- Email: [email protected]
Solitary infantile myofibroma compromising the airway
International Journal of Pediatric Otorhinolaryngology (2004) 68, 1533—1537
www.elsevier.com/locate/ijporl
CASE REPORT
Solitary infantile myofibroma compromising the airway N. Ezea,*, L. Pitkina, S. Crowleyb, P. Wilsonc, H. Dayaa a
Department of Otolaryngology, St. George’s Hospital, London, UK Department of Paediatrics, St. George’s Hospital, London, UK c Department of Histopathology, St. George’s Hospital, London, UK b
Received 1 July 2003; received in revised form 7 February 2004; accepted 7 April 2004
KEYWORDS Neonate; Myofibroma; Airway; Obstruction
Summary Infantile myofibromatosis is an uncommon and benign condition presenting in the neonatal period. It is self-limiting disease that may present as a localised or generalised process. Various examples of this entity have been reported in the literature. This report describes a neonate with a rapidly growing oropharyngeal lesion obstructing the airway that had the typical histological features of an infantile myofibroma. This case report highlights that a solitary myofibroma may be incredibly extensive making complete excision impossible and can be particularly challenging to manage in terms of airway stabilisation. # 2004 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Infantile myofibroma is an uncommon fibrous tumour of infancy [1] and was first described by Stout in 1954 [2]. Infant males are most commonly affected [3]. Myofibroma also occurs in adults and may be multiple or solitary [3,4]. Solitary lesions are more common than multiple ones. They usually involve the head and neck region and the trunk [5]. The lesions appear in the skin, muscle, subcutaneous tissue, bone and viscera and are usually firm, rubbery or hard masses. They may be anywhere between a few millimetres to several centi* Corresponding author. Present address: Department of Otorhinolaryngology, St. George’s Hospital, London SW17 0QT, UK. Tel.: +44 208 672 1255; fax: +44 208 725 3306. E-mail address: [email protected] (N. Eze).
metres in diameter [6] and are generally non-tender and slow growing. Solitary lesions pursue a benign course and regress spontaneously by age 1 or 2. The prognosis is excellent if limited to skin and bone but some may have an aggressive clinical presentation. Patients with visceral involvement usually have multiple lesions involving internal organs and have an 80% mortality [7]. Surgical excision is the treatment of choice. The recurrence rate is low at 10% despite the lesions being non-encapsulated and is attributable to incomplete excision in anatomically inaccessible areas [8]. Although oral myofibroma have been extensively described in the literature, they usually involve the mandible, buccal mucosa and tongue in reducing frequency [9]. Lesions of oral origin have not been described in the literature as extensive enough to
0165-5876/$ — see front matter # 2004 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2004.04.035
1534
obstruct the airway or as a cause of tracheal compression. Infantile myofibroma is an extremely uncommon cause of neonatal airway obstruction. More frequently encountered tracheal tumours include papilloma and haemangioma, which may be treated with surgical excision.
2. Case report A 32/40 premature Caucasian boy of fit unrelated parents presented soon after birth with deteriorating respiratory distress leading to the requirement of continuous positive airway pressure (CPAP) and subsequently intubation and ventilation. Intubation proved to be difficult due to resistance to endotracheal tube advancement beyond the vocal cords. Microlaryngoscopy and bronchoscopy on day 3 revealed stenosis in the region of the subglottis and cricoid ring. This obstruction progressed, resulting in respiratory difficulties. In order to protect the airway, an elective tracheostomy was performed on day 5. A further routine microlaryngoscopy and bronchoscopy was performed after 4 months. During the procedure, no identifiable larynx was visible as it was obscured by a large papillomatous lesion in the oropharynx seeming to arise from the left parapharyngeal wall and filling the hypopharynx (Fig. 1). No airway was visible. This mass was not seen at the previous examination on day 3. Biopsies were taken
