ArticleLiterature Review

A mixed form of intravascular papillary endothelial hyperplasia in an uncommon location: case and literature review

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Abstract

Intravascular papillary endothelial hyperplasia (IPEH) is an unusual benign, non-neoplastic vascular lesion that usually occurs in skin, but is uncommon in the oral cavity. Herein, we review the pertinent literature of oraiiPEH and report a new mixed form. A 61-year- old man presented with an ulcerated nodule in the lingual portion of the gingiva related to the left mandibular canine. An excisional biopsy was performed presuming the clinical diagnosis of pyogenic granuloma. Histopathological analysis showed areas of granulation tissue consistent with pyogenic granuloma. But in addition, there were thin-wall dilated vessels with papillary projections of endothelial cells producing vascular channels, associated with an area of organizing thrombus. These microscopic findings led to the diagnosis of pyogenic granuloma associated with IPEH. The immunohistochemical reactions revealed a diffuse positivity of the vascular cells for CD-34 and smooth muscle actin antibodies.In addition,there was partial positivity for podoplanin and negativity for CD-1OS in the IPEH areas. No signs of recurrence were observed after 6 months of follow-up. The most prevalent site of IPEH in the oral region is the lower lip. IPEH is slightly more common in women and exhibits peaks of prevalence between the fourth and sixth decades of life.

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... Intravascular Papillary Endothelial Hyperplasia (IPEH), also known as Masson's tumor or intravascular vegetative hemangioendothelioma, is an unusual benign, non-neoplastic vascular lesion (Milhan et al., 2018). Which was described for the first time in 1923 by the pathologist Frances Pierre Masson (Cohen et al., 2009;Garcia-Guliarte et al., 2009;Milhan et al., 2018;Voruz et al., 2020) in a 68-year-old male patient with a painful, ulcerated hemorrhoid that could not be reduced. ...
... Intravascular Papillary Endothelial Hyperplasia (IPEH), also known as Masson's tumor or intravascular vegetative hemangioendothelioma, is an unusual benign, non-neoplastic vascular lesion (Milhan et al., 2018). Which was described for the first time in 1923 by the pathologist Frances Pierre Masson (Cohen et al., 2009;Garcia-Guliarte et al., 2009;Milhan et al., 2018;Voruz et al., 2020) in a 68-year-old male patient with a painful, ulcerated hemorrhoid that could not be reduced. The hemorrhoid was excised and it was determined that histologically it was characterized by an atypical papillary endothelial proliferation that mimicked angiosarcoma (Tosios et al., 1994;Steffen, 2001;Milhan et al., 2018). ...
... Which was described for the first time in 1923 by the pathologist Frances Pierre Masson (Cohen et al., 2009;Garcia-Guliarte et al., 2009;Milhan et al., 2018;Voruz et al., 2020) in a 68-year-old male patient with a painful, ulcerated hemorrhoid that could not be reduced. The hemorrhoid was excised and it was determined that histologically it was characterized by an atypical papillary endothelial proliferation that mimicked angiosarcoma (Tosios et al., 1994;Steffen, 2001;Milhan et al., 2018). IPEH was named again by Clearkin and Enzinger in 1976, as a benign tumor that arises in the oral region and other regions, where it is established that the entity is a reactive phenomenon rather than a true neoplasm (Yonezawa et al., 2009;Vieira et al., 2021), defining it as a reactive benign lesion of vascular origin that is produced by an excessive proliferation of endothelial cells (Cohen et al., 2009). ...
... 10,15 Another histopathological mimicker is epithelioid hemangioma, which shares with IPEH the growth of the endothelium within a blood vessel lumen but shows more solid growth, exhibits epithelioid cells, and is not linked to thrombosis. 16 Finally, IPEH may show diffuse reactivity with CD34 and SMA confirming the vascular origin (as observed in our cases) in addition to focal expression of a lymphatic endothelial marker (podoplanin). 16 This might indicate a role of the lymphatic vessels' endothelial cells in the development of IPEH. ...
... 16 Finally, IPEH may show diffuse reactivity with CD34 and SMA confirming the vascular origin (as observed in our cases) in addition to focal expression of a lymphatic endothelial marker (podoplanin). 16 This might indicate a role of the lymphatic vessels' endothelial cells in the development of IPEH. 16,17 This further supports our findings of underlying lymphatic-related lesions in 6/7 of our cases (4 LV malformations and 2 conjunctival hemorrhagic lymphangiectasia), which might need further extensive studying. ...
