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Masson's tumour: Differential diagnosis of neck lump in children
International Journal of Pediatric Otorhinolaryngology Extra (2006) 1, 196—199
www.elsevier.com/locate/ijporl
CASE REPORT
Masson’s tumour: Differential diagnosis of neck lump in children Pablo Martinez Devesa, Salil Sood *, Meena Chaudhari, Deepak Gupta, Suchir Maitra Otolaryngology Departments, Great Western Hospital, Marlborough Road, Swindon, Wiltshire SN3 6BB, UK Received 2 April 2006; accepted 5 May 2006
KEYWORDS Masson’s tumour; Angiosarcoma
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. # 2006 Elsevier Ireland Ltd. All rights reserved.
1. Introduction PEH is a rare diagnosis in the differential of lateral neck masses. The lesion was originally named ´mangioendothe ´liome ve ´ge ´tant intravasculaire’ ‘he 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 * Corresponding author at: Department of ENT, Great Western Hospital, Swindon, SN3 6BB, UK. Tel.: +44 1793 604020/1197; fax: +44 1793 604406. E-mail address: [email protected] (S. Sood).
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—5], intracranial [6,7], orbit [8], nasal sinuses [9,10], parotid [11], mandible [12], oral cavity [13—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—21]) and lateral neck [5].
2. Case report A 5-year-old girl presented to the Otolaryngology department with a 3-month history of a mass in the
1871-4048/$ — see front matter # 2006 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.pedex.2006.05.003
Masson’s tumour: Differential diagnosis of neck lump in children
197
Plate 1 The specimen contains a circumscribed lesion with a wall of fibrous tissue filled with blood and fibrous tissue.
Fig. 1 T1 19 mm 43 mm mass within the left supraclavicular fossa. It returns predominantly intermediate signal on T1 weighted sequences although there are a few areas of high signal.
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 19 mm 43 mm mass within the left supraclavicular
Fig. 2 On T2 image it is predominantly low signal. There are internal septations, which return high signal.
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. 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.
3. Discussion Masson first described a case of a 68-year-old man with a painful hemorrhoid that could not be
Plate 2 High power view showing a blood vessel filled with fibrous tissue arranged in papillae covered by bland endothelial cells.
198 reduced. He observed microscopically that the internal lining of the large veins contained papillae, covered by endothelium (Slides 1 and 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
P.M. Devesa et al. 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].
References [1] C. Steffen, The man behind the eponym: C.L. Masson, Am. J. Dermatopathol. 25 (1) (2003) 71—76. [2] J. Amerigo, A. Matilla, R. Gonzalez-Campora, H. GaleraDavidson, Vegetant intravascular heangioendothelioma of the skin. Report of 3 cases with a critical pathogenic study, Dermatologica 159 (1979) 476—481. [3] C. Cozzutto, M. Guarino, P. Dodero, G. Nattero, Intravascular endotelial proliferations in children, Am. J. Clin. Pathol. 71 (1979) 247—252. [4] E.B. Luce, M.T. Montgomery, S.W. Redding, T.B. Aufdemore, Intravascular angiomatosis (Masson’s lesion), J. Oral Maxillofac. Surg. 46 (1988) 736—744. [5] L. Pantanowitz, R. Muc, M. Spanger, H. Sonnendecker, W.A. McIntosh, Intravascular papillary endothelial hyperplasia (Masson’s tumour) manifesting as a lateral neck mass, Ear Nose Throat J. 79 (10) (2000) 806—812. [6] A.M. Avellino, G.A. Grant, A.B. Harris, S.K. Wallace, C.M. Shaw, Recurrent intracranial Masson’s vegetant intravascular hemangioendothelioma. Case report and review of the literature, J. Neurosurg. 91 (1999) 308—312. [7] M.R. Stoffman, J.H. Kim, Masson’s vegetant hemangioendothelioma: case report and literature review, J. Neurooncol. 61 (2003) 17—22. [8] F.L. Weber, J. Babel, Intravascular papillary endothelial hyperplasia of the orbit, Br. J. Ophthalmol. 65 (1981) 18—22. [9] Y. Stern, D. Braslavsky, K. Segal, T. Shpitzer, A. Abraham, Intravascular papillary endothelial hyperplasia in the maxillary sinus, Arch. Otolaryngol. Head Neck Surg. 117 (1991) 1182—1184. [10] J.L. Lancaster, D.J. Alderson, I.W. Sherman, A.H. Clark, Papillary endothelial hyperplasia (Masson’s tumour) of the maxillary sinus, J. Laryngol. Otolaryngol. 112 (5) (1998) 500—502. [11] R.L. Corio, R.B. Brannon, T.M. Tarpley, Intravascular papillary endothelial hyperplasia of the head and neck, Ear Nose Throat J. 61 (1982) 88—91. [12] A. Komori, M. Koike, T. Kinjo, T. Azuma, M. Yoshinari, H. Inaba, Central intravascular papillary endothelial hyperplasia of the mandible, Virchows Arch. A: Pathol. Ant. Histopathol. 403 (4) (1984) 453—459. [13] K. Tsosios, I.G. Koutlas, S.I. Papanicolau, Intravascular papillary endotelial hyperplasia of the oral soft tisu ´es: report of 18 cases and review of the literature, J. Oral Maxillofac. Surg. 52 (1994) 1263—1268. [14] R. Torne Escasanay, P. Umbert Millet, Masson’s pseudoangiosarcoma of the tongue: report of two cases, J. Cutan. Pathol. 12 (1985) 66—71. [15] Y. Stern, D. Braslavsky, T. Shpitzer, R. Feinmesser, Papillary endothelial hyperplasia in the tongue: a benign lesion that may be mistaken for angiosarcoma, J. Otolaryngol. 23 (1994) 81—83. [16] G.C. Ejeckam, M. Orizaga, J. Gerin-Lajoic, G. Castilio, Intravascular angiomatosis of the pharynx. (Masson’s vegetant intravascular haemangioendothelioma)–—a case report, Br. J. Oral Surg. 16 (1978) 105—110.
