Intravascular papillary endothelial hyperplasia: collection of four cases and a review of the literature (original) (raw)
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Intravascular papillary endothelial hyperplasia: report of 2 cases and immunohistochemical study
Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics, 2008
Intravascular papillary endothelial hyperplasia (IPEH) is a benign, nonneoplastic, vascular lesion. The main significance of the lesion lies in the fact that it may be mistaken for angiosarcoma. Oral lesions are uncommon and the present paper reports 2 cases of oral IPEH, in different sites. Histologically, the tissue was characterized by papillary fronds lined by proliferating endothelium. Immunohistochemically (IHC), the lesion was positive for CD34, smooth muscle actin (SMA), type I and IV collagen, vimentin, and laminin, but it was negative for CD105. Local excision was the treatment of choice. No recurrence was observed during a 1-year and 6-month follow-up period, respectively. The clinical, histological, and immunohistochemical characteristics are discussed.
Periocular intravascular papillary endothelial hyperplasia: A retrospective study
European Journal of Ophthalmology., 2021
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 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. 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.
Australian and New Zealand Journal of Ophthalmology - AUST N Z J OPHTHALMOL, 1995
Background: An g iosa rcom as are uncommon malignant tumours of vascular endothelium. They frequently affect the face and scalp, but to our knowledge there are only three reports of eyelid involvement. Methods/results: We report a case of angiosarcoma arising from the eyelid skin in a 79-year-old woman. Treatment was surgical with wide excision and staged reconstruction of the defect. Systemic oncological work-up for metastatic disease was negative. Adjuvant systemic chemotherapy and radiotherapy were considered but were not felt to be of proven benefit and were not used. The patient died seven months after presentation from a myocardial infarct. Conclusion: Angiosarcoma should be considered in the differential diagnosis of unusual or atypical eyelid lesions. Management is wide surgical excision, although adjuvant systemic therapy should be considered in selected cases in consultation with an oncologist.
Journal of Medical Case Reports, 2012
Introduction: Intravascular papillary endothelial hyperplasia is a reactive proliferative lesion of endothelial cells in blood vessels. It typically presents as a painless, reddish purple lesion in the sites affected. The orbit remains an uncommon site of affectation of this relatively common disease. It is noteworthy that this is the first reported case, to the best of our knowledge, of orbital intravascular papillary endothelial hyperplasia in a Nigerian child. Case presentation: The case reported here is an orbital intravascular papillary endothelial hyperplasia causing non-axial proptosis and loss of vision in a 14-year-old Nigerian boy. We describe the clinical and histological findings of intravascular papillary endothelial hyperplasia in the orbit of this 14-year-old boy. The key distinguishing features are discussed and relevant literature is reviewed. Conclusion: Although unusual in presentation, intravascular papillary endothelial proliferation should be considered in the list of differentials of proptosis due to mass lesion in young Nigerians and, possibly, Africans.
Oncology Letters, 2018
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.
Intravascular papillary endothelial hyperplasia: An unusual histopathological entity
Indian Dermatology Online Journal, 2015
Intravascular papillary endothelial hyperplasia (Masson′s tumor) is a benign lesion of the skin and subcutaneous tissue consisting of a reactive proliferation of endothelial cells with papillary formations related to a thrombus. It poses a diagnostic challenge as the clinical signs and symptoms are nonspecific and may mimic a soft tissue sarcoma. The diagnosis is based on histopathology. Here we report two cases of Masson′s hemangioma occurring on the upper lip and on the left hand.
Auris Nasus Larynx, 2009
Background: Intravascular papillary endothelial hyperplasia (IPEH) is an abundant, usually intravascular, benign endothelial proliferation that may mimic angiosarcoma clinically and histopathologically. Its occurrence in the sinonasal cavity is extremely rare. To the best of our knowledge, we present the first case with the most extensive IPEH ever found within the sinonasal cavity. Methods: A 42-year-old man of intravascular papillary endothelial hyperplasia in the sinonasal cavity was reported. He complained of a 1year history of left-sided nasal obstruction accompanied by unilateral rhinorrhea, repeated epistaxes and frontal headache. Anterior rhinoscopy demonstrated a smooth-surfaced reddish mass occupying the left nasal cavity. Endoscopic surgery was used to clear this uncommon tumor in the sinonasal cavity. A review of the pertinent literature was also presented. Conclusions: IPEH may be mistaken for an angiosarcoma clinically and histopathologically. Complete endoscope-guided surgical excision is the best choice of therapy for patients with IPEH and is both diagnostic and curative. Awareness of this lesion will prevent incorrect diagnosis and overly aggressive treatment.
Adrenal Intravascular Papillary Endothelial Hyperplasia
World Journal of Endocrine Surgery
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.
Intravascular Papillary Endothelial Hyperplasia of the Central Nervous System-Four Case Reports
Neurologia medico-chirurgica, 2004
Four rare cases of intracranial intravascular papillary endothelial hyperplasia (IPEH) manifesting as cranial nerve disturbances occurred in 16-, 18-, 24-, and 28-year-old females. Magnetic resonance imaging showed all lesions as isointense with strong enhancement on T 1-weighted images, and as hyperintense on T 2-weighted images. All lesions were removed via craniotomies. Histological examination found vascular structures and papillary spaces lined with endothelial cells showing immunoreactivity for CD31. Complete removal was curative in two cases, whereas incomplete removal resulted in cure in one case and residual deficits in one case. Iatrogenic deficits should be avoided in IPEH treatment by surgery. Differentiation from neoplasm such as angiosarcoma depends on histological characteristics.
Intravascular Papillary Endothelial Hyperplasia Simulating Malignant Melanoma
Acta Dermato-venereologica, 2010
Intravascular papillary endothelial hyperplasia (IPEH) is a benign endothelial proliferation, usually intravascular, that may mimic angiosarcoma. In this report, four new cases of IPEH involving the oral region are described. The affected sites were the lower lip, labial comissure and the submandibular region. After clinical evaluation, the complete removal of the lesions showed a circumscribed and soft mass. Histologically, the major feature was a reactive proliferation of endothelial cells composed of small papillary structures with hypocellular and hyalinized cores arising in an organized thrombus. Immunohistochemical staining for CD34 was strongly positive in endothelial cells. Vimentin and laminin immunolabelling were also consistent with a vascular origin. In order to verify the proliferative potential of the lesions, the Ki-67 antibody was used, revealing low percentage of labeled cells (<20%). No immunoreactivity for GLUT-1 was observed. Since the complete removal is curative, no additional treatment was necessary, and no signs of recurrence had been observed until now. Due to the particular features of IPEH, it is important for pathologists and clinicians to become familiar with this lesion. Additionally, the specific histological arrangement, including the absence of cellular pleomorphism, mitotic activity and necrosis, represents a guide to help in the differential diagnosis. Moreover, the vascular origin and the proliferative index should be assessed by immunohistochemistry in order to provide an accurate diagnosis.