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.

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

INTRODUCTION: Intravascular papillary endothelial hyperplasia (IPEH) predominantly occurs in the subcutaneous and dermal regions and rarely originates from the sinonasal mucosa. CASE PRESENTATION: We 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. CONCLUSION: This 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.

Intravascular papillary endothelial hyperplasia (Masson's tumour) in the jejunum: A case report of unusual gastrointestinal bleeding.

INTRODUCTION: Intravascular papillary endothelial hyperplasia (IPEH), originally described by Pierre Masson in 1923, is a benign vascular lesion characterised by the reactive proliferation of endothelial cells. This tumour typically manifests on the fingers, head, neck, or trunk. However, involvement of other organ systems, including abdominal organs, is possible although exceedingly rare. CASE PRESENTATION: A 57-year-old male patient presented to the emergency department with a 24-h history of recurrent light-headedness. The patient was haemodynamically stable, and physical examination showed no abdominal pain. Digital rectal examination unveiled melena. Initial blood analysis indicated a haemoglobin level of 10.5 g/dL. Comprehensive workup with abdominal computed tomography, upper and lower endoscopy, and gastrointestinal lumen MRI failed to yield significant findings. DISCUSSION: On the fourth day of hospitalisation, persistent melena and transfusion of four units of blood triggered a new upper endoscopy. This endoscopy extended to the jejunum, revealing a submucosal lesion measuring 20 mm, situated approximately 40 cm distal to the ligament of Treitz. Due to ongoing intraluminal bleeding with decreased haemoglobin levels, a segmental jejunal resection was performed. Pathological examination confirmed the diagnosis of a completely resected submucosal IPEH. CONCLUSION: IPEH, also known as Masson's tumour, is an uncommon vascular lesion within the intestinal tract, occasionally resulting in persistent haemorrhage. The preferred treatment is total surgical resection, with a low likelihood of recurrence. Currently, postoperative surveillance is not recommended. To the best of our knowledge, no cases of recurrence have been documented for Masson's tumour localised in the gastrointestinal tract in the existing literature.

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

BACKGROUND: 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. CASE REPORT: 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. CONCLUSION: 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.

Masson's Tumor as an Uncommon Cause of Neck Mass: A Case Presentation.

BACKGROUND: Masson's tumor, commonly referred to as intravascular papillary endothelial hyperplasia (IPEH), is an uncommon growth of endothelial cells within a vessel wall that is frequently assumed to indicate an abnormal resolution of thrombosis. IPEH is most typically found in the extremities however it is rare for IPEH to appear as a neck tumor. The issue with IPEH is that it could clinically, radiologically, and pathologically imitate some malignant neoplasms such as angiosarcomas creating a diagnostic challenge. CASE REPORT: We describe a 21-year-old male patient who presented with right anterolateral neck swelling for 12 months. Ultrasound revealed a 9.0 × 8.0 cm well-defined echogenic hyper-vascular lesion. The contrast computed tomography (CT) scan of the neck revealed an oval, well-defined subcutaneous mass, measuring 9 × 4.5 cm, situated over and separable from the right sternocleidomastoid muscle with no significant enhancement in the post-contract study. T1-weighted and T2-weighted MRI revealed a 10 × 9 × 7 cm well-defined subcutaneous lobulated lesion superficial to the sternocleidomastoid expanding upward to the Rt. side of the cheek and below to the suprasternal region, eliciting an intermediate signal in T1 and a heterogenous bright signal (mostly fluid) in T2 with low signal foci within the mass. The decision had been reached to entirely excise the lesion surgically with safety margins for histological evaluation. Histological examination indicated thrombosed variable-sized ectatic vascular spaces with papillary formations related to the thrombus, covered with a single layer of flat endothelium, and no features suggestive of malignancy. There was no recurrence at 18 months follow-up post-surgery. CONCLUSION: Masson's tumor is a benign intravascular disease with an unclear origin and no confirmed inheritance pattern. Presentation of Masson's tumor as a neck mass is incredibly uncommon. Masson's tumor lacks a distinct or distinguishing clinical and radiological appearance. Histopathologic examination is the sole definitive way for diagnosing the disease and the only tool for distinguishing it from angiosarcoma. Surgical excision is the best treatment for IPEH. Recurrence is extremely rare.

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.

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.

A Case of Left Ventricular Masson's Tumor: Not Every Heart Tumor Is a Myxoma.

Masson's tumor is a benign tumor that usually arises secondary to vascular trauma or thrombi, leading to vascular proliferation. Masson's tumors are most commonly reported in the head, neck, and extremities. Cases in the heart are exceedingly rare, with most case reports describing the left atrium as the most common location. Even though the tumor is benign, excision is recommended due to the risk of embolization. This is a case of Masson's tumor located in the left ventricle. The patient is a 24-year-old female, who presented complaining of palpitations and lightheadedness. Transthoracic echocardiography showed a mobile echodensity in the left ventricle. Cardiac MRI showed characteristics similar to a myxoma. The patient underwent surgical resection and a biopsy showed Masson's tumor. This case report focuses on the histopathological features and imaging findings of Masson's tumor.

