Papillary Endothelial Hyperplasia Research Articles

Masson tumor of the right atrial appendage presenting as cardiac tamponade: a case report

BackgroundIntravascular papillary endothelial hyperplasia (Masson tumor) is a rare, benign vascular tumor characterized by the proliferation of endothelial cells within blood vessels. While it typically affects the skin and subcutaneous tissues, it can occasionally appear in unusual locations.Case presentationWe report a rare case of Masson tumor located in the right atrial appendage of a healthy individual. This case was complicated by atrial wall rupture and subsequent cardiac tamponade. The tumor was successfully resected under cardiopulmonary bypass.ConclusionThis case highlights a rare occurrence of Masson tumor in the heart, emphasizing the importance of prompt surgical intervention to prevent life-threatening complications such as cardiac tamponade.

Epithelioid (Histiocytoid) hemangioma of the testis: A case report and literature review of a rare benign tumor.

Testicular hemangioma is a rare benign vascular neoplasm originating from the inner layer of the tunica albuginea, predominantly affecting the pediatric population and often associated with hemangiomas in other organs. Histopathologically can be categorized into four types, including capillary, cavernous, papillary endothelial hyperplasia, and epithelioid (histiocytoid). We report a case of a 24-year-old presenting with left inguinal pain, diagnosed with an epithelioid (histiocytoid) hemangioma of the testis. Diagnostic evaluation included scrotal doppler ultrasonography, which revealed a small, hypoechoic formation with evident vascularization. Serum tumor markers were within normal limits. Surgical excision via testis-sparing surgery (TSS) was performed based on frozen section analysis. A literature review underscores the rarity of testicular histiocytoid hemangiomas. Advancements in diagnostic technique have improved the accuracy of diagnosis and allowed for more conservative surgical approaches, preserving fertility and minimizing long-term adverse effects. In conclusion, while testicular hemangiomas may present characteristic features on ultrasound, frozen section examination remains crucial in guiding surgical decision-making. TSS offers significant advantages over traditional excisional surgery, including reduced morbidity and preservation of cancer control, making it an essential option in the management of testicular hemangiomas, particularly in young patients concerned about fertility preservation.

Mediastinal Teratoma with Angiosarcomatous Overgrowth in a Background of Vasculogenic Mesenchymal Tumor

Abstract Introduction/Objective Vasculogenic mesenchymal tumor refers to neoplastic reiteration of embryonic vasculogenesis. It is most commonly associated with nonseminomatous germ cell tumors of the mediastinum, in particular yolk sac tumor. In rare instances these lesions progress to angiosarcoma. Herein, we describe a case of angiosarcoma in a background of vasculogenic mesenchymal tumor in the setting of a mediastinal teratoma. Methods/Case Report A 33-year-old male, with a past medical history of anxiety, and right orchiectomy for testicular torsion, presented with shortness of breath, chest pain, and left arm swelling. Imaging studies demonstrated a mediastinal mass. Biopsy of the mass showed a germ cell tumor most compatible with a teratoma in the limited specimen. The patient received two rounds of chemotherapy and additional treatments were discontinued due to poor tolerance. Subsequent imaging showed continued increase in the size along with mass effect causing moderate to severe compression of the superior vena cava. Subsequently, the patient underwent resection of the mass. Grossly the mass was predominantly encapsulated with areas of capsular disruption. Sectioning revealed heterogeneous cut surfaces with fleshy, cystic, and necrotic areas. Morphologically the tumor was composed predominantly of teratomatous elements with areas of anastomosing atypical vascular channels within a hypocellular stroma suggestive of vascular genic mesenchymal as well as areas reminiscent of Masson lesion. Focally within these areas were malignant spindle cell proliferations compatible with angiosarcoma. The patient had an elevated AFP; however, no yolk sac tumor component was identified after thorough sampling. Final diagnosis of teratoma with angiosarcoma in a background of vasculogenic mesenchymal tumor was rendered after expert consultation. Results (if a Case Study enter NA) NA Conclusion Somatic-type malignancy and sarcomatous overgrowth are known to occur in mediastinal germ cell tumors; however, classification of the sarcomatous overgrowth can be challenging. Vasculogenic mesenchymal tumor is an exceedingly rare sarcomatous overgrowth that can rarely progress to angiosarcoma.

Intravascular Papillary Endothelial Hyperplasia Arising within Angiokeratoma of Fordyce in a Young Male

Intravascular Papillary Endothelial Hyperplasia Arising within Angiokeratoma of Fordyce in a Young Male

Soft tissue hemangioma of the right upper extremity with intraosseous extension and secondary intravascular papillary endothelial hyperplasia.