N. Eze et al.
for histological analysis and a diagnosis of infantile myofibroma was made (Fig. 2). To assess the extent of the lesion, an MRI scan of the neck was obtained which revealed an extensive abnormality (Figs. 3 and 4). A lesion was seen arising from the oropharynx, extending to the thoracic inlet and encircling the trachea. It incorporated the thyroid gland and the left submandibular gland. On the left side, the mass extended anteriorly into the floor of the mouth. Surgical excision was not possible due to the size and vast extent of the mass, therefore, the child was referred to the paediatric oncologists for trial of empiric chemotherapy. Chemotherapy commenced in July 2003, 3 weeks after diagnosis. The MMT 951 protocol of intravenous vincristine and actinomycin D on a 3-weekly cycle was followed and was well tolerated [10]. In order to assess any change, a repeat MRI was performed after 13 weeks of chemotherapy. This revealed no change in the size of the mass. At this point, another examination under anaesthetic confirmed the lesion still to be present but appearing less inflamed. The large biopsies taken showed chronic inflammation but no evidence of recurrent myofibroma.
3. Discussion There are several reports of infantile myofibroma in the head and neck region including the oral cavity.
Fig. 1 Intra-operative photograph of oropharynx. The uvula is recognisable in the centre of the picture. There is no identifiable airway as the base of the tongue is filled with myofibroma.
Solitary infantile myofibroma compromising the airway
1535
Fig. 2 Low power view showing the papilloma-like intralaryngeal presentation of the mass. There is a spindle cell lesion with morphological and immunohistochemical evidence of myofibroblastic differentiation.
Fig. 3 Axial MRI showing extent of myofibroma. There is gross abnormality within the oropharynx particularly in the base of the tongue. On the left side, it extends anteriorly in the region of the floor of the mouth.
1536
N. Eze et al.
Fig. 4 Coronal MRI showing extent of myofibroma encircling and obliterating the airway from the oropharynx to the thoracic inlet. The thyroid gland and left submandibular gland are involved.
There have only been two described definite subglottic fibromas compromising the airway and requiring a tracheostomy in the literature [11,12]. In both cases, the lesions recurred despite multiple excisions during microlaryngoscopy and bronchoscopy. These cases demonstrate the difficulty in obtaining complete excision of an intratracheal lesion and the subsequent risk of recurrence. There has been no description of such a vastly extensive oropharyngeal myofibroma resulting in complete airway obstruction. Tostevin et al. [13] reported a case of a posterior mediastinal myofibroma compressing the trachea, which was excised via a right thoracotomy after airway stabilisation with a tracheostomy. This was complicated with a right phrenic nerve palsy and multiple respiratory arrests leading to death secondary to hypoxic brain damage. This case has similarities to the one described in this report due to the external compression of the trachea and highlights the potential complications that may arise as a result of surgical intervention. In the case described in this report, due to the relative size, rapid growth of the lesion and proximity to the airway and important structures
in the neck, it would be impossible to achieve simple resection and it would be unwise to wait for possible regression of the lesion. This case has proven to be extremely difficult to manage as the myofibroma is now compressing the posterior tracheal region just above the carina and there is additional airway pathology; tracheomalacia and left bronchomalacia. An adjustable flange tracheostomy tube is now in place to stent the trachea bypassing the posterior obstructive swelling but this often becomes blocked resulting in severe respiratory distress. The use of CPAP and steroids has stabilised the airway. Low dose chemotherapy has been shown to be potentially effective in the treatment of generalised myofibromatosis but the ideal combination of chemotherapeutic agents and the optimal treatment time has not been described and requires further study [10].
4. MMT 951 protocol Intravenous vincristine (0.025 mg/kg/dose) and actinomycin D (0.025 mg/kg/dose) on a 3-weekly cycle for 6 months.
Solitary infantile myofibroma compromising the airway
Acknowledgements We would like to thank Professor Cyril Fisher, professor of soft tissue pathology at the Royal Marsden Hospital, Fulham Road, London, UK, for his second opinion of the histological features of this lesion.
