Intravascular Papillary Endothelial Hyperplasia of the Maxillary Sinus in Patient with Tricuspid Atresia

  • Peter CookeEmail author
  • David Goldrich
  • Alfred Marc Iloreta
  • Abeer Salama
  • Raj Shrivastava
Case Report


Intravascular papillary endothelial hyperplasia (IPEH) is a benign, highly vascularized, endothelial growth that can be mischaracterized as a malignancy. While hundreds of IPEH cases are reported, only four occurred in the maxillary sinus. We present the case of a 28-year-old male who underwent surgical resection of IPEH of the right maxillary sinus. An additional consideration was the patient’s condition of univentricular tricuspid atresia which contributed to chronic hypoxemia and polycythemia. After complete resection from the maxillary sinus, post-operational workup determined the lesion to be IPEH. Given the potential for misdiagnosis of IPEH, careful histopathologic evaluation is required in order to avoid improper treatment.


Intravascular papillary endothelial hyperplasia Masson’s tumor Univentricular tricuspid atresia Hypercoagulable Sphenopalatine artery 


Compliance with Ethical Standards

Conflict of interest

The authors declare that they have no conflicts of interest or financial disclosures.


  1. 1.
    Luce EB, Montgomery MT, Redding SW, Aufdemorte TB. Intravascular angiomatosis (Masson’s lesion). J Oral Maxillofac Surg. 1988;46:736–41.CrossRefGoogle Scholar
  2. 2.
    Park KK, Won YS, Yang JY, Choi CS, Han KY. Intravascular papillary endothelial hyperplasia (Masson tumor) of the skull: case report and literature review. J Korean Neurosurg Soc. 2012;52(1):52–4.CrossRefGoogle Scholar
  3. 3.
    Pins MR, Rosenthal DI, Springfield DS, Rosenberg AE. Florid extravascular papillary endothelial hyperplasia (Masson’s pseudoangiosarcoma) presenting as a soft-tissue sarcoma. Arch Pathol Lab Med. 1993;117:259–63.Google Scholar
  4. 4.
    Stern Y, Braslavsky D, Segal K, Shpitzer T, Abraham A. Intravascular papillary endothelial hyperplasia in the maxillary sinus: a benign lesion that may be mistaken for angiosarcoma. Arch Otolaryngol Head Neck Surg. 1991;117(10):1182–4.CrossRefGoogle Scholar
  5. 5.
    Lancaster J, Alderson D, Sherman I, Clark A. Papillary endothelial hyperplasia (Masson’s tumour) of the maxillary sinus. J Laryngol Otol. 1998;112(5):500–2.CrossRefGoogle Scholar
  6. 6.
    Wang ZH, Hsin CH, Chen SY, Lo CY, Cheng PW. Sinonasal intravascular papillary endothelial hyperplasia successfully treated by endoscopic excision: a case report and review of the literature. Auris Nasus Larynx. 2009;36:363–6.CrossRefGoogle Scholar
  7. 7.
    D’Aguanno V, Ralli M, De Virgilio A, Greco A, de Vincentiis M. The role of differential diagnosis in intravascular papillary endothelial hyperplasia of the sinonasal cavity mimicking angiosarcoma: a case report. Oncol Lett. 2019;17(1):1253.Google Scholar
  8. 8.
    Avellin AM, Grant GA, Harris A, Wallace SK, Shaw C. Recurrent intracranial Masson’s vegetant intravascular hemangioendothelioma. J Neurosurg. 1999;91(2):308–12.CrossRefGoogle Scholar
  9. 9.
    Akdur NC, Donmez M, Gozel S, Ustun H, Hucumenoglu S. Intravascular papillary endothelial hyperplasia: histomorphological and immunohistochemical features. Diagn Pathol. 2013;8(1):167.CrossRefGoogle Scholar
  10. 10.
    Ohsawa M, Naka N, Tomita Y, Kawamori D, Kanno H, Aozasa K. Use of immunohistochemical procedures in diagnosing angiosarcoma. Evaluation of 98 cases. Cancer. 1995;75:2867–74.CrossRefGoogle Scholar
  11. 11.
    Masson P. Haemangioendothelioma vegetant intra-vasculare. Bull Soc Anat Paris [In French]. 1923;8:517–23.Google Scholar
  12. 12.
    Rosai J, Akerman LR. Intravenous atypical vascular proliferation: a cutaneous lesion simulating a malignant blood vessel tumor. Arch Dermatol. 1974;109(5):714–7.CrossRefGoogle Scholar
  13. 13.
    Kavanagh GM, Rooney N, Kennedy CT. Masson’s pseudoangiosarcoma. J R Soc Med. 1991;84(12):745–6.CrossRefGoogle Scholar
  14. 14.
    Clearkin KP, Enzinger FM. Intravascular papillary endothelial hyperplasia. Arch Pathol Lab Med. 1976;100:441–4.Google Scholar
  15. 15.
    Hashimoto H, Daimaru Y, Enjoji M, et al. Intravascular papillary endothelial hyperplasia: a clinicopathologic study of 91 cases. Am J Dermatopathol. 1983;5:539–46.CrossRefGoogle Scholar
  16. 16.
    Moon WS, Chung GH, Hong KH. Intravascular papillary endothelial hyperplasia in a vascular lesion of the paranasal sinus. Arch Pathol Lab Med. 2000;124:1224–7.Google Scholar
  17. 17.
    Kuo TT, Sayers CP, Rosai J. Masson’s, “vegetant intravascular hemangioendothelioma:” a lesion often mistaken for angiosarcoma. Study of seventeen cases located in the skin and soft tissues. Cancer. 1976;38(3):1227–36.CrossRefGoogle Scholar
  18. 18.
    Kristof RA, Van Roost D, Wolf HK, Schramm J. Intravascular papillary endothelial hyperplasia of the sellar region. Report of three cases and review of the literature. J Neurosurg. 1997;86:558–63.CrossRefGoogle Scholar
  19. 19.
    Prchal JT. Hypoxia and thrombosis. Blood. 2018;132(4):348–9.CrossRefGoogle Scholar
  20. 20.
    Van Doorn CA, Tsang VT, de Leval MR. Tricuspid atresia and the functionally single ventricle. In: Yuh DD, Vricella LA, Yang SC, Doty JR, editors. Johns Hopkins textbook of cardiothoracic surgery. 2nd ed. New York: McGraw-Hill; 2014.Google Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Otolaryngology- Head and Neck SurgeryIcahn School of Medicine at Mount SinaiNew YorkUSA
  2. 2.Department of PathologyIcahn School of Medicine at Mount SinaiNew YorkUSA
  3. 3.Department of NeurosurgeryIcahn School of Medicine at Mount SinaiNew YorkUSA

Personalised recommendations