Intravascular papillary endothelial hyperplasia

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Intravascular papillary endothelial hyperplasia
Other namesMasson's hemangio-endotheliome vegetant intravasculaire, Masson's lesion, Masson's pseudoangiosarcoma, Masson's tumor, and papillary endothelial hyperplasia [1]
Intravascular papillary endothelial hyperplasia -- very low mag.jpg
Micrograph of intravascular papillary endothelial hyperplasia. H&E stain.
Specialty Oncology, rheumatology   OOjs UI icon edit-ltr-progressive.svg

Intravascular papillary endothelial hyperplasia (IPEH), also known as Masson's hemangio-endotheliome vegetant intravasculaire, Masson's lesion, Masson's pseudoangiosarcoma, Masson's tumor, and papillary endothelial hyperplasia, is a rare, benign tumor. It may mimic an angiosarcoma, with lesions that are red or purplish 5-mm to 5-cm papules and deep nodules on the head, neck, or upper extremities. [1] [2] : 592 

Contents

The cause of intravascular papillary endothelial hyperplasia is unknown. Risk factors include a history of local trauma or prior vascular disorders.

Immunohistochemistry and histopathology are used to make the diagnosis of intravascular papillary endothelial hyperplasia. Differential diagnosis includes vascular malformation, angioma, pyogenic granuloma, angiosarcoma, epithelioid hemangioendothelioma, Kaposi’s sarcoma, and other more rare vascular tumors.

Three forms of intravascular papillary endothelial hyperplasia exist: the primary or pure form, the secondary or mixed form, and the extravascular form.

The treatment for intravascular papillary endothelial hyperplasia is complete surgical resection.

Signs and symptoms

Intravascular papillary endothelial hyperplasia typically manifest as deep nodules or well-defined, round, red, or purple superficial papules. They are usually tiny, ranging in size from 0.5 to 5 cm. [3] IPEH lesions are most common in the digits and among blood arteries throughout the body, although they can also form in the head, neck, and body. [4] [5] Atypical IPEH lesions have occasionally been seen in cerebral aneurysms or the abdominal organs. [6] [7]

Causes

Although there are numerous possibilities, the pathophysiology and causation of intravascular papillary endothelial hyperplasia remain unknown. [8] A history of local trauma or prior vascular disorders (such as hemangiomas, blood stasis, etc.) are risk factors. [9] It will usually be idiopathic. [8]

Diagnosis

Immunohistochemistry staining in addition to a thorough histomorphological evaluation are necessary for a correct diagnosis. [10] A crucial tool for diagnosing it is microscopy. IPEH is made up of an intravascular proliferation of many papillae with an endothelial surface and a connective tissue core. [11] It differs from other neoplastic lesions in that it is often encapsulated or well-circumscribed, displaying distinctive papillary fronds, and the vascular wall completely restricts the proliferative process. [12]

Only in cases when the endothelium origin of the lesion is in doubt may immunohistochemical confirmation be necessary. In these situations, endothelial cell markers that would highlight the endothelium lining around the papillary tufts, such as von Willebrand factor, CD31, factor XIIIa, and CD43, may be employed. [10]

The differential diagnosis includes vascular malformation, angioma, pyogenic granuloma, angiosarcoma, epithelioid hemangioendothelioma, Kaposi’s sarcoma, and other more rare vascular tumors. [3]

Classification

Three forms of intravascular papillary endothelial hyperplasia exist: the primary or pure form, the secondary or mixed form, and the extravascular form. [3]

The most prevalent variety, known as the primary or pure form, develops in subcutaneous soft tissue and usually arises in a dilated channel, most commonly a vein rather than an artery. [13] Preexisting vascular anomalies are found in the secondary/mixed presentation. [9] Lastly, the least frequent form of hematomas is the extravascular variety. [5]

Treatment

Complete surgical resection is the course of treatment. [3] Wide margins of excision are not indicated, although partial resection can lead to recurrence. [14]

