Papillary Endothelial Hyperplasia Research Articles

Dermoscopy of Umbilical Lesions-A Systematic Review.

Background: The umbilicus is a fibrous remnant located in the centre of the abdomen. Various entities may be encountered in this special anatomical location; however, little is known about their dermoscopic presentation. The aim of this study was to provide a comprehensive summary of existing evidence on dermoscopic features of umbilical lesions. Methods: Studies assessing dermoscopic images of umbilical lesions were included in this study. No age, ethnicity or skin phototype restrictions were applied. Papers assessing lesions outside of the umbilical area, lacking dermoscopic images and/or dermoscopic description and not related to the topic were excluded. Embase, Medline and Cochrane Library were searched from inception to the end of May 2023. The Joanna Briggs Institute critical appraisal tools were used to evaluate the risk of bias of the selected studies. The quality and the level of evidence of included studies were assessed according to the Oxford 2011 Levels of Evidence. Thirty-four studies reporting a total of 39 lesions met the inclusion criteria and were included in qualitative analysis. Results: A qualitative synthesis of the following entities was performed: melanoma, nevi, basal cell carcinoma, fibroepithelioma of Pinkus, Sister Mary Joseph nodule, mycosis fungoides, dermatofibroma, endometriosis, epidermal cyst, granuloma, intravascular papillary endothelial hyperplasia, lichen planus, omphalolith, seborrheic keratosis, and syringoma. Conclusions: Dermoscopy is a non-invasive technique that may be useful in the differential diagnosis of umbilical lesions. The main limitations of this study were lack of a high level of evidence in the studies and the lack of uniformity in applied dermoscopic terminology between included studies.

Intravascular papillary endothelial hyperplasia of the maxillary sinus extending into the contralateral nasal cavity

IntroductionIntravascular papillary endothelial hyperplasia (IPEH) predominantly occurs in the subcutaneous and dermal regions and rarely originates from the sinonasal mucosa.Case presentationWe report on the case of a 58-year-old male patient who presented with progressive bilateral nasal obstruction, left-sided epiphora, and intermittent epistaxis. Computed tomography revealed a soft tissue opacity in the left maxillary sinus with intersinusoidal nasal wall demineralization, extending into the surrounding ethmoid cells and the right nasal cavity through a contralateral deviation of the nasal septum. Contrast-enhanced T1-weighted magnetic resonance imaging further confirmed these findings. The IPEH originating from the maxillary sinus extended into the contralateral nasal cavity, and it was successfully removed using an endoscopic endonasal approach, avoiding overly aggressive treatment.ConclusionThis case report highlights the diagnostic challenges of IPEH in the sinonasal region and the importance of considering IPEH as a differential diagnosis in patients presenting with nasal obstruction, epiphora, and intermittent epistaxis.

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

Phenomena of Intussusceptive Angiogenesis and Intussusceptive Lymphangiogenesis in Blood and Lymphatic Vessel Tumors.

Intussusceptive angiogenesis (IA) and intussusceptive lymphangiogenesis (IL) play a key role in the growth and morphogenesis of vessels. However, there are very few studies in this regard in vessel tumors (VTs). Our objective is to assess the presence, characteristics, and possible mechanisms of the formation of intussusceptive structures in a broad spectrum of VTs. For this purpose, examples of benign and malignant blood and lymphatic VTs were studied via conventional procedures, semithin sections, and immunochemistry and immunofluorescence microscopy. The results demonstrated intussusceptive structures (pillars, meshes, and folds) in benign (lobular capillary hemangioma or pyogenic granuloma, intravascular papillary endothelial hyperplasia or Masson tumor, sinusoidal hemangioma, cavernous hemangioma, glomeruloid hemangioma, angiolipoma, and lymphangiomas), low-grade malignancy (retiform hemangioendothelioma and Dabska tumor), and malignant (angiosarcoma and Kaposi sarcoma) VTs. Intussusceptive structures showed an endothelial cover and a core formed of connective tissue components and presented findings suggesting an origin through vessel loops, endothelialized thrombus, interendothelial bridges, and/or splitting and fusion, and conditioned VT morphology. In conclusion, the findings support the participation of IA and IL, in association with sprouting angiogenesis, in VTs, and therefore in their growth and morphogenesis, which is of pathophysiological interest and lays the groundwork for in-depth molecular studies with therapeutic purposes.