References [1] T.E. Winsell, J. Davis, et al. Infantile myofibromatosis: the most common fibrous tumour of infancy, J. Pediatr. Surg. 23 (1998) 314—318. [2] A.P. Stout, Juvenile fibromatosis, Cancer 7 (1954) 953—978. [3] E.B. Chung, F.M. Enzinger, Infantile myofibromatosis, Cancer 48 (1981) 1807. [4] Y. Daimaru, H. Hashimoto, et al. Am. J. Surg. Pathol. 13 (1989) 859.
1537
[5] N. Vigneswaran, C.A. Waldron, Solitary infantile myofibromatosis of the mandible, Oral Surg. Oral Med. Oral Pathol. 73 (1992) 84. [6] J.T. Wolfe, P.H. Cooper, Solitary cutaneous ‘‘infantile’’ myofibroma in a 49-year old woman, Hum. Pathol. 21 (1990) 62. [7] N.S. Goldberg, B. Bauer, et al. Infantile myofibromatosis: a review of the clinicopathology with perspectives on new treatment choice, Pediatr. Dermatol. 5 (1988) 37—46. [8] S.F. Hogan, J.R. Salassa, Recurrent adult myofibromatosis: a case report, J. Clin. Pathol. 97 (1992) 810. [9] M. Lingen, R. Mostofi, D. Solt, Myofibromas of the oral cavity, Oral Surg. Oral Med. Oral Pathol. 80 (1995) 297—302. [10] M.M. Gandhi, P.C. Nathan, et al. Successful treatment of life threatening generalised infantile myofibromatosis, J. Pediatr. Hematol. Oncol. 25 (9) (2003) 750—754. [11] Tsui, J.M. Kore, Congenital subglottic fibroma in the newborn, Laryngoscope 86 (4) (1976) 571—576. [12] J.E. O’connell, F. Raafat, et al. Congenital subglottic fibromatosis, J. Laryngol. Otol. 103 (1989) 983—985. [13] P. Tostevin, M. Wyatt, A. Hosni, Six cases of fibromatosis of the head and neck in children, Int. J. Pediatr. Otorhinolarngol. 53 (2000) 235—244.
www.elsevier.com/locate/ijporl
CASE REPORT
Solitary infantile myofibroma compromising the airway N. Ezea,*, L. Pitkina, S. Crowleyb, P. Wilsonc, H. Dayaa a
Department of Otolaryngology, St. George’s Hospital, London, UK Department of Paediatrics, St. George’s Hospital, London, UK c Department of Histopathology, St. George’s Hospital, London, UK b
Received 1 July 2003; received in revised form 7 February 2004; accepted 7 April 2004
KEYWORDS Neonate; Myofibroma; Airway; Obstruction
Summary Infantile myofibromatosis is an uncommon and benign condition presenting in the neonatal period. It is self-limiting disease that may present as a localised or generalised process. Various examples of this entity have been reported in the literature. This report describes a neonate with a rapidly growing oropharyngeal lesion obstructing the airway that had the typical histological features of an infantile myofibroma. This case report highlights that a solitary myofibroma may be incredibly extensive making complete excision impossible and can be particularly challenging to manage in terms of airway stabilisation. # 2004 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Infantile myofibroma is an uncommon fibrous tumour of infancy [1] and was first described by Stout in 1954 [2]. Infant males are most commonly affected [3]. Myofibroma also occurs in adults and may be multiple or solitary [3,4]. Solitary lesions are more common than multiple ones. They usually involve the head and neck region and the trunk [5]. The lesions appear in the skin, muscle, subcutaneous tissue, bone and viscera and are usually firm, rubbery or hard masses. They may be anywhere between a few millimetres to several centi* Corresponding author. Present address: Department of Otorhinolaryngology, St. George’s Hospital, London SW17 0QT, UK. Tel.: +44 208 672 1255; fax: +44 208 725 3306. E-mail address: [email protected] (N. Eze).
