Pathology of Intravascular Papillary Endothelial Hyperplasia (Masson's Tumour)

 Dr Sampurna Roy MD         2022  

Intravascular papillary endothelial hyperplasia (Masson's tumour) was first described by Pierre Masson, who named it 'hemangioendotheliome vegetant intravasculaire' [Bull Soc Anat- (Paris) 1923; 93:517- 532].

Nine years later, Henschen  described this proliferation as a reactive process rather than a true neoplasm and named the lesion ‘endovasculite proliférante thrombopoïétique’.

In 1976, Clearkin and Enzinger coined the current name ‘intravascular papillary endothelial hyperplasia’, which has gained wide acceptance as the best description of this lesion.

It is a reactive condition representing an unusual form of organizing thrombus.

Masson's tumour may either occur in pure form (primary), as a focal change in a  preexisting vascular lesion (hemangioma, pyogenic granuloma or vascular malformation) and rarely in an extravascular location as a result of organization of a haematoma.

Site:  Masson's tumour may occur in any blood vessel in the body, but is commonly located on the fingers, head and neck and trunk. Less frequently, this tumour  has been documented in the upper respiratory tract, skeletal muscle, urogenital systems, renal and hepatic systems, and rarely in the gastrointestinal tract.

Macroscopic Features: The lesion presents as a small (less than 2 cm in diameter), firm, blue or purple nodule.

Sectioning reveals cystic mass containing clotted blood and surrounded by fibrous  pseudocapsule.  

 

Microscopic Features : 

This is a well circumscribed lesion which is usually present within a blood vessel (commonly thin-walled vein).

Multiple small, delicate papillary structures  project into the lumen and these are  associated with some thrombus.

These papillae are lined by single layer of plump endothelial cells surrounding a collagenized core.

There is no multilayering, tufting, solid areas, necrosis and little or no atypia.

There is little evidence of mitoses.  

In the early lesions the the papillae are composed of fibrin.

In the late stage there is clumping and fusion of papillae forming an anastomosing network of blood vessel set in a loose meshlike connective tissue.

Differential diagnosis: This lesion should not be confused with angiosarcoma.

Unlike angiosarcoma, Masson's tumour is usually confined to the blood vessel (passive extension may occur following rupture of vessel) and there is no evidence of pleomorphism, tissue necrosis and mitoses.

Masson's tumour is typically positive for CD31, CD34, SMA, and factor VIII–related antigen. CD105 is expressed on vascular endothelial cells and is positive in primary vascular neoplasms, differentiating  Masson's tumour from angiosarcoma.

This lesion has an excellent prognosis and are usually cured by simple excision.

Intravascular Papillary Endothelial Hyperplasia (Masson's Tumour) - Pathology Infographic

The man behind the eponym: C. L. Pierre Masson.

Intravascular papillary endothelial hyperplasia (Masson's hemangioma) presenting as a soft-tissue sarcoma.

Intravascular papillary endothelial hyperplasia of renal vein: report of 2 cases.  

Intravascular papillary endothelial hyperplasia (Masson's hemangioma) of the liver: a new hepatic lesion.

Extravascular papillary endothelial hyperplasia arising from parapharyngeal space.

Left ventricular haemangioma with papillary endothelial hyperplasia and liver involvement.

Papillary endothelial hyperplasia of the breast: the great impostor for angiosarcoma: a clinicopathologic review of 17 cases.

Intravascular papillary endothelial hyperplasia of the neck masquerading as malignancy on fine-needle aspiration cytology.

Fine needle aspiration cytology of papillary endothelial hyperplasia. A case report.

A case of intravascular papillary endothelial hyperplasia (Masson's tumor) arising from renal sinus.