International Journal of Pediatric Otorhinolaryngology Extra
Volume 1, Issue 3 , Pages 196-199, September 2006

Masson's tumour: Differential diagnosis of neck lump in children

Otolaryngology Departments, Great Western Hospital, Marlborough Road, Swindon, Wiltshire SN3 6BB, UK

Received 2 April 2006; accepted 5 May 2006.

Article Outline

Summary 

Masson's tumour or papillary endothelial hyperplasia (PEH) also known as Vegetant Intravascular Haemangioendothelioma or Intravascular Angiomatosis is a uncommon, benign proliferation of endothelial cells in normal blood vessels or vascular conditions like hemangiomas, pyogenic granuloma, etc. It is important to distinguish PEH from angiosarcoma.

Although it has been reported to affect the head and neck region it is still frequently forgotten in the differential diagnosis of lateral neck masses in children.

We discuss the management of a case of Masson's tumour presenting as a lateral neck mass in a 5-year old, and review the literature available in this particular topic.

Keywords: Masson's tumour, Angiosarcoma

 

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1. Introduction 

PEH is a rare diagnosis in the differential of lateral neck masses. The lesion was originally named ‘hémangioendothéliome végétant intravasculaire’ by Pierre Masson [1] in 1923. PEH is a benign lesion of vascular origin. Hennschen believed this was caused by an excessive proliferation of endothelial cells in normal blood vessels or vascular malformations in response to inflammation and stasis. Most evidence to date suggest this is an unusual form of organizing thrombus. It is important to distinguish PEH from angiosarcoma, with which it shares some histological similarities (its primary growth pattern).

There are reports of Masson's tumour involving the skin and subcutaneous tissues of scalp, face and neck [2], [3], [4], [5], intracranial [6], [7], orbit [8], nasal sinuses [9], [10], parotid [11], mandible [12], oral cavity [13], [14], [15] and pharynx [16].

There are six reports of this condition presenting as a neck mass, mainly anterior neck (external jugular vein [17], platysma [18], thyroid [19], [20], [21]) and lateral neck [5].

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2. Case report 

A 5-year-old girl presented to the Otolaryngology department with a 3-month history of a mass in the left supraclavicular fossa. There was a family history of neurofibromatosis.

A chest X-ray was normal. An ultrasound scan of the neck revealed a well-defined benign looking mass which lay just beneath the skin and separate from other structures. It had a very well-defined border and a number of blood vessels within it. The appearances suggested that it was a neurofibroma of a superficial nerve.

A MRI scan of the head and neck areas showed a 19mm×43mm mass within the left supraclavicular fossa. It had unusual imaging characteristic. It returned predominantly intermediate signal on T1 weighted sequences although there were a few areas of high signal (Figure 1). On T2 it was predominantly low signal (Figure 2). There were internal septations, which returned a high signal. There was also a nodular area. The lesion was separate from the underlying clavicle and deltoid muscle. It appeared also separate from subclavian vessels and brachial plexus.

  • View full-size image.
  • Fig. 1. 

    T1 19mm×43mm mass within the left supraclavicular fossa. It returns predominantly intermediate signal on T1 weighted sequences although there are a few areas of high signal.

A small volume cervical lymphadenopathy was also noticed, but no other lesion was identified.

With the suspected clinical diagnosis (not challenged by imaging) of neurofibroma an excision biopsy of the left neck lump was carried out completely and uneventfully.

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3. Discussion 

Masson first described a case of a 68-year-old man with a painful hemorrhoid that could not be reduced. He observed microscopically that the internal lining of the large veins contained papillae, covered by endothelium (Plate 1, Plate 2). These papillae enlarged to form polypoid projections or vegetations. The blood could not circulate except through tiny spaces, and finally a thrombus formed.

PEH affects females more than males and can occur at any age group.

Clinically, like other vascular malformations usually presents as a reddish-blue or purple nodule under the skin or mucous membrane. Although vascular in origin, these are solid lesions. In our case there were no clinical signs of a vascular malformation, nor of it being cystic in nature, but of a solid mass, well defined and attached to the subcutaneous tissue.

Imaging plays an important role in the investigation of any neck mass. Ultrasound is a non-invasive, operator-dependent imaging modality particularly effective in differentiating vascular/cystic structures from solid lesions. In this case, despite a number of vessels in it, the suspected diagnosis (influenced by the family history) was that of neurofibroma.

Magnetic resonance imaging, inspite of its exquisite soft tissue discrimination is equivocal in the diagnosis [22], [23], as the image depends on the amount of blood vessels and thrombi within the lesion. PEH will appear to show low to intermediate signal with areas of high signal intensity on T1 weighted sequences, and predominantly low signal on T2 weighted sequences, with or without significant postcontrast enhancement (not used in our case).

Our case was thought to represent a complicated neurofibroma, although low signal in the neurofibroma is unusual, this could happen if there was previous haemorrhage. The imaging features would be unusual for a lymph node and there was evidence of vascular nature.

PEH can be classified in three types: a pure or primary form that arises de novo in dilated vascular spaces; a mixed type (secondary or reactive) due to focal change in a pre-existing vascular lesion (haemangioma, pyogenic granuloma or vascular malformation); and rarely, in an extra vascular location as a result of organization of a haematoma.

Histologically, the diagnosis is straightforward in an intact vessel. However, rupture of vessel of origin leads to processes spilling over into the adjacent tissue. The papillary growth pattern and prominent endothelium can simulate the growth pattern of a haemangiosarcoma [24].

In our patient, the biopsy contained solid lesion consisting of pre-existing vessels filled with a papillary proliferation consisting of collagenous covered by endothelial cells. There was no pleomorphism and atypia and the diagnosis was readily made.

Masson's tumour is cured by simple excision and very rarely recurs [6].

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References 

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  2. Amerigo J, Matilla A, Gonzalez-Campora R, Galera-Davidson H. Vegetant intravascular heangioendothelioma of the skin. Report of 3 cases with a critical pathogenic study. Dermatologica. 1979;159:476–481
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PII: S1871-4048(06)00057-8

doi:10.1016/j.pedex.2006.05.003

International Journal of Pediatric Otorhinolaryngology Extra
Volume 1, Issue 3 , Pages 196-199, September 2006

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