Case report: Multiple brain intravascular papillary endothelial hyperplasia: incidence, diagnostic challenges, and management approach.

Multiple hemorrhagic brain lesions are mainly diagnosed based on clinico-radiological features integrated with histological data. Intravascular papillary endothelial hyperplasia (IPEH), or Masson's tumor, is a very rare entity, particularly when localized in the brain. In this study, we describe a case of multiple recurrent brain IPEHs and provide details on the diagnostic phase, therapeutic approaches, and related challenges. A 55-year-old woman presented with a relapsing neurological deficit. Brain magnetic resonance imaging (MRI) revealed a hemorrhagic right frontal-parietal lesion. When new neurological symptoms occurred, subsequent MRI scans detected more bleeding cerebral lesions. She underwent a series of single hemorrhagic lesion debulking. For any samples that underwent histopathological examination, the first results were not informative; the second and the third results revealed hemangioendothelioma (HE); and the fourth results led to the IPEH diagnosis. Interferon alpha (IFN-α) and subsequently sirolimus were prescribed. Both were well tolerated. Clinical and radiological features remained stable 43 months after starting sirolimus therapy and 132 months after the first diagnosis. To date, 45 cases of intracranial IPEH have been reported, mostly as single lesions without parenchymal location. They are usually treated by surgery and sometimes by radiotherapy upon recurrence. Our case is notable for two main reasons: because of the consecutive recurrent multifocal exclusively cerebral lesions and the therapeutic approach we used. Based on multifocal brain recurrence and good performance, we propose pharmacological therapy, including IFN-α and sirolimus, to stabilize IPEH.

Parotid Masson's Tumor in a 29-years-old woman: A Case Report.

Introduction: Intravascular papillary endothelial hyperplasia (IPEH) is a papillary hyperplasia of the endothelial vascular cells, also called Masson's tumor. Masson's etiology and risk factors remain unclear but trauma and vascular pathologic conditions may start the tumor process from its common regions like extremities. Common presentations include swelling and mild pain. Our Radiologic modality of choice is Contrast-enhanced MRI which can help us before operating parotidectomy, the gold standard of tumor treatment. As presented in this study, Parotid Masson's tumor, is a very rare form of Masson's,making it even more exceptional. Case Report: This paper reports a case of a 29-years-old woman with a mass in herright parotid gland from 17 years ago, which has slowly increased in size during these years. She underwent a total parotidectomy following unsuccessful Fibrovein injections, which caused her inflammation. Embolization was performed before the resection to decrease the risk of its hemorrhage. Postoperative follow-up confirmed the reliability of this treatment method as the patient declared no side effects. Apart from its tough diagnosis, since Masson's tumors, especially the ones that emerge in the parotid, are rare, we decided to introduce this case to deliver more information about the treatment and diagnosis of this rare disease to other colleagues. Conclusions: The prognosis of parotid Masson's is admirable following a total resection. The patient had no postoperative complaints with no need for multiple visits after resection.

Masson's tumor of the reconstructed breast.

Intravascular papillary endothelial hyperplasia (Masson's Tumor) is a rare benign endothelial vascular lesion that can mimic angiosarcoma if not properly recognized. It represents less than 2% of all vascular tumors, but has been seen in the postradiation setting, which also makes differentiating it from angiosarcoma crucial. It is classically characterized as a circumscribed, intravascular mass that is hypoechoic on ultrasound, and T1 isointense and T2 heterogenous on MRI with variable enhancement. Histologically, it demonstrates papillary architecture without significant atypia, and associated vascular thrombus. Although it typically occurs in the soft tissues of the trunk and neck, a very small percentage of cases have been found in the breast. The following case will involve a 64-year-old female with a Masson's tumor involving the capsule of her left breast implant, in the setting of previously treated ductal carcinoma in situ, which was surgically excised and irradiated over 20 years prior.

Intravascular Papillary Endothelial Hyperplasia (Masson's Tumor) of the Submandibular Gland: A Case Report.

Intravascular papillary endothelial hyperplasia (IPEH), a rare benign endothelial vascular lesion related to thrombosis, generally develops in fingers, trunk, head, and neck. Nevertheless, it has been rarely reported in the salivary gland. In this article, we report a case of IPEH of the right submandibular gland in a 37-year-old female whose initial impression was sialadenitis secondary to sialolithiasis. To date, our case may be the first well-documented report of IPEH in the submandibular gland. The patient underwent ablation of the submandibular gland, and no evidence of recurrence was found during follow-up.

Spinal intravascular papillary endothelial hyperplasia. Case report and review of the literature.