... 16 This might indicate a role of the lymphatic vessels' endothelial cells in the development of IPEH. 16,17 This further supports our findings of underlying lymphatic-related lesions in 6/7 of our cases (4 LV malformations and 2 conjunctival hemorrhagic lymphangiectasia), which might need further extensive studying. ...
Article
Introduction: 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. Methods: 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. Results: 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 69years with a median age of 36years. 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. Conclusions: 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.
... In this context, patients typically present with a short history of a distinct vascular lump and need to be differentiated from primary vascular tumors such as angiosarcoma [4] 2. Secondary IPEH occurs in relation to a preexisting dilated vascular lesion such as a varix, hemangioma, or arteriovenous malformation. These are long-standing lesions, and often, IPEH is identified as an incidental finding after surgical excision [5] 3. Finally, an extremely rare extravascular form of IPEH has been described in long-standing organized hematomas. [6] The exact pathophysiology of IPEH is poorly understood. ...
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Masson hemangioma is a rare benign vascular tumor characterized by intravascular papillary endothelial hyperplasia (IPEH). Numerous case reports have described lesions in the digits, neck, oral cavity, and intracranially. IPEH involving primary brachial artery aneurysm is extremely rare, with only a handful of reported cases. We present a 43-year-old female with a true distal brachial artery aneurysm who presented with a painful pulsatile mass in the antecubital region. She underwent aneurysm resection and end-to-end repair with a saphenous vein graft. Histology confirmed the diagnosis of IPEH with characteristic features of papillary hyperplasia within the intravascular thrombus.
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Article
Intravascular papillary endothelial hyperplasia (IPEH) is a benign non-neoplastic lesion histopathologically characterized by papillary proliferation of endothelial cells in dilated vascular lumen, and occurrence in the oral region is relatively rare. In this study, we performed clinical, histopathological, and immunohistochemical evaluations in 13 cases of oral IPEH, and examined the pathogenetic mechanism, pathophysiology, and differentiation from other vascular anomalies. The upper lip was the most common lesion site (5 cases), followed by the lower lip (3 cases) and the tongue (2 cases). The most common clinical diagnosis was hemangioma in 7 cases, followed by benign tumor in 4 cases. On gross examination, the tumor was dark red or mucosal color, similar to a hemangioma, and the surface was smooth, elastic and soft. Histological classification was mixed form in 11 cases and pure form in 2 cases. In immunohistochemical staining, CD31 was expressed in the cytoplasm and on cell membrane of vascular endothelial cells in all 13 cases; ERG was expressed in the nuclei of vascular endothelial cells in all 13 cases; α-SMA was expressed on cell membrane of smooth muscle cells in the blood vessel wall in all 13 cases; CD105 was expressed in the cytoplasm of vascular endothelial cells in 10 of 13 cases; and VEGF-A was expressed in the cytoplasm of vascular endothelial cells in all 13 cases. D2-40 was expressed in the cytoplasm and on cell membrane of endothelial cells within the papillary structures in only 2 cases, but Glut-1 expression was not detected in any of the cases. IPEH was easily differentiated from angiosarcoma, Kaposi’s sarcoma, mucous cyst, and pyogenic granuloma. The pathogenetic mechanism of this disease suggested by this study is that thrombus formation induces a hypoxia and low glucose state at the lesion site, and VEGF produced by endothelial cells stimulates reactive proliferation of the endothelial cells to form IPEH.
Article
Masson's hemangioma (MH), also known as intravascular papillary endothelial hyperplasia, is a rare benign vascular tumor. Histopathology helps to confirm the diagnosis, wherein a papillary proliferation of thin-walled capillaries is almost always associated with thrombus formation in the lumen of a vessel. We report the case of a 48-year-old female with a purple-colored swelling over the right thigh that was diagnosed as MH with the help of histopathological examination. Dermoscopy was also done, and the findings were reported.
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Case Intravascular papillary endothelial hyperplasia (IPEH) or Masson's tumor is a rare and apparently benign entity that has rarely been reported in the foot. Its exact etiology is not known but repetitive local trauma might play a role. We hereby present a case of IPEH in the foot, associated with tailor's bunion. Conclusion This is, to our knowledge, the first report of IPEH associated with tailor's bunion. Further research on the relationship between mechanical injury and occurrence of IPEH in the foot is needed, particularly when bunions or bunionettes are present.
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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.