Masson’s tumour: Differential diagnosis of neck lump in children [17] J.K. Pye, A case of Masson’s vegetant, intravascular haemangio endothelioma presenting in a large neck vein, Clin. Oncol. 9 (1983) 57—59. [18] J.S. Youngerman, Intravascular papillary endothelial hyperplasia, Arch. Otolaryngol. 110 (1984) 274—275, 277. [19] K.T. Chen, Extravascular papillary endothelial hyperplasia, J. Surg. Oncol. 36 (1987) 52—54. [20] H. Hashimoto, Y. Daimaru, M. Enjoji, Intravascular papillary endothelial hyperplasia. A clinicopathologic study of 91 cases, Am. J. Dermatopathol. 5 (1983) 539—546.
199
[21] A. Winkler, B. Mueller, P. Diem, Masson’s papillary endothelial hyperplasia mimicking a poor differentiated thyroid carcinoma: a case report, Eur. J. Endocrinol. 145 (2001) 667—668. [22] B.E. Garber, A.J. Prestipino, H.M. Pollack, S.R. Levine, K.E. Whitmore, Masson’s tumour of the kidney: a new renal lesion, J. Urol. 143 (1990) 344—346. [23] M. Gleeson, A. Herbert, A. Richards, Management of lateral neck masses in adults. BMJ 320 (2000) 1521—1524. [24] S. Roy, Intravascular papillary endothelial hyperplasia (Masson’s tumour), GI Path Online-India.
www.elsevier.com/locate/ijporl
CASE REPORT
Masson’s tumour: Differential diagnosis of neck lump in children Pablo Martinez Devesa, Salil Sood *, Meena Chaudhari, Deepak Gupta, Suchir Maitra Otolaryngology Departments, Great Western Hospital, Marlborough Road, Swindon, Wiltshire SN3 6BB, UK Received 2 April 2006; accepted 5 May 2006
KEYWORDS Masson’s tumour; Angiosarcoma
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. # 2006 Elsevier Ireland Ltd. All rights reserved.
1. Introduction PEH is a rare diagnosis in the differential of lateral neck masses. The lesion was originally named ´mangioendothe ´liome ve ´ge ´tant intravasculaire’ ‘he 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 * Corresponding author at: Department of ENT, Great Western Hospital, Swindon, SN3 6BB, UK. Tel.: +44 1793 604020/1197; fax: +44 1793 604406. E-mail address: [email protected] (S. Sood).
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—5], intracranial [6,7], orbit [8], nasal sinuses [9,10], parotid [11], mandible [12], oral cavity [13—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—21]) and lateral neck [5].
2. Case report A 5-year-old girl presented to the Otolaryngology department with a 3-month history of a mass in the
1871-4048/$ — see front matter # 2006 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.pedex.2006.05.003
Masson’s tumour: Differential diagnosis of neck lump in children
197
Plate 1 The specimen contains a circumscribed lesion with a wall of fibrous tissue filled with blood and fibrous tissue.
Fig. 1 T1 19 mm 43 mm mass within the left supraclavicular fossa. It returns predominantly intermediate signal on T1 weighted sequences although there are a few areas of high signal.
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 19 mm 43 mm mass within the left supraclavicular
Fig. 2 On T2 image it is predominantly low signal. There are internal septations, which return high signal.
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. 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.
3. Discussion Masson first described a case of a 68-year-old man with a painful hemorrhoid that could not be
Plate 2 High power view showing a blood vessel filled with fibrous tissue arranged in papillae covered by bland endothelial cells.
198 reduced. He observed microscopically that the internal lining of the large veins contained papillae, covered by endothelium (Slides 1 and 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
P.M. Devesa et al. 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].
