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.

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.

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.

A novel IRF2BP2::CDX2 Gene fusion in digital intravascular myoepithelioma of soft tissue: An enigma!

Soft tissue myoepitheliomas (STM) are benign myoepithelial neoplasms (of nonsalivary gland origin) arising, most commonly within subcutaneous and deep soft tissues of the extremities and rarely within bones. To the best of our knowledge, the intravascular location of STM as well as the identification of a novel IRF2BP2::CDX2 fusion have not been previously reported. Herein, we report a case of spindle cell myoepithelioma arising within the intravascular space of the right index finger in a 52-year-old male of more than 20 years duration. Histopathology demonstrated an intravascular tumefactive lesion composed of predominantly plump banal spindle cells in a fascicular arrangement within a mixed collagenous and chondromyxoid stroma colliding with papillary endothelial hyperplasia (Masson tumor). By immunohistochemistry, the lesional cells were positive for keratin-AE1/3, epithelial membrane antigen, S100, SOX10, glial fibrillary acid protein, calponin and negative for CD34, smooth muscle actin, desmin, p63, and ERG. Fluorescence in situ hybridization for EWSR1 gene rearrangement was negative. Next-generation sequencing detected a novel IRF2BP2::CDX2 fusion involving Exon 1 of the IRF2BP2 gene and Exon 2 of the CDX2 gene confirmed by reverse transcriptase polymerase chain reaction and Sanger sequencing. Further, clinical evaluation for a salivary gland mass in the head and neck region and magnetic resonance imaging (MRI) of the chest, abdomen, and pelvis was performed with no evidence of tumor elsewhere. Taken together, the overall features were considered diagnostic of STM. Our current case underscores the novelty of the IRF2BP2::CDX2 gene fusion in STM and its exceptionally rare intravascular location.

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 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.

Intravascular papillary endothelial hyperplasia (Masson's tumor) of the conjunctiva. Unusual location of a rare vascular tumor.

Intravascular papillary endothelial hyperplasia (IPEH) is characterized by proliferation of endothelial cells usually occurring within a long-standing vascular malformation or tumor. IPEH usually affects neck, head and lower extremities, and few orbital and eyelid cases have been reported. We present a unique case, a 48-years-old man with a purple, elevated, multilobular conjunctival lesion of three-weeks duration that underwent an excisional biopsy. Pathological features revealed intravascular papillary endothelial hyperplasia (Masson's tumor).

Hiperplasia endotelial papilar intravascular (tumor de Masson) de la conjuntiva. Ubicación inusual de un tumor vascular endotelial raro / Intravascular papillary endothelial hyperplasia (Masson's tumor) of the conjunctiva. Unusual location of a rare vascular tumor

La hiperplasia endotelial papilar intravascular (IPEH) se caracteriza por la proliferación de células endoteliales generalmente producida dentro de una malformación vascular previa o un tumor. La IPEH suele afectar cuello, cabeza y extremidades inferiores, se han publicado pocos casos en la órbita ocular y párpados. Presentamos un caso único, un hombre de 48 años con una lesión conjuntival púrpura, elevada y multilobular de tres semanas de duración que se sometió a una biopsia excisional. Las características patológicas revelaron una hiperplasia endotelial papilar intravascular (tumor de Masson) (AU)

Intravascular papillary endothelial hyperplasia (IPEH) is characterized by proliferation of endothelial cells usually occurring within a long-standing vascular malformation or tumor. IPEH usually affects neck, head and lower extremities, and few orbital and eyelid cases have been reported. We present a unique case, a 48-year-old man with a purple, elevated, multilobular conjunctival lesion of three-weeks duration that underwent an excisional biopsy. Pathological features revealed intravascular papillary endothelial hyperplasia (Masson's tumor) (AU)

Masson's Tumor of the Distal Phalanx May Present Like a Felon, Report of a Rare Case.