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This report describes a case of intravascular papillary endothelial hyperplasia (IPEH) arising in the upper lip. A 7-year-old girl was referred to our department because of painless swelling of the upper lip in October 2015. She had suffered a bruise on her upper lip 4 months before consultation. Physical examination revealed an elastic hard submucosal mass, measuring 20 × 10 mm, on the left side of the upper lip. Magnetic resonance imaging (MRI) indicated a well-defined neoplastic lesion. The lesion was clinically diagnosed as a benign tumor and was surgically removed under general anesthesia. The histopathological diagnosis of the excised specimen was IPEH (pure form). IPEH is very difficult to clinically diagnose, since it shows various clinical findings. In previous reports, there are some cases in which IPEH was confirmed by surgical resection with a clinical diagnosis of hemangioma. It has been pointed out that its occurrence mechanism is related to a history of trauma, and it was the same in our case. IPEH (pure form) rarely recurs after complete resection. In our case, four years have passed since the operation and there is no sign of recurrence.
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Background Pyogenic granuloma (PG) is a lesion characterized by the proliferation of blood vessels commonly affecting the skin and the mouth. We aimed to compare clinical, microscopic, and immunohistochemical features of the two types of oral PG: lobular capillary hemangioma (LCH) and non‐LCH (NLCH). Methods Epidemiological and clinical data from 2000 to 2018 were collected from the archives of our institution, and histopathological sections of PG were reviewed. Immunohistochemical analyses (CD34, D2‐40, SMA, mast cell, and Ki‐67) were performed in 34 cases. Results Sixty‐two LCH and 107 non‐LCH samples were included. The mean (±SD) age of the patients was 38.59±16.96 years; 55.62% were female; 39.64% of cases occurred in the gingiva, 44% of the nodules were pedunculated, and 13.02% of patients reported a history of trauma. NLCH was more prevalent among older patients than LCH. The most prevalent site of LCH was the lips, while NLCH occurred more in the gingiva (p<0.05). Epithelial atrophy, microvessels, SMA‐positive areas, and Ki‐67‐positive nuclei were more prevalent in LCH (p<0.05). Conclusions PG accounted for 2.25% of lesions archived in the pathology service and most cases were NLCH. LCH and NLCH exhibited clinicopathological differences in terms of age, site, epithelial atrophy, vascularization, and proliferation rate. This article is protected by copyright. All rights reserved.
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Intravascular proliferations of the skin are clinically heterogeneous and may present with a wide range of clinical features, including violaceous papules, nodules, plaques, or other unspecific cutaneous lesions. Histopathologically, these conditions are characterized by proliferation of different cell types within the lumina of dermal vessels and endothelial cell hyperplasia. Immunohistochemistry is the best tool to identify the nature of the intravascular proliferating cells and the type of involved vessel. In this review, we analyzed the clinicopathologic and immunohistochemical characteristics of intravascular large cell lymphoma, T-cell and natural killer-cell intravascular large cell lymphoma, intralymphatic variant of CD30 cutaneous lymphoproliferative disorders, benign atypical intralymphatic CD30 T-cell proliferation, reactive angioendotheliomatosis, intralymphatic histiocytosis, papillary intralymphatic angioendothelioma or Dabska tumor, glomeruloid hemangioma, papillary hemangioma, intravascular papillary endothelial hyperplasia or Masson phenomenon, and the intralymphatic involvement of Merkel cell carcinoma, cutaneous metastases, and cutaneous angiosarcoma.
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Intravascular papillary endothelial hyperplasia (IPEH), often referred to as Masson’s Tumor, is an uncommon yet benign vascular disease of the skin and subcutaneous tissues. It usually arises within a blood vessel, but is considered to be a non-neoplastic reactive endothelial proliferation commonly associated with vascular injury. Although it is rare, knowledge of this disease is important as it may mimic other benign and malignant tumors, especially angiosarcoma, which may lead to unnecessary aggressive management. Typically, IPEHs are asymptomatic and are slow growing soft-tissue masses with extremely low-recurrence rates. In this article, we describe a 19-year-old male with a recurrence of Masson’s Tumor over the right little finger within 2 months of a routine excision of the lesion. We also present accompanying multimodality clinical, radiological, and pathological imaging. This case illustrates the innocuous nature of the initial lesion easily mistaken for a hemangioma. Awareness of the possibility of a recurrence of a Masson’s Tumor is important for clinicians to rule out the presence of malignant vascular lesions.
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