metres in diameter [6] and are generally non-tender and slow growing. Solitary lesions pursue a benign course and regress spontaneously by age 1 or 2. The prognosis is excellent if limited to skin and bone but some may have an aggressive clinical presentation. Patients with visceral involvement usually have multiple lesions involving internal organs and have an 80% mortality [7]. Surgical excision is the treatment of choice. The recurrence rate is low at 10% despite the lesions being non-encapsulated and is attributable to incomplete excision in anatomically inaccessible areas [8]. Although oral myofibroma have been extensively described in the literature, they usually involve the mandible, buccal mucosa and tongue in reducing frequency [9]. Lesions of oral origin have not been described in the literature as extensive enough to
0165-5876/$ — see front matter # 2004 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2004.04.035
1534
obstruct the airway or as a cause of tracheal compression. Infantile myofibroma is an extremely uncommon cause of neonatal airway obstruction. More frequently encountered tracheal tumours include papilloma and haemangioma, which may be treated with surgical excision.
2. Case report A 32/40 premature Caucasian boy of fit unrelated parents presented soon after birth with deteriorating respiratory distress leading to the requirement of continuous positive airway pressure (CPAP) and subsequently intubation and ventilation. Intubation proved to be difficult due to resistance to endotracheal tube advancement beyond the vocal cords. Microlaryngoscopy and bronchoscopy on day 3 revealed stenosis in the region of the subglottis and cricoid ring. This obstruction progressed, resulting in respiratory difficulties. In order to protect the airway, an elective tracheostomy was performed on day 5. A further routine microlaryngoscopy and bronchoscopy was performed after 4 months. During the procedure, no identifiable larynx was visible as it was obscured by a large papillomatous lesion in the oropharynx seeming to arise from the left parapharyngeal wall and filling the hypopharynx (Fig. 1). No airway was visible. This mass was not seen at the previous examination on day 3. Biopsies were taken
N. Eze et al.
for histological analysis and a diagnosis of infantile myofibroma was made (Fig. 2). To assess the extent of the lesion, an MRI scan of the neck was obtained which revealed an extensive abnormality (Figs. 3 and 4). A lesion was seen arising from the oropharynx, extending to the thoracic inlet and encircling the trachea. It incorporated the thyroid gland and the left submandibular gland. On the left side, the mass extended anteriorly into the floor of the mouth. Surgical excision was not possible due to the size and vast extent of the mass, therefore, the child was referred to the paediatric oncologists for trial of empiric chemotherapy. Chemotherapy commenced in July 2003, 3 weeks after diagnosis. The MMT 951 protocol of intravenous vincristine and actinomycin D on a 3-weekly cycle was followed and was well tolerated [10]. In order to assess any change, a repeat MRI was performed after 13 weeks of chemotherapy. This revealed no change in the size of the mass. At this point, another examination under anaesthetic confirmed the lesion still to be present but appearing less inflamed. The large biopsies taken showed chronic inflammation but no evidence of recurrent myofibroma.
3. Discussion There are several reports of infantile myofibroma in the head and neck region including the oral cavity.
Fig. 1 Intra-operative photograph of oropharynx. The uvula is recognisable in the centre of the picture. There is no identifiable airway as the base of the tongue is filled with myofibroma.
Solitary infantile myofibroma compromising the airway
1535
Fig. 2 Low power view showing the papilloma-like intralaryngeal presentation of the mass. There is a spindle cell lesion with morphological and immunohistochemical evidence of myofibroblastic differentiation.
Fig. 3 Axial MRI showing extent of myofibroma. There is gross abnormality within the oropharynx particularly in the base of the tongue. On the left side, it extends anteriorly in the region of the floor of the mouth.
1536
N. Eze et al.
Fig. 4 Coronal MRI showing extent of myofibroma encircling and obliterating the airway from the oropharynx to the thoracic inlet. The thyroid gland and left submandibular gland are involved.