Also known as intravascular papillary endothelial hyperplasia, Masson's tumor is a relatively rare soft-tissue vascular tumor that usually arises in the hand. Felon is an abscess formation in the distal phalanx that usually occurs following a penetrating microtrauma. We present a 30-year-old patient who was referred to our clinic with a palpable mass in the distal phalanx of the index finger after a needle stick injury. At first, the lesion was treated as a felon but finally and after treatment failure, a complete reevaluation revealed the lesion to be a Masson's tumor of the distal phalanx.

Intravascular papillary endotelial hiperplasia, the unusual diagnosis of an orbital mass.

Intravascular papillary endotelial hyperplasia is a bening vascular lesion that rarely involves the periocular region, nevertheless, it should be considered in the differential diagnosis of a periorbital mass. It is histopatologically characterized by intravascular fibrous papillary fronds covered by endothelial cells. Histopathological differentiation from angiosarcoma can be challenging but is very important to avoid aggressive treatments. Complete surgical excision is frequently curative. Uncompleted excision can lead to recurrences. We describe the clinical and histological findings of intravascular papillary endotelial hyperplasia in the left lower lid of a 72-year-old woman who had a painless mass since 15 years ago, that caused hypertropia of her left eye. Histopathological examination revealed intralesional calcification, which is not an usual feature in this entity. The patient underwent complete surgical resection of the mass and there was no evidence of recurrence after 5 months of follow-up after surgery.

Hiperplasia endotelial papilar intravascular, el inusual diagnóstico de una masa orbitaria / Intravascular papillary endotelial hiperplasia, the unusual diagnosis of an orbital mass

La hiperplasia endotelial papilar intravascular es una lesión vascular benigna, infrecuente en la región periocular, pero que debe ser tenida en cuenta en el diagnóstico diferencial de una masa periorbitaria.Histopatológicamente se caracteriza por la proliferación intravascular de proyecciones papilares de tejido conectivo recubiertas por células endoteliales. La diferenciación anatomopatológica con el angiosarcoma puede ser difícil, pero es muy importante para evitar tratamientos agresivos. Generalmente, la escisión quirúrgica completa es curativa, mientras que una escisión incompleta puede causar recurrencias.Describimos un caso clínico de hiperplasia endotelial papilar intravascular en una mujer de 72 años que presentaba una masa palpebral inferior izquierda de 15 años de evolución que le provocaba hiperglobo del ojo izquierdo. El estudio histopatológico mostró la presencia de calcificación intralesional, característica infrecuente en esta patología.La masa fue extirpada completamente y tras 5 meses de seguimiento no mostró signos de recurrencia.

Intravascular papillary endotelial hyperplasia is a bening vascular lesion that rarely involves the periocular region, nevertheless, it should be considered in the differential diagnosis of a periorbital mass.It is histopatologically characterized by intravascular fibrous papillary fronds covered by endothelial cells. Histopathological differentiation from angiosarcoma can be challenging but is very important to avoid aggressive treatments. Complete surgical excision is frequently curative. Uncompleted excision can lead to recurrences.We describe the clinical and histological findings of intravascular papillary endotelial hyperplasia in the left lower lid of a 72-year-old woman who had a painless mass since 15 years ago, that caused hypertropia of her left eye. Histopathological examination revealed intralesional calcification, which is not an usual feature in this entity.The patient underwent complete surgical resection of the mass and there was no evidence of recurrence after 5 months of follow-up after surgery.

Masson's tumor of the ocular surface - A rare clinical entity.

Masson's tumor or intravascular papillary endothelial hyperplasia (IPEH) is a benign vascular lesion usually involving the head-and-neck region. On histopathological examination, it consists of reactive proliferation of endothelial cells with papillary formations which is the key to diagnosis. This rare entity was first described in 1923 by Pierre Masson. Lesions involving orbit and eyelids have been reported before. Here, we report a case of Masson's tumor which occurred in the lid margin and later in the conjunctiva which regressed completely after excision.

Intravascular Papillary Endothelial Hyperplasia: Case Report of a Recurrent Masson's Tumor of the Finger and Review of Literature.

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.