Intravascular papillary endothelial hyperplasia (IPEH), also known as Masson's tumor, is an uncommon exuberant form of organizing thrombus that may occur within a vessel, vascular tumor, or hematoma and may change the imaging appearance to mimic an aggressive process. It must be distinguished pathologically from angiosarcoma. They have been most commonly reported within superficial soft tissue tumors, and rapid growth and effect on bone are rarely described. We present a case of a patient with a soft tissue hemangioma with IPEH with intraosseous extension that presented with a pathologic fracture of her right humerus with an aggressive appearing osseous lesion. CT and MRI demonstrated a multifocal ill-defined soft tissue mass throughout the right upper extremity with underlying cortical tunneling and scalloping of the proximal humerus. Similar imaging findings were also present in the distal humerus and ipsilateral scapula and evolved during her hospitalization. Following percutaneous biopsy revealing hemangioma with features of papillary endothelial hyperplasia with intraosseous extension, the patient died in the ICU secondary to unrelated septic shock. Diagnosis was confirmed at autopsy. Primary and secondary IPEH have been generally characterized as well-defined solitary masses, most often in the superficial soft tissues. This case of a deep soft tissue hemangioma with type II IPEH, intraosseous extension, and imaging findings of regional multicompartmental involvement is very unusual. Reporting of this case in the literature should be beneficial for pathologic correlation with similar confounding masses as well as propose a possible mechanism for intraosseous extension of soft tissue hemangiomas.

Report of intraosseous intravascular papillary endothelial hyperplasia associated with an odontogenic cyst in the maxilla and literature review

Intravascular papillary endothelial hyperplasia (IPEH) represents an uncommon reactive endothelial hyperplastic proliferation. A 46-year-old man experienced increased volume in the right maxilla, elevation of the nasal ala, and swelling of the hard palate with a reddish hue for 3 months. Computed tomography revealed an expansive hypodense region and cortical bone destruction associated with an impacted supernumerary tooth and an endodontically treated tooth. Under the differential diagnoses of a radicular cyst, dentigerous cyst, and ameloblastoma, an exploratory aspiration and incisional biopsy were performed. This revealed the formation of blood vessels of various diameters lined by endothelium, forming intravascular papillae positive for CD-34. The definitive diagnosis was IPEH, and the patient was treated by embolization and surgery. Histological analysis confirmed the presence of IPEH associated with an odontogenic cyst. After 12 months of follow-up, no recurrence was observed. Also, we reviewed case reports of IPEH affecting the maxilla and mandible. Fourteen intraosseous cases were reported in the maxilla and mandible, with a preference for males and affecting a wide age range. Complete surgical excision was the treatment of choice, and recurrences were not reported. The pathogenesis of IPEH is controversial and may originate from trauma or inflammatory processes. To the best of our knowledge, this is the first report of an association of IPEH with an odontogenic cyst. The importance of IPEH in the differential diagnosis of intraosseous lesions in the jaws is emphasized, and preoperative semiotic maneuvers are needed to prevent surgical complications.

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.

Masson’s tumor of the thoracic spine: A rare cause of slowly progressive paraplegia

Introduction. Intravascular papillary endothelial hyperplasia is an unusual reactive proliferation of endothelial cells around an organized thrombus, which occurs either in a dilated blood vessel, hematoma, or preexisting vascular lesion. These tumors rarely affect the central nervous system. Symptoms depend on the localization of the process itself. Localization in the central nervous system is limited to the intracranial space. Localization in the spinal canal is extremely rare, and only a few clinical cases have been described so far in the literature. Case report. A 67- year-old female patient was examined neurologically initially due to bilateral weakness of the lower extremities, accompanied by a feeling of pain and muscle tension, dominantly in the upper legs, more to the right. The complaints were present a year ago, and before that period, the patient was in a stable state of health. Due to a severe neurological deficit and the need for detailed exploration, the patient was hospitalized. A diagnosis was performed, which showed the localization of the pathological process in the thoracic 5?6 region of the spinal column. Decompression surgery was performed, and with the ex tempore findings metastasis was ruled out. Definitive pathohistological findings proved Masson?s tumor. After the operation, the neurological weakness recovered. Conclusion. Masson?s tumor, although rarely localized in spinal canal, is curable if it is correctly diagnosed and if an adequate therapeutic approach is applied. The initially presented symptoms may resemble numerous neurological or systemic diseases, which requires the clinician to be continuously aware of such rare pathological processes.

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.

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

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

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.