References [1] C. Steffen, The man behind the eponym: C.L. Masson, Am. J. Dermatopathol. 25 (1) (2003) 71—76. [2] J. Amerigo, A. Matilla, R. Gonzalez-Campora, H. GaleraDavidson, Vegetant intravascular heangioendothelioma of the skin. Report of 3 cases with a critical pathogenic study, Dermatologica 159 (1979) 476—481. [3] C. Cozzutto, M. Guarino, P. Dodero, G. Nattero, Intravascular endotelial proliferations in children, Am. J. Clin. Pathol. 71 (1979) 247—252. [4] E.B. Luce, M.T. Montgomery, S.W. Redding, T.B. Aufdemore, Intravascular angiomatosis (Masson’s lesion), J. Oral Maxillofac. Surg. 46 (1988) 736—744. [5] L. Pantanowitz, R. Muc, M. Spanger, H. Sonnendecker, W.A. McIntosh, Intravascular papillary endothelial hyperplasia (Masson’s tumour) manifesting as a lateral neck mass, Ear Nose Throat J. 79 (10) (2000) 806—812. [6] A.M. Avellino, G.A. Grant, A.B. Harris, S.K. Wallace, C.M. Shaw, Recurrent intracranial Masson’s vegetant intravascular hemangioendothelioma. Case report and review of the literature, J. Neurosurg. 91 (1999) 308—312. [7] M.R. Stoffman, J.H. Kim, Masson’s vegetant hemangioendothelioma: case report and literature review, J. Neurooncol. 61 (2003) 17—22. [8] F.L. Weber, J. Babel, Intravascular papillary endothelial hyperplasia of the orbit, Br. J. Ophthalmol. 65 (1981) 18—22. [9] Y. Stern, D. Braslavsky, K. Segal, T. Shpitzer, A. Abraham, Intravascular papillary endothelial hyperplasia in the maxillary sinus, Arch. Otolaryngol. Head Neck Surg. 117 (1991) 1182—1184. [10] J.L. Lancaster, D.J. Alderson, I.W. Sherman, A.H. Clark, Papillary endothelial hyperplasia (Masson’s tumour) of the maxillary sinus, J. Laryngol. Otolaryngol. 112 (5) (1998) 500—502. [11] R.L. Corio, R.B. Brannon, T.M. Tarpley, Intravascular papillary endothelial hyperplasia of the head and neck, Ear Nose Throat J. 61 (1982) 88—91. [12] A. Komori, M. Koike, T. Kinjo, T. Azuma, M. Yoshinari, H. Inaba, Central intravascular papillary endothelial hyperplasia of the mandible, Virchows Arch. A: Pathol. Ant. Histopathol. 403 (4) (1984) 453—459. [13] K. Tsosios, I.G. Koutlas, S.I. Papanicolau, Intravascular papillary endotelial hyperplasia of the oral soft tisu ´es: report of 18 cases and review of the literature, J. Oral Maxillofac. Surg. 52 (1994) 1263—1268. [14] R. Torne Escasanay, P. Umbert Millet, Masson’s pseudoangiosarcoma of the tongue: report of two cases, J. Cutan. Pathol. 12 (1985) 66—71. [15] Y. Stern, D. Braslavsky, T. Shpitzer, R. Feinmesser, Papillary endothelial hyperplasia in the tongue: a benign lesion that may be mistaken for angiosarcoma, J. Otolaryngol. 23 (1994) 81—83. [16] G.C. Ejeckam, M. Orizaga, J. Gerin-Lajoic, G. Castilio, Intravascular angiomatosis of the pharynx. (Masson’s vegetant intravascular haemangioendothelioma)–—a case report, Br. J. Oral Surg. 16 (1978) 105—110.
Masson’s tumour: Differential diagnosis of neck lump in children [17] J.K. Pye, A case of Masson’s vegetant, intravascular haemangio endothelioma presenting in a large neck vein, Clin. Oncol. 9 (1983) 57—59. [18] J.S. Youngerman, Intravascular papillary endothelial hyperplasia, Arch. Otolaryngol. 110 (1984) 274—275, 277. [19] K.T. Chen, Extravascular papillary endothelial hyperplasia, J. Surg. Oncol. 36 (1987) 52—54. [20] H. Hashimoto, Y. Daimaru, M. Enjoji, Intravascular papillary endothelial hyperplasia. A clinicopathologic study of 91 cases, Am. J. Dermatopathol. 5 (1983) 539—546.
199
[21] A. Winkler, B. Mueller, P. Diem, Masson’s papillary endothelial hyperplasia mimicking a poor differentiated thyroid carcinoma: a case report, Eur. J. Endocrinol. 145 (2001) 667—668. [22] B.E. Garber, A.J. Prestipino, H.M. Pollack, S.R. Levine, K.E. Whitmore, Masson’s tumour of the kidney: a new renal lesion, J. Urol. 143 (1990) 344—346. [23] M. Gleeson, A. Herbert, A. Richards, Management of lateral neck masses in adults. BMJ 320 (2000) 1521—1524. [24] S. Roy, Intravascular papillary endothelial hyperplasia (Masson’s tumour), GI Path Online-India.