Indian Journal of Dermatopathology and Diagnostic Dermatology

CASE REPORT
Year
: 2019  |  Volume : 6  |  Issue : 2  |  Page : 86--88

Masson's tumor on the forehead: A rare and Benign entity


Bhushan A Darkase1, Atul M Dongre2, Paras Choudhary1, Uday S Khopkar1,  
1 Department of Dermatology Venereology, Seth G.S. College and K.E.M Hospital, Mumbai, Maharashtra, India
2 Department of Leprology, Seth G.S. College and K.E.M Hospital, Mumbai, Maharashtra, India

Correspondence Address:
Dr. Uday S Khopkar
Seth G.S. College and K.E.M Hospital, Mumbai, Maharashtra
India

Abstract

Masson's tumor is a rare, benign, reactive proliferating vascular lesion. The most commonly affected sites are the head, neck, and extremities. We present a 29-year-old male, a case of Masson's tumor over the forehead. Masson's tumor, with the involvement of the forehead is rare and intriguing in this case. Masson's tumor is a benign tumor that may get confused on histopathology with malignant tumors; hence, dermatologists need to understand this entity to avoid unnecessary confusion.



How to cite this article:
Darkase BA, Dongre AM, Choudhary P, Khopkar US. Masson's tumor on the forehead: A rare and Benign entity.Indian J Dermatopathol Diagn Dermatol 2019;6:86-88


How to cite this URL:
Darkase BA, Dongre AM, Choudhary P, Khopkar US. Masson's tumor on the forehead: A rare and Benign entity. Indian J Dermatopathol Diagn Dermatol [serial online] 2019 [cited 2022 May 26 ];6:86-88
Available from: https://www.ijdpdd.com/text.asp?2019/6/2/86/271946


Full Text



 Introduction



Masson's tumor, also termed as intravascular papillary endothelial hyperplasia (IPEH)[1] is a rare benign tumor contributing only 2%[2] of all skin and soft-tissue vascular tumors. The clinical presentation of Masson's tumor as a skin-colored-to-bluish nodule may be confused not only with other benign and malignant vascular lesions but also with nonvascular lesions. We report this case to increase awareness regarding this uncommon and benign tumor.

 Case Report



A 29-year-old male presented with an asymptomatic swelling over the forehead for 1 year. The lesion was completely asymptomatic, initially small, and slow-growing.

On examination, 3 cm × 3 cm soft, painless, nonindurated, skin-colored nodular swelling over the forehead was present without any skin surface changes [Figure 1]. The swelling was compressible, nonpulsatile, and not fixed to any underlying or overlying structures.{Figure 1}

The lesion was completely excised with keeping in differentials as appendegeal tumor and neurofibroma.

Histopathology of the excised specimen showed normal epidermis and upper dermis. The reticular dermis showed intravascular thrombotic tumor with hyalinized eosinophilic material [Figure 2]. Anastomosing vascular channels with intraluminal papillary projections (fronds) were also seen [Figure 3]. The intraluminal papillary projections were lined by prominent endothelial cells [Figure 4]. There was no nuclear atypia.{Figure 2}{Figure 3}{Figure 4}

The tumor was strictly confirmed to intravascular location. The absence of nuclear atypia, solid areas, or necrosis ruled out malignancy.

Classical histopathological appearance with clinical correlation confirmed the diagnosis of Masson's tumor. Immunohistochemical markers were not performed due to the unavailability of facility.

 Discussion



In 1923, Pierre Masson first observed an intravascular papillary endothelium proliferation tumor within the lumen of inflamed hemorrhoidal plexus and called it as “Hemangioendotheliomevegetant intravasculaire.”[3] The reactive and nonneoplastic nature of this lesion was first recognized later by Henschen.[4] Clerkin and Enzinger coined the most widely accepted histological term IPEH in 1976.[1]

Masson's tumor accounts for 2%[2] of benign and malignant vascular tumor of the skin and soft tissue. Masson's tumor affects the skin and subcutis of the head and neck region (23%), lower extremities (77%), and fingers (16%) commonly.[5] Less than 250 cases in the head and neck have been reported over the past 35 years, with <5% involving the forehead.[6]

