis of angiosarcoma was investigated and rejected. IPEH presents specific microscopic characteristics that along with clinical data lead to an accurate diagnosis. The general dentist, the first to participate in the diagnostic process, must share the responsibility for diagnosis with the pathologist, and they must work together to determine the correct diagnosis and management. Oral lesions of IPEH are uncommon. Their main significance is that they show a microscopic resemblance to angiosarcoma. Thus, clinicians should have more information regarding this benign entity. Finally, we suggest that in recurrent cases exhibiting strong immunolabeling of proliferative markers the possibility of angiosarcoma should be investigated.