See also

References

  1. 1 2 Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN   978-1-4160-2999-1.
  2. ↑ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN   0-7216-2921-0.
  3. 1 2 3 4 Boukovalas, Stefanos; Dillard, Rachel; Qiu, Suimin; Cole, Eric L (2017). "Intravascular Papillary Endothelial Hyperplasia (Masson's Tumor): Diagnosis the Plastic Surgeon Should Be Aware of". Plastic and Reconstructive Surgery — Global Open. 5 (1). Ovid Technologies (Wolters Kluwer Health): e1122. doi:10.1097/gox.0000000000001122. ISSN   2169-7574. PMC   5293289 .
  4. ↑ Clearkin, K. P.; Enzinger, F. M. (1976). "Intravascular papillary endothelial hyperplasia". Archives of Pathology & Laboratory Medicine. 100 (8): 441–444. ISSN   0003-9985. PMID   947306.
  5. 1 2 T, Kuo; CP, Sayers; J, Rosai (1976). "Masson's "vegetant intravascular hemangioendothelioma:" a lesion often mistaken for angiosarcoma: study of seventeen cases located in the skin and soft tissues". Cancer. 38 (3): 1227–1236. doi:10.1002/1097-0142(197609)38:3<1227::aid-cncr2820380324>3.0.co;2-6. ISSN   0008-543X. PMID   986234.
  6. ↑ Hong, Seok-Gi; Cho, Hyeon-Min; Chin, Hyung-min; Park, Il-Young; Yoo, Jin-Young; Hwang, Sung-Soo; Kim, Jun-Gi; Park, Woo-Bae; Chun, Chung-Soo (2004). "Intravascular Papillary Endothelial Hyperplasia (Masson's Hemangioma) of the Liver: A New Hepatic Lesion". Journal of Korean Medical Science. 19 (2). Korean Academy of Medical Sciences: 305. doi:10.3346/jkms.2004.19.2.305. ISSN   1011-8934. PMC   2822318 .
  7. ↑ Lee, W.; Hui, F.; Sitoh, Y. Y. (2004). "Intravascular papillary endothelial hyperplasia in an intracranial thrombosed aneurysm: 3T magnetic resonance imaging and angiographical features". Singapore Medical Journal. 45 (7): 330–333. ISSN   0037-5675. PMID   15221049.
  8. 1 2 Tedla, Miroslav; Bežová, Miroslava; Biró, Csaba; Tedlová, Eva; Eng, Chee-Yean; Zeleník, Karol (2014). "Intravascular papillary endothelial hyperplasia of larynx: Case report and literature review of all head and neck cases". Otolaryngologia Polska. 68 (4). Index Copernicus: 200–203. doi:10.1016/j.otpol.2014.03.002. ISSN   0030-6657. PMID   24981303.
  9. 1 2 Nwokolo, Vania; Veeramachaneni, Ravindra; Rivas, Saul D. (2020). "Vulvar intravascular papillary endothelial hyperplasia or Masson's tumor: A case report". Case Reports in Women's Health. 28 e00250. Elsevier BV. doi:10.1016/j.crwh.2020.e00250. ISSN   2214-9112. PMC   7451632 . PMID   32874931.
  10. 1 2 Akdur, Noyan Can; Donmez, Melahat; Gozel, Serap; Ustun, Huseyin; Hucumenoglu, Sema (2013-10-14). "Intravascular papillary endothelial hyperplasia: histomorphological and immunohistochemical features". Diagnostic Pathology. 8 (1) 167. Springer Science and Business Media LLC. doi: 10.1186/1746-1596-8-167 . ISSN   1746-1596. PMC   4016006 .
  11. ↑ Malik, Ajay; Mahapatra, QuryS; Sahai, Kavita; Mani, NS (2015). "Intravascular papillary endothelial hyperplasia: An unusual histopathological entity". Indian Dermatology Online Journal. 6 (4). Medknow: 277. doi: 10.4103/2229-5178.160269 . ISSN   2229-5178. PMC   4513410 . PMID   26225335.
  12. ↑ Fasina, Oluyemi; Adeoye, Adewunmi; Akang, Effiong (2012-09-13). "Orbital intravascular papillary endothelial hyperplasia in a Nigerian child: a case report and review of the literature". Journal of Medical Case Reports. 6 (1) 300. Springer Science and Business Media LLC. doi: 10.1186/1752-1947-6-300 . ISSN   1752-1947. PMC   3459712 . PMID   22974134.
  13. ↑ Kim, Ok Hwa; Kim, Yeon Mee; Choo, Hye Jung; Lee, Sun Joo; Kim, Yeong Mi; Yi, Jae Hyuck; Lee, Yong Hwan (2016). "Subcutaneous intravascular papillary endothelial hyperplasia: ultrasound features and pathological correlation". Skeletal Radiology. 45 (2): 227–233. doi:10.1007/s00256-015-2281-7. ISSN   0364-2348. PMID   26559670.
  14. ↑ Avellino, Anthony M.; Grant, Gerald A.; Harris, A. Basil; Wallace, Sharon K.; Shaw, Cheng-Mei (1999). "Recurrent intracranial Masson's vegetant intravascular hemangioendothelioma". Journal of Neurosurgery. 91 (2). Journal of Neurosurgery Publishing Group (JNSPG): 308–312. doi:10.3171/jns.1999.91.2.0308. ISSN   0022-3085. PMID   10433320.

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