IPEH is not a primary vascular tumor even though it arises within a vessel. Various possible mechanisms have been postulated regarding etiopathogenesis, a vascular injury resulting in inflammation and stasis with the release of endothelial basic fibroblast growth factor by macrophages plays a crucial role in the formation of IPEH.[6]

Hashimoto et al.[7] classified IPEH into three types; Type I, a “pure,” primary type, which arises de novo in dilated vessels; Type II, a secondary, “mixed” form, which occurs within pre-existing vascular anomalies that may exhibit thrombosis; and Type III or the undermined type, an extravascular type, which occurs in hematomas and is rare.

The first type is the most common.[8] In our case, IPEH is of type I.

Clinically, the primary lesions are usually tender nodules less than 2 cm in size. Clinically, the lesion needs to be differentiated from hemangioma, lymphangioma, angiosarcoma, hematoma, Kaposi sarcoma, traumatic fibroma, traumatic neuroma, and neurofibroma.[8]

The imaging findings in IPEH are usually dubious; histopathology usually confirms the diagnosis. Characteristic histopathology of IPEH shows the intravascular nature of the process consisting of a mass of anastomosing vascular channels with a variable degree of hyaline papillary projections lined by prominent endothelial cells without any atypia.

Masson's tumor is a reactive condition which needs to be distinguished from its histological differentials such as Kaposi's sarcoma, and malignant angiosarcoma, endovascular papillary angioendothelioma or Dabska's tumor [9] since the lesion is, usually, curable by surgical excision alone.

Masson's tumor shows commonly a papillary architecture confined to the intravascular location with an absence of solid areas and necrosis without any evidence of cellular pleomorphism, unlike angiosarcoma.[8] CD105 is positive in primary vascular neoplasms, differentiating IPEH from angiosarcoma.[6]

The prognosis of IPEH is excellent but may recur if it arises in a primary vascular lesion or if the lesion is incompletely excised.

Complete excision was done in our case with computed tomography-scan and local ultrasonography showing no extension to surrounding tissue. The patient is followed up for any evidence of recurrence.

 Conclusion



Masson's tumor is a benign tumor that may get confused on histopathology with malignant tumors; hence, dermatologists need to understand this entity to avoid unnecessary confusion.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Clearkin KP, Enzinger FM. Intravascular papillary endothelial hyperplasia. Arch Pathol Lab Med 1976;100:441-4.
2Bologna-Molina R, Amezcua-Rosas G, Guardado-Luevanos I, Mendoza-Roaf PL, González-Montemayor T, Molina-Frechero N, et al. Intravascular papillary endothelial hyperplasia (Masson's tumor) of the mouth – A case report. Case Rep Dermatol 2010;2:22-6.
3Masson P. Hemangio endothelioma vegetans intravascular. Bull Soc Anat 1923;93:517-23.
4Henschen F. Endovasculitis proliferative and rombopoietic during the local vascular lesion. Ann Anat Pathol 1932;9:113-21.
5Liu DT, Shields CL, Tse GM, Lam DS. Periocular papillary endothelial hyperplasia (Masson's tumour) in Behçet's disease. Acta Ophthalmol 2012;90:e413-5.
6Boukovalas S, Dillard R, Qiu S, Cole EL. Intravascular papillary endothelial hyperplasia (Masson's tumor): Diagnosis the plastic surgeon should be aware of. Plast Reconstr Surg Glob Open 2017;5:e1122.
7Hashimoto H, Daimaru Y, Enjoji M. Intravascular papillary endothelial hyperplasia. A clinicopathologic study of 91 cases. Am J Dermatopathol 1983;5:539-46.
8Gupta A, Chaturvedi S, Jha K, Nazir W. Intravascular papillary endothelial hyperplasia presenting as a cystic mass in the scalp with underlying bone involvement: A rare entity. Int J Appl Basic Med Res 2017;7:269-71.
9Makos CP, Nikolaidou AJ. Intravascular papillary endothelial hyperplasia (Masson's tumor) of the oral mucosa-presentation of two cases and review. Oral Oncol Extra 2004;40:59-62.