Intravascular papillary endothelial hyperplasia (Masson's vegetated hemangioendothelioma) is a rare condition affecting the neuroaxis. In the literature, only eight cases of this lesion involving the vertebral canal with spinal cord compression has been reported. We present a 37-year-old man with thoracic location mimicking schwannoma. Differential diagnosis, management, and review of literature are discussed in this short report.

A Rare Instance of Chronic Masson's Tumor Presenting in the External Auditory Meatus and Surgical Plan: A Case Report.

Intravascular papillary endothelial hyperplasia (IPEH), or Masson's tumor, is a rare and benign proliferation of endothelial cells typically of vascular origin. Common locations of Masson's tumor include the head, neck, orbit, lip, pharynx, and mandible. It is typically seen in middle-aged adult life and females. Possible differential diagnoses include hemangioma, benign vascular formation, angiosarcoma, and neurofibromatosis. The exact pathophysiology of Masson's tumor is currently unknown. We present the case of a middle-aged 47-year-old male with a pure type of Masson's tumor presenting with pedunculated, malleable lesions across the posterior scalp and circumferential neck, on the pinna of the right ear, and within the right external auditory meatus. The lesions within the right external auditory meatus caused conductive hearing loss. The plan is a complete surgical excision without wide margins. The patient was referred to an ear, nose, and throat (ENT) surgeon due to the complicated location of the lesion within the external auditory meatus. This case serves as a differential diagnosis of conductive hearing loss complicated by Masson's tumor.

Intravascular Papillary Endothelial Hyperplasia Involving the Hand: A Case Report of a Rare Entity.

Masson's tumor, also named intravascular papillary endothelial hyperplasia (IPEH), is a rare, benign vascular tumor. Evaluation by clinical features can be confused with other soft tissue tumors. Therefore, the diagnosis should be confirmed by histopathological examination. The patient reported here is 67 years old and came to us with a small painful lesion over the left thumb of about two months duration. Histopathological examination was consistent with Masson's tumor (IPEH) following excisional biopsy, with good functional outcomes. To the best of our knowledge, this is the first report of this entity from Kuwait. Dermatologists and surgeons should know about this rare entity and its unusual presentation, to be able to distinguish it from similar presenting serious conditions, especially angiosarcoma. Through this report, we purport to facilitate recognition of this condition apart from some other conditions it may mimic.

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

BACKGROUND: Intravascular 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 PRESENTATION: A 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. CONCLUSION: Clinicians 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.

A Unique Surgical Case of Ancient Calcified Intravascular Papillary Endothelial Hyperplasia in the Tibia with Knee Joint Osteoarthritis.

BACKGROUND: Intravascular papillary endothelial hyperplasia (IPEH) is a reactive lesion histopathologically characterized by papillary growth of vascular endothelial cells. IPEH is most commonly found in the skin and subcutaneous tissues of the head, neck, and extremities. Furthermore, it has been reported to occur in oral surgery, but its occurrence in bone is extremely rare. CASE PRESENTATION: We present the case of a 77-year-old man with a chief complaint of left knee arthralgia. The knee joint X-ray showed Kellgren-Lawrence grade 4 osteoarthritis and a mass lesion with decreased permeability within the bone in the medial part of the proximal tibia. Computerized tomography (CT) scan of the left knee showed a localized mass in the left proximal tibia with clear margins and granular internal calcification. The preoperative diagnosis was left knee osteoarthritis and a benign tumor of the left proximal tibia (enchondroma or hemangioma). The patient requested surgical treatment, so left total knee arthroplasty (TKA) and resection of the tumor were performed. The pathology revealed a rare intraosseous IPEH with marked calcification. CONCLUSIONS: Since intraosseous IPEH could not be considered from the clinical findings, the pathological diagnosis was the decisive factor. This report showed the world's first case of intraosseous IPEH with marked calcification. Similar to the calcification of intraosseous hemangiomas, we considered the possibility that, in IPEH, the thrombus may fibrosis and organize in concentric circles, causing necrosis at the center and resulting in calcification. TKA was performed on the degenerative knee joint with IPEH, and a good patient outcome was obtained.

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 PRESENTATION: A 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.

Peri-orbital Masson's tumor: a case report and literature review.

Masson tumor is a benign vascular lesion characterized by an intravascular papillary endothelial hyperplasia. Peri orbital locations are rare. We report a case of Masson tumor localized in the upper internal angle of the left orbit revealed by progressive ocular proptosis. Orbital computed tomography (CT) scan and magnetic resonance imaging (MRI) showed a vascular mass in the left internal canthus mimicking an arterioveinous malformation. The patient underwent total removal of the lesion with a favorable postoperative follow up. Histological examination found an intravascular papillary endothelial hyperplasia without atypical features corresponding to Masson tumor. A thorough literature review of Masson tumor is presented with a discussion of clinical findings and management.