Immunohistochemical Study of Differential Expressions of CD31, ERG, VEGF-A, α-SMA, Glut-1, CD105, D2-40 and Ki-67 in Oral Intravascular Papillary Endothelial Hyperplasia

Immunohistochemical Study of Differential Expressions of CD31, ERG, VEGF-A, α-SMA, Glut-1, CD105, D2-40 and Ki-67 in Oral Intravascular Papillary Endothelial Hyperplasia

Case Report: Intravascular Papillary Endothelial Hyperplasia In An Older Man

Masson's tumor is a benign tumor consisting of reactive proliferation of endothelial cells. It was first described by the French doctor, Pierre Masson, in 1923. A higher incidence was observed among women (1.3:1), and the average age at which it occurs is 31 years. It appears as a firm mass, with the appearance of a nodule or papule, located subcutaneously. The adjacent integument is bluish or reddish. On palpation it can be sensitive or painless. We will present the case of an 81-year-old man with Masson's tumor located at the level of the volar side of the left forearm, at the level of the flexor ulnar muscle of the carpi, which affects the ulnar artery.

Intravascular papillary endothelial hyperplasia in the lungs of a wild Korean raccoon dog.

A male Korean raccoon dog of unknown age was rescued and placed at the Daejeon Wildlife Rescue Center, Korea. Physical examination revealed severe emaciation and dehydration, as well as thick crusts and alopecia over most of the body. During medical care, the animal died and was submitted for postmortem examination. Firm, brown-red lesions of various sizes were observed on the surface of the lungs. In cross-sections of the lungs, pulmonary vessels were thickened and dilated, with white irregular papillary luminal projections. Histologically, pulmonary blood vessels were severely hyperplastic, characterized by thickened dilated walls and fibrous papillary projections covered with a single layer of endothelial cells (ECs). Hyperplastic fibrous connective tissue was confirmed by Masson trichrome staining. The ECs expressed CD31. We diagnosed the lesion as intravascular papillary endothelial hyperplasia, a unique non-neoplastic reactive process that has not been reported previously in pulmonary vessels of canids, equids, or felids, to our knowledge.

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.

PRAME immunohistochemistry in soft tissue tumors and mimics: a study of 350 cases highlighting its imperfect specificity but potentially useful diagnostic applications.

Preferentially expressed antigen in melanoma (PRAME) immunohistochemistry is currently used in pathology for the assessment of melanocytic neoplasms; however, knowledge of its expression patterns in soft tissue tumors is limited. PRAME immunohistochemistry (clone QR005) was assessed on whole tissue sections of 350 soft-tissue tumors and mimics (> 50 histotypes). PRAME immunoreactivity was evaluated as follows: 0 "negative" (0% positive cells); 1+ (1-25% positive cells); 2+ (26-50% positive cells); 3+ (51-75% positive cells), and 4+ "diffuse" (> 75% positive cells). PRAME was expressed in 111 lesions (0 benign, 6 intermediate malignancy, and 105 malignant), including fibrosarcomatous dermatofibrosarcoma protuberans (2/4, 0 diffuse), NTRK-rearranged spindle cell neoplasm (2/4, 0 diffuse), atypical fibroxanthoma (1/7, 0 diffuse), Kaposi sarcoma (1/5, 0 diffuse), myxoid liposarcoma (11/11, 9 diffuse), synovial sarcoma (11/11, 6 diffuse), intimal sarcoma (7/7, 5 diffuse), biphenotypic sinonasal sarcoma (3/3, 1 diffuse), angiosarcoma (10/15, 6 diffuse), malignant peripheral nerve sheath tumor (9/12, 4 diffuse), pleomorphic rhabdomyosarcoma (2/3, 2 diffuse), alveolar rhabdomyosarcoma (2/6, 0 diffuse), embryonal rhabdomyosarcoma (7/7, 4 diffuse), undifferentiated pleomorphic sarcoma (2/12, 1 diffuse), leiomyosarcoma (2/15, 1 diffuse), clear cell sarcoma of soft tissue (1/10, 0 diffuse), low-grade fibromyxoid sarcoma (1/5, 0 diffuse), Ewing sarcoma (2/10, 1 diffuse), CIC-rearranged sarcoma (8/8, 4 diffuse), BCOR-sarcoma (2/5, 1 diffuse), melanoma (20/20, 14 diffuse), and thoracic SMARCA4-deficient undifferentiated tumor (5/5, all diffuse). All tested cases of spindle cell lipoma, dedifferentiated/pleomorphic liposarcoma, dermatofibrosarcoma protuberans, solitary fibrous tumor, inflammatory myofibroblastic tumor, myxoinflammatory fibroblastic sarcoma, nodular fasciitis, myxofibrosarcoma, epithelioid hemangioendothelioma, atypical vascular lesion, hemangioma, lymphangioma, vascular malformation, papillary endothelial hyperplasia, GIST, gastrointestinal clear-cell sarcoma, malignant melanotic nerve sheath tumor, neurofibroma, schwannoma, granular cell tumor, alveolar soft part sarcoma, epithelioid sarcoma, extraskeletal myxoid chondrosarcoma, myoepithelioma, ossifying fibromyxoid tumor, angiomatoid fibrous histiocytoma, PEComa, dermatofibroma, pleomorphic dermal sarcoma, and chordoma were negative. PRAME shows imperfect specificity in soft-tissue pathology but may serve as a diagnostic adjunct in selected differential diagnoses that show contrasting expression patterns.