There have only been two described definite subglottic fibromas compromising the airway and requiring a tracheostomy in the literature [11,12]. In both cases, the lesions recurred despite multiple excisions during microlaryngoscopy and bronchoscopy. These cases demonstrate the difficulty in obtaining complete excision of an intratracheal lesion and the subsequent risk of recurrence. There has been no description of such a vastly extensive oropharyngeal myofibroma resulting in complete airway obstruction. Tostevin et al. [13] reported a case of a posterior mediastinal myofibroma compressing the trachea, which was excised via a right thoracotomy after airway stabilisation with a tracheostomy. This was complicated with a right phrenic nerve palsy and multiple respiratory arrests leading to death secondary to hypoxic brain damage. This case has similarities to the one described in this report due to the external compression of the trachea and highlights the potential complications that may arise as a result of surgical intervention. In the case described in this report, due to the relative size, rapid growth of the lesion and proximity to the airway and important structures
in the neck, it would be impossible to achieve simple resection and it would be unwise to wait for possible regression of the lesion. This case has proven to be extremely difficult to manage as the myofibroma is now compressing the posterior tracheal region just above the carina and there is additional airway pathology; tracheomalacia and left bronchomalacia. An adjustable flange tracheostomy tube is now in place to stent the trachea bypassing the posterior obstructive swelling but this often becomes blocked resulting in severe respiratory distress. The use of CPAP and steroids has stabilised the airway. Low dose chemotherapy has been shown to be potentially effective in the treatment of generalised myofibromatosis but the ideal combination of chemotherapeutic agents and the optimal treatment time has not been described and requires further study [10].
4. MMT 951 protocol Intravenous vincristine (0.025 mg/kg/dose) and actinomycin D (0.025 mg/kg/dose) on a 3-weekly cycle for 6 months.
Solitary infantile myofibroma compromising the airway
Acknowledgements We would like to thank Professor Cyril Fisher, professor of soft tissue pathology at the Royal Marsden Hospital, Fulham Road, London, UK, for his second opinion of the histological features of this lesion.
References [1] T.E. Winsell, J. Davis, et al. Infantile myofibromatosis: the most common fibrous tumour of infancy, J. Pediatr. Surg. 23 (1998) 314—318. [2] A.P. Stout, Juvenile fibromatosis, Cancer 7 (1954) 953—978. [3] E.B. Chung, F.M. Enzinger, Infantile myofibromatosis, Cancer 48 (1981) 1807. [4] Y. Daimaru, H. Hashimoto, et al. Am. J. Surg. Pathol. 13 (1989) 859.
1537
[5] N. Vigneswaran, C.A. Waldron, Solitary infantile myofibromatosis of the mandible, Oral Surg. Oral Med. Oral Pathol. 73 (1992) 84. [6] J.T. Wolfe, P.H. Cooper, Solitary cutaneous ‘‘infantile’’ myofibroma in a 49-year old woman, Hum. Pathol. 21 (1990) 62. [7] N.S. Goldberg, B. Bauer, et al. Infantile myofibromatosis: a review of the clinicopathology with perspectives on new treatment choice, Pediatr. Dermatol. 5 (1988) 37—46. [8] S.F. Hogan, J.R. Salassa, Recurrent adult myofibromatosis: a case report, J. Clin. Pathol. 97 (1992) 810. [9] M. Lingen, R. Mostofi, D. Solt, Myofibromas of the oral cavity, Oral Surg. Oral Med. Oral Pathol. 80 (1995) 297—302. [10] M.M. Gandhi, P.C. Nathan, et al. Successful treatment of life threatening generalised infantile myofibromatosis, J. Pediatr. Hematol. Oncol. 25 (9) (2003) 750—754. [11] Tsui, J.M. Kore, Congenital subglottic fibroma in the newborn, Laryngoscope 86 (4) (1976) 571—576. [12] J.E. O’connell, F. Raafat, et al. Congenital subglottic fibromatosis, J. Laryngol. Otol. 103 (1989) 983—985. [13] P. Tostevin, M. Wyatt, A. Hosni, Six cases of fibromatosis of the head and neck in children, Int. J. Pediatr. Otorhinolarngol. 53 (2000) 235—244.