Papillary Endothelial Hyperplasia

A man in his 30s had a unilateral mass of the epididymis. There was no prior history of local trauma, and ultrasonographic imaging did not reveal the presence of any other testicular abnormality. A 1.4-cm unilocular cystic lesion was excised. Histopathologic examination showed the presence of blood vessels with organizing thrombi adjacent to the epididymis (Figure 1A, B ). On microscopic examination, several foci of proliferative clusters of cells with epithelioid to vaguely spindled morphology were identified (Figure 1C, D). What is the lineage of the epithelioid cells, and is this lesion benign or malignant?a.Vascular lineage, malignant (angiosarcoma)b.Carcinoma, malignantc.Vascular lineage, benign (papillary endothelial hyperplasia)d.Melanoma, malignant Answer: c. Vascular lineage, benign (papillary endothelial hyperplasia) The images show the presence of an intravascular lesion with organizing thrombus that is broken down into collagenized clumps admixed with hemorrhagic material (Figure 2A, B ). These collagenized clusters are lined by vascular endothelial cells, and immunohistochemistry for ERG and CD31 confirms vascular lineage (Figure 2C, D). These findings are consistent with papillary endothelial hyperplasia.1Akdur N.C. Donmez M. Gozel S. Ustun H. Hucumenoglu S. Intravascular papillary endothelial hyperplasia: histomorphological and immunohistochemical features.Diagn Pathol. 2013; 8: 167Crossref PubMed Scopus (51) Google Scholar Papillary endothelial hyperplasia is thought to represent breakdown of intravascular thrombi with accompanying ingrowth of vascular endothelial cells along the edges of thrombotic material. In late stages, as in this case, these lesions may impart an appearance of a papillary lesion with an anastomotic network of vascular endothelial cells that is frequently misinterpreted as an angiosarcoma. However, in contrast to angiosarcoma, these lesions are almost always exclusively intravascular and lack infiltrative growth. Infrequently, papillary endothelial hyperplasia may be recognized in an extravascular location secondary to rupture or as part of organization of an extravascular hematoma. These lesions exhibit minimal nuclear pleomorphism, mitotic activity, or necrosis. Finally, papillary endothelial hyperplasia has an excellent prognosis and does not recur when completely excised.

Tumor de Masson en la mama: un reto diagnóstico

Papillary endothelial hyperplasia (PEH) or Masson's tumor is a rare benign vascular tumor that usually appears in the soft tissues of the head and neck, trunk and extremities, being extremely rare in the breast. Its diagnosis can be a challenge, especially in the follow-up of patients with previous disease of breast carcinoma. We present the case of a 65-year-old patient, with a history of bilateral breast cancer and reconstruction with implants, who presented a Masson's tumor during follow-up. An ultrasound scan was performed, showing a well-circumscribed mass in the left breast, located in the posterior contour of the implant. Subsequently, magnetic resonance imaging (MR) depicted an enhancing tumor, without infiltration of adjacent structures. Finally, the definitive anatomopathological diagnosis was obtained after surgical excision.

Intravascular papillary endothelial hyperplasia: Olanzapine-induced vascular proliferation?

Intravascular papillary endothelial hyperplasia (Masson's tumor) is a reactive vascular lesion of obscure etiopathogenesis, often seen in the head and neck. Its presentation as a scalp swelling, however, is extremely uncommon. We describe the first report in an adult, being treated for bipolar illness. A young male presented with a right frontotemporal scalp swelling since 3 weeks. He was also being treated for bipolar illness with olanzapine. Examination revealed a soft, non-pulsatile swelling. After inconclusive aspiration results, a complete excision was performed. Histopathology revealed proliferating endothelial cells arranged as papillary fronds confined to vessel lumina, devoid of atypia, accompanied by thrombosed vessels facilitating a diagnosis of Masson's tumor. The patient is free of recurrence five months after surgery. Further studies on a possible effect of olanzapine on vascular proliferation in experimental in vivo and in vitro models would definitely aid in elucidating clinical relevance, if any.

Masson's Tumour: A Rare Case of Submental Swelling.

Masson's Tumour: A Rare Case of Submental Swelling.

Peculiar Histological Features of Oral Intravascular Papillary Endothelial Hyperplasia.

A 55-year-old male patient with single and well-circumscribed nodule in the lower lip. Accurate diagnosis is based only on histopathological examination using hematoxylin and eosin and immunohistochemical approach, which a large, organized thrombus within the dilated lumen of a poorly demarcated vein, associated with papillary projections of endothelial proliferation occupying vascular spaces. The final diagnosis was intravascular papillary endothelial hyperplasia (IPEH) associated with a thrombus. Oral IPEH is rare and has historically been difficult to diagnose due to its resemblance to other oral lesions. However, the distinctive histological features of oral IPEH associated with a thrombus now allow for its diagnosis through hematoxylin and eosin staining alone, without the need for additional techniques. Therefore, it is crucial for pathologists to be familiar with these unique morphological features to accurately diagnose oral IPEH and differentiate it from more common benign, malignant, or reactive vascular lesions in the oral cavity.

Intravascular papillary hemangioendothelioma disguised as a peripheral sheath tumor of median nerve at the wrist: a case report and literature review

BackgroundIntravascular papillary hemangioendothelioma (IVPH) is a benign lesion previously reported in the nasal cavity, neck, upper extremities, and breast. Diagnosis with cross-sectional imaging can prove difficult, with histopathological examination necessary for diagnosis. IVPH resulting in carpal tunnel symptoms is quite rare.Case presentationWe report the case of a 37-year-old woman who presented with a radial, volar right wrist mass enlarging over the span of 5 years. She noted numbness and tingling in her wrist and thumb, exacerbated by minor accidental collisions and wrist hyperextension. There was no antecedent trauma. On examination, a mildly tender, mobile mass was evident at the volar aspect of the right wrist. Magnetic resonance imaging (MRI) with contrast demonstrated a lobulated, predominantly T2 hyperintense, heterogeneously enhancing mass thought to be a peripheral nerve sheath tumor. The patient elected for surgical excision of the mass, and the histopathological examination showed organizing thrombi with prominent papillary endothelial hyperplasia. At the 2-month follow-up, the patient had full range of motion of her fingers and wrist, with subjectively normal sensation in the distribution of the median nerve.ConclusionCarpal tunnel syndrome, in exceedingly rare occasions, can result from an IVPH. MRI findings may be confused with more common entities. Histopathological confirmation remains necessary for conclusive diagnosis.

Intravascular papillary endothelial hyperplasia of the spleen in a child: a case report

BackgroundIntravascular papillary endothelial hyperplasia (IPEH) is a vascular tumor characterized by the proliferation of endothelial cells with papillary formation. It is a rare benign condition affecting the head and neck. Currently, no cases of IPEH of the spleen have been reported. Here, we report a case of IPEH of the spleen in a child and discuss its clinical manifestations, imaging features, and surgical treatment.Case presentationA 5-year-old female presented with a 4-month-old tumor in the left upper abdomen, abdominal pain, and constipation. She underwent radiography, barium enema, US, and MRI. A solid space-occupying mass was found in the left abdominal cavity on preoperative imaging, and it was diagnosed as angiosarcoma. The lesion was surgically resected. Histopathological analysis was consistent with IPEH.ConclusionClinicians should consider the possibility of IPEH in patients presenting with tumors in the spleen, which is curable by surgical resection. Malignant vascular tumors must be excluded in the differential diagnosis of IPEH to prevent misdiagnosis and inappropriate overtreatment.

Intravascular Endothelial Hyperplasia of the Foot

Intravascular endothelial hyperplasia is a benign soft tissue mass rarely reported in the foot. Advanced imaging and confirming a benign diagnosis are critical for any soft tissue mass. This paper identifies 2 patients that developed intravascular endothelial hyperplasia tumors which required surgical excision. A 17-year-old male patient presented to clinic complaining of a painful bump to the arch of his right foot which he related to an injury 9 months prior. Magnetic resonance imaging of the right foot revealed a mass within the plantar subcutaneous fat that was serpiginous in nature similar to adjacent branching vessels favoring a low-flow vascular malformation. A 38-year-old female with Multiple Sclerosis presented with complaints of persistent symptoms of pain to the 1st interspace, difficult ambulation and neuritis. Ultrasound and MRI observed solid, multilobulated mass, with internal vascular malformation, MRI describing intrinsic involvement along the abductor musculature and flexor tendons. Both lesions were surgically excised and sent for pathology. Pathology report indicated a diagnosis of intravascular papillary endothelial hyperplasia or Masson's tumor in both cases. Pathology diagnosis of intravascular papillary endothelial hyperplasia is generally good with wide resection leading to low recurrence rates. Both patients in the current study have progressed postoperatively with resolution of symptoms and without recurrence.

Intravascular papillary endothelial hyperplasia in the cecum: a case report

Background Intravascular papillary endothelial hyperplasia (IPEH), also known as Masson's tumor, is a benign, non-neoplastic vascular lesion that is characterized by reactive proliferation of papillary endothelial cells associated with a thrombus. These lesions typically develop in the vascular regions of the head and neck, oral cavity, or extremities; however, other organ systems have been affected. IPEH in the gastrointestinal tract is rare, with only a few cases reported to date. Thus, the pathogenesis and clinical features of IPEH in the gastrointestinal tract are not entirely understood. Moreover, the local excision of certain subtypes of IPEH can be curative; this makes timely diagnosis essential. We present the case of a patient with IPEH in the cecum that was discovered while investigating the cause of severe anemia.Case presentationA 29-year-old woman visited a general practitioner (GP) with the complaint of abdominal pain. She was diagnosed with acute appendicitis and was prescribed antibiotics. After treatment, her abdominal pain disappeared. However, she was found to be severely anemic (hemoglobin level, 6.5 g/dl). To determine the cause of her anemia, the GP referred her to our hospital for further examination and treatment. Computed tomography scan revealed cecal wall thickening. Further, a lower gastrointestinal endoscopy revealed a 2-cm raised mass-like lesion in the cecum. This lesion was pathologically identified as an inflammatory granuloma. The cause of her anemia was determined to be bleeding from the lesion in the cecum. She underwent laparoscopic ileocecal resection.Histopathological examination of the surgical specimen revealed a spongy structure comprising many small papillary fibrous tissues lined by a typical monolayer endothelium. Further, immunohistochemical analysis showed that the cells of the endothelium monolayer expressed CD31, CD34. The Ki-67 labeling index was < 1%. Based on these findings, the lesion was identified as an IPEH in the cecum. The patient’s postoperative course was uneventful, and there was no evidence of recurrence during the 1.3 years of follow-up.Conclusions IPEH rarely arises within the abdominal cavity. Surgery remains the only treatment for IPEH and is associated with an excellent prognosis and a low recurrence rate. More aggressive lesions such as angiosarcoma should be excluded when considering the histologic diagnoses of IPEH, and expert pathologic review is vital. This is the first report of IPEH occurring in the cecum and represents a novel cause of gastrointestinal bleeding which the clinician should consider when evaluating a patient with atypical or difficult gastrointestinal bleeding sources.

Value of ultrasonography in diagnosis and classification of Masson's tumor

A retrospective analysis was performed on 11 cases of Masson's tumor admitted to Liaocheng People's Hospital from January 2010 to July 2021. Among them, there were 4 males and 7 females, aged from 14 to 62 years, with a medical history of 1 to 24 months. All of the patients complained of touching the mass under the skin. In this group, 9 cases were pure form, 1 case was mixed form and 1 case was extravascular form. Ultrasound imaging can reflect the characteristics of Masson's tumor to a certain extent, which has a certain value in the diagnosis, classification and differential diagnosis.

Characterization of intravascular papillary endothelial hyperplasia: a multicentre cohort.

Intravascular papillary endothelial hyperplasia (IPEH) is an uncommon benign malformation of the skin and subcutaneous tissue. In this retrospective multicentre study, we aimed to investigate the clinical and pathological features of 261 patients with IPEH. IPEH is classified into three categories; in our study, the proportions were pure (50%), mixed (46%) and extravascular (4%). IPEH frequently stained positive for immunohistochemical markers such as CD31, CD34, smooth muscle actin and erythroblast transformation-specific-related gene. Clinicians' initial impression of the lesion often included ambiguous terms such as 'soft tissue mass'. There is an opportunity for increased awareness of this lesion and its consideration within